Section 2 Flashcards by Robert Euro Djojoseputro

Hydrocephalus (HCP) is abnormal accumulation of CSF within ventricles of brain. What is the most common cause of communicating hydrocephalus in pediatric population?
● A. Chiari type 2 malformation with MMC
● B. Chiari type 1 malformation without MMC
● C. Periaqueductal stenosis
● D. Infection
● E. Post hemorrhagic

D. Infection

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A 2-month-old child presents with irritability, N/V, and poor head control. On examination, he/she has bulging fontanelle, engorged scalp veins, setting sun sign, and positive Macewan’s sign. MRI of brain showed periaqueductal gliosis in this patient. Following are the causes of congenital hydrocephalus in pediatric population except?
● A. Chiari type 2 with MMC
● B. Chiari type 1 without MMC
● C. Secondary aqueductal gliosis
● D. Dandy Walker malformation
● E. Colloid cyst blocking CSF flow at aqueduct

E. Colloid cyst blocking CSF flow at aqueduct

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Occipital frontal circumference is used to monitor head growth and to assess possible pathological process causing change in head measurement from normal. Active hydrocephalus, subdural hematoma, or subdural eﬀusion is signified by which of the following?
● A. Progressive upward deviation from the normal curve
● B. Continued head growth of more than 1.25 cm/wk
● C. OFC measuring 2 standard deviations above normal
● D. Head circumference out of proportion to body length or weight
● E. All of the above

E. All of the above

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Pregeniculate blindness is because of lesion anterior to the lateral geniculate body while postgeniculate blindness is because of lesion posterior to the lateral geniculate body. Following are the causes of blindness from hydrocephalus except?
● A. Occlusion of posterior cerebral arteries (caused by downward transtentorial herniation)
● B. Diﬀuse hypoxic injury to occipital lobes caused by hypotension
● C. Chronic papilledema causing injury to optic nerve at optic disk
● D. Dilatation of third ventricle with compression of optic chiasma
● E. Compression of PCA caused by upward herniation of cerebellum

B. Diﬀuse hypoxic injury to occipital lobes caused by hypotension

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Following are the specific radiologic criteria for the diagnosis of hydrocephalus except?
● A. Evans ratio (FH/BPD) more than 0.3 shows hydrocephalus
● B. Size of both temporal horns > 2 mm shows HCP
● C. Slit third ventricle
● D. Periventricular low density on plain CT of brain and hyperintensity on MRI of brain
● E. Ballooning of lateral and frontal ventricles

C. Slit third ventricle

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Following are features indicative of chronic hydrocephalus except?
● A. Beaten copper cranium
● B. Third ventricle herniating into sella
● C. Erosion of sella turcica
● D. The temporal horns are markedly prominent
● E. Atrophy and upward bowing of corpus callosum

D. The temporal horns are markedly prominent

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Pseudohydrocephalus refers to conditions that may mimic hydrocephalus but are not due to inadequate CSF absorption. Which of the following can be called as pseudohydrocephalus?
● A. Hydrocephalus ex vacuo due to cerebral atrophy
● B. Developmental anomalies like agenesis of corpus callosum, septo-optic dysplasia, or hydranencephaly
● C. Otic hydrocephalus
● D. External hydrocephalus
● E. All of the above

E. All of the above

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A 4-month-old child presents with macrocrania and frontal bossing. Plain CT of brain showed enlarged subarachnoid spaces over the cortical sulci of frontal poles. Basal cisterns and anterior hemispheric fissure are also seen to be enlarged. A diagnosis of external hydrocephalus or benign external hydrocephalus is made. What is the most appropriate treatment for this condition?
● A. Usually resolves spontaneously before 2 years of age
● B. Parents may need to periodically reposition the head while the child is sleeping
● C. Shunt may be needed for cosmetic reasons in severe macrocrania
● D. Parents are reinsured that this is not cortical atrophy
● E. All of the above are correct

E. All of the above are correct

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A disconnected ventriculoperitoneal shunt may continue to function through an endothelialized subcutaneous tract. Which of the following statements is true regarding repair or removing of a VP shunt?
● A. Indications for shunt repair are marginally functioning shunt with signs and symptoms of increased ICP
● B. Most of the patients with spina bifida or aqueductal stenosis are not shunt dependent
● C. If a shunt is nonfunctioning and the patient is not shunt dependent then the shunt should be removed regardless of shunt infection or not
● D. A patient with nonfunctioning shunt with no symptoms does not need to be followed up
● E. All of the above are correct

A. Indications for shunt repair are marginally functioning shunt with signs and symptoms of increased ICP

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A patient above 60 years of age presents with gait disturbance (wide based, short shuﬄing steps like glued to the ground), dementia with slowing of thoughts (bradyphrenia), and urinary incontinence with urgency. On examination, there is no papilledema, hyperreflexia, or lateralizing signs. LP was done which showed normal pressure. MRI showed ventricular enlargement without any blockage. Following are features that correlate with favorable response to shunt in this condition except?
● A. Periventricular low density on plain CT of brain and high intensity on T2WI MRI
● B. Compression of convexity sulci
● C. Rounding of the frontal horns
● D. Dilated subarachnoid spaces in high convexity
● E. Upward bowing and thinning of corpus collosum

D. Dilated subarachnoid spaces in high convexity

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Which of the following is a component of iNPH Radscale?
● A. Evans ratio increased
● B. Tight high convexity
● C. Sylvian fissure enlargement
● D. Decrease of callosal angle
● E. All of the above are true

E. All of the above are true

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Which of the following is true regarding treatment of normal pressure hydrocephalus?
● A. Positive response on tap test increase the likelihood of responding to shunting
● B. VP shunt is the procedure of choice with use of medium pressure valve
● C. ETV has been advocated by some authors
● D. Most likely symptom to improve with shunting is incontinence then gait disturbance and then dementia
● E. All of the above

E. All of the above

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Diuretic therapy may be tried in premature infants with bloody CSF as long as there is no evidence of active hydrocephalus, and satisfactory control is reported in about half of the infants. What is the initial dose of acetazolamide in mg/kg/d in divided doses?
● A. 1
● B. 5
● C. 10
● D. 25
● E. 50

D. 25

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A 4-year-old child was admitted with hydrocephalus and tectal glioma. He had no previous surgery. What is the ETV success score?
● A. 10
● B. 20
● C. 30
● D. 60
● E. 80

E. 80

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What is the most common cause of shunt malfunction?
● A. Proximal end blockade
● B. Distal end blockade
● C. Shunt fracture
● D. Silicone allergy
● E. Streptococcus viridians infection

A. Proximal end blockade

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Upward gaze palsy or setting sun sign is associated with which of the following?
● A. Parinaud’s syndrome
● B. Pulfrich syndrome
● C. Anton’s syndrome
● D. Ridoch’s phenomenon
● E. Weber syndrome

A. Parinaud’s syndrome

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Contrast medium commonly used for X-ray shuntogram is what?
● A. Gadolinium
● B. Iohexol
● C. 99m-technetium pertechnetate
● D. Gadopentate dimeglumine
● E. Ferric ammonium citrate

B. Iohexol

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What is the shunt malfunction rate in percentage during the first year of placement in the pediatric population?
● A. 1
● B. 3
● C. 7
● D. 17
● E. 49

D. 17

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What is the treatment of choice for short-term symptoms of intracranial hypotension due to over-shunting of a VP shunt?
● A. Antibiotics
● B. Steroids
● C. Antisiphon device
● D. Shunt removal
● E. Y connector shunt

C. Antisiphon device

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What do the three dots visible on the shunt valve on X-ray represent?
● A. Very low pressure
● B. Low pressure
● C. Medium pressure
● D. High pressure
● E. Very high pressure

D. High pressure

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Diuretic therapy is used in premature infants for bloody CSF with no evidence of active hydrocephalus as an adjunct to definitive treatment or as a temporizing measure. Satisfactory control of hydrocephalus can also be achieved in children less than 1 year of age with stable vital signs, normal renal function, and no symptoms of elevated ICP (apnea, lethargy, vomiting) using diuretic therapy. Following are true regarding diuretic therapy except?
● A. Acetazolamide is started at 25 mg/kg/d with simultaneous start of furosemide at 1 mg/kg/d
● B. To counteract acidosis, tricitrate is used at a dose of 4 mL/kg/d
● C. There is no need to measure electrolytes along with diuretic therapy
● D. Weekly ultrasound or CT of brain is performed and ventricular shunt is inserted if ventriculomegaly occurs
● E. This therapy is used for 6 months and then dosage is tapered over 2 to 4 weeks

C. There is no need to measure electrolytes along with diuretic therapy

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Surgical methods to control hydrocephalus include third ventriculostomy, various shunting methods, eliminating the obstruction like tumor, and choroid plexectomy. What is the goal of surgery in such patients?
● A. Normal sized ventricles
● B. Opened basal cisterns
● C. Optimum neurologic function and good cosmetic result
● D. Opened subarachnoid spaces
● E. Both A and B

C. Optimum neurologic function and good cosmetic result

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Endoscopic third ventriculostomy is an endoscopic method of dealing with hydrocephalus. Which of the following is an indication of endoscopic third ventriculostomy?
● A. It is used in patients with obstructive hydrocephalus
● B. In managing shunt infection as a means to remove all hardware without subjecting the patient to increased ICP
● C. It is also proposed as an option for patients who developed subdural hematomas after shunting in which the shunt is removed before ETV is performed
● D. It is also indicated for slit ventricle syndrome
● E. All of the above are indications of ETV

E. All of the above are indications of ETV

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During ETV complications can occur such as injury to basilar artery, PCom, or PCA, injury to pituitary stalk and hypothalamus, or transient 3rd and 6th nerve palsies. ETV has low success rate in infants because of which of the following?
● A. There are higher chances of complications to occur
● B. It is diﬃcult to do ventriculostomy in infants
● C. Mortality in infants in high after ETV
● D. They may not have a normally developed subarachnoid space
● E. All of the above

D. They may not have a normally developed subarachnoid space

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ETV success score is used to predict the likelihood of success of ETV. Scores less than 40% correlate with very low chance of success while scores more than 80% show better chance of success compared to shunting. A child 3 years of age with tectal tumor and no history of previous shunt has ETV success score of what? ● A. 60% ● B. 70% ● C. 80% ● D. 90% ● E. 50%

C. 80%

There are various types of shunts including ventriculoperitoneal shunt (VP), ventriculoatrial shunt (VA), Torkildsen shunt (shunts ventricles to CSF space), ventriculopleural shunt, and lumboperitoneal shunt. VP shunt is the most commonly used shunt in modern era. What are indications of VA shunt instead of VP shunt in a patient? ● A. Extensive abdominal surgery ● B. Peritonitis ● C. Morbid obesity ● D. In patients who have had NEC ● E. All of the above

E. All of the above

What is the most common complication of a shunt? ● A. Obstruction ● B. Disconnection at a junction or breakage at any point ● C. Infection ● D. Seizures ● E. Extraneural metastasis of certain tumors

A. Obstruction

A child underwent VP shunting with reprogrammable shunt, the pressure setting on which can be seen using an X-ray perpendicular to the shunt valve. This boy after some time presents with N/V, headache, lethargy, and ataxia. On physical examination, this child has bulging fontanelles and upward gaze palsy. What is the appropriate method for evaluation of shunt malfunction? ● A. Shunt series (plain X-rays to visualize the entire shunt) ● B. Using ultrasound in patient with open fontanelles ● C. Plain CT of brain ● D. MRI of brain ● E. All of the above

A. Shunt series (plain X-rays to visualize the entire shunt)

What are possible complications of over-shunting of VP shunt? ● A. Dilated ventricles ● B. Intracranial hypertension ● C. Subdural hematomas ● D. Wide opened sylvian aqueduct ● E. All of the above

C. Subdural hematomas

A patient after VP shunting presents after 1 year with intermittent headaches, nausea, vomiting, irritability, and impaired mentation. Plain CT of brain showed totally collapsed ventricles. What is the procedure of choice in this patient? ● A. Expectant management ● B. Upgradation to a higher-pressure valve ● C. Upgradation to low-pressure valve ● D. Removal of shunt and reinserting again ● E. Antisiphon devices have no role in these patients

B. Upgradation to a higher-pressure valve

A patient was brought to the OPD with history of seizures. His mother showed you a video recording of seizure episode where you noticed fairly symmetric, bilateral, synchronous, semirhythmic jerking of the upper and lower extremities, with elbow flexion and knee extension. What are these? ● A. Partial seizures ● B. Tonic seizures ● C. Clonic seizures ● D. Atonic seizures ● E. Astatic seizures

C. Clonic seizures

A patient presented to you with history of seizures. On further inquiry you find that there is no history of aura, motor involvement, and postictal confusion. His EEG revealed spike and wave at exactly 3/sec. These features are most consistent which of the following? ● A. Uncal seizures ● B. Mesial temporal seizures ● C. Absence seizures ● D. Partial seizures ● E. Grand mal seizures

● C. Absence seizures

What is the most common cause of intractable temporal lobe epilepsy? ● A. Absence seizures ● B. Uncal epilepsy ● C. Mesial temporal sclerosis ● D. Superior temporal gyrus syndrome ● E. Temporal lobe hemorrhagic contusion

C. Mesial temporal sclerosis

A patient with recently diagnosed epilepsy presented to the OPD for follow-up. During the review of the radiology, the clinician notices hippocampal atrophy on MRI. What is the most likely diagnosis? ● A. West syndrome ● B. Lennox Gustate syndrome ● C. Mesial temporal lobe epilepsy ● D. Juvenile myoclonic epilepsy ● E. Uncinate seizures

C. Mesial temporal lobe epilepsy

A patient with known case of temporal lobe epilepsy presents with kakosmia. This subtype of seizures is classified as? ● A. Uncinate seizures ● B. Complex seizures ● C. Focal seizures ● D. Sensory seizures

A. Uncinate seizures

Factors that lower the seizure threshold (make it easier to provoke a seizure) include sleep deprivation, hyperventilation, photic stimulation, infection, metabolic disturbances, and head trauma. Todd's paralysis is a phenomenon that occurs after seizure activity. Which of the following is true regarding Todd's paralysis? ● A. Partial or total paralysis usually occurs in areas involved in a partial seizure ● B. It occurs more commonly in nonstructural lesion ● C. The paralysis usually resolves over days to weeks ● D. Postictal aphasia or hemianopia are not included in this ● E. All of the above are true

A. Partial or total paralysis usually occurs in areas involved in a partial seizure

A patient presents in neurosurgical emergency with complaint of first occurrence of focal seizure with retained consciousness (simple partial seizure). As a rule of thumb, what is the cause of such seizure? ● A. Metabolic cause ● B. Insult to brain within previous 2 weeks ● C. Structural lesion within brain ● D. Febrile seizure ● E. None of the above

C. Structural lesion within brain

Tonic–clonic seizure is a type of motor seizure which is a generalized seizure that evolves from tonic to clonic motor activity. A patient presenting with other type of motor seizure in which there is shock-like body jerks (one or more in succession) with generalized EEG discharges is said to have which type of seizure? ● A. Clonic ● B. Tonic ● C. Myoclonic tonic clonic ● D. Myoclonic ● E. Myoclonic-tonic

D. Myoclonic

A patient with known family history of seizure is presented with complex partial seizure that begins with arrest and stare, and oroalimentary and complex automatism signs with posturing of arm. This seizure activity lasts for 1 to 2 minutes and there is preictal olfactory aura for few seconds as well. He is suspected to have mesial temporal lobe epilepsy or mesial temporal sclerosis. Which of the following will help in the diagnosis in this case? ● A. MRI of brain showing hippocampal atrophy and signal alteration with ipsilateral dilatation of temporal horn of lateral ventricle ● B. Unilateral or bilateral independent anterior temporal EEG spikes with maximal amplitude in basal electrodes ● C. PET scan showing hypometabolism in temporal lobe ● D. Neuropsychological testing showing memory dysfunction specific to involved temporal lobe ● E. All of the above

E. All of the above

A patient with strong family history of epilepsy presents with history of myoclonic jerks on waking up in morning. EEG shows poly spike discharges and MRI shows no structural lesion. The patient is suspected to have a generalized epilepsy syndrome with generalized tonic clonic and absence types in addition to the type mentioned above. What is this syndrome called? ● A. West syndrome ● B. Lennox–Gastaut syndrome ● C. Juvenile myoclonic epilepsy ● D. Temporal lobe syndrome ● E. Juvenile tonic clonic epilepsy

C. Juvenile myoclonic epilepsy

Which of the following is true regarding West syndrome? ● A. It is a seizure disorder that typically begins in first year of life ● B. It consists of recurrent gross fixation and occasionally extension of trunks and limbs ● C. It is usually associated with mental retardation with EEG showing interictal hypsarrhythmia or modified hypsarrhythmia ● D. Seizures usually respond dramatically to ACTH or corticosteroids ● E. All of the above

E. All of the above

Hippocampus is composed of Ammon's horn and dentate gyrus. The dorsal hippocampus subserves aspects of spatial memory, verbal memory, and learning conceptual information. Hippocampal sclerosis can cause which of the following? ● A. Total memory impairment ● B. Loss of all functions of basal ganglia ● C. Olfactory loss ● D. Intractable temporal lobe epilepsy ● E. All of the above

D. Intractable temporal lobe epilepsy

Following are motor seizures except? ● A. Tonic ● B. Gelastic seizures ● C. Clonic ● D. Atonic ● E. Epileptic spasms

B. Gelastic seizures

Symptomatic seizure also known as secondary seizure results from structural abnormality within the brain. Which of the following is included in this type of seizure? ● A. Cavernous malformation ● B. Stroke ● C. Brian tumor ● D. Mesial temporal sclerosis ● E. All of the above

E. All of the above

Mortality in patients with epilepsy is 2 to 4 times that of general population. Which of the following deaths are directly related to seizure? ● A. Status epilepticus ● B. Accidents during seizure activity ● C. Suicides ● D. Sudden unexplained death in epilepsy ● E. All of the above

A. Status epilepticus

A patient is on antiepileptic drugs (AED) for 2 years. On follow-up, his liver function tests were deranged. AED should be discontinued based on derangement of which parameter? ● A. Fatty liver on USG ● B. Acute decrease in ALT ● C. Decrease in ALT/AST ratio ● D. GGT levels twice the normal ● E. Hyperalbuminemia

D. GGT levels twice the normal

What is the dose adjustment of phenytoin in renal failure? ● A. Continue same dosage ● B. Two-thirds of the previous dosage ● C. Half the previous dose ● D. Double the previous dose ● E. Only give on SOS basis

A. Continue same dosage

What is the parenteral loading dose of phenytoin for adults? ● A. 16 mg/kg ● B. 18 mg/kg ● C. 20 mg/kg ● D. 22 mg/kg ● E. 25 mg/kg

B. 18 mg/kg

What is the most common electrolyte imbalance associated with carbamazepine? ● A. Hypernatremia ● B. Hyponatremia ● C. Hypochloremia ● D. Hyperchloremia ● E. Hypermagnesia

B. Hyponatremia

Carbamazepine should be discontinued when CBC shows a WBC count of what? ● A. < 10,000 ● B. < 7000 ● C. < 6000 ● D. < 5000 ● E. < 4000

< 4000

Oxcarbazepine was prescribed to a patient for epilepsy. Which blood tests should be employed to monitor the side effects? ● A. CBC ● B. RFTs ● C. Electrolytes ● D. Urinalysis ● E. No blood tests required

E. No blood tests required

What is the maximum recommended adult dose of valproic acid? ● A. 20 mg/kg/d ● B. 30 mg/kg/d ● C. 40 mg/kg/d ● D. 50 mg/kg/d ● E. 60 mg/kg/d

E. 60 mg/kg/d

What is the therapeutic level of valproic acid? ● A. 5 to 10 μg/mL ● B. 10 to 20 μg/mL ● C. 20 to 30 μg/mL ● D. 30 to 40 μg/mL ● E. 50 to 100 μg/mL

E. 50 to 100 μg/mL

What is the loading dose of phenobarbital in adults? ● A. 10 mg/kg ● B. 12 mg/kg ● C. 15 mg/kg ● D. 18 mg/kg ● E. 20 mg/kg

E. 20 mg/kg

What is the recommended daily dose of ethosuximide in adults? ● A. 500 to 1,500 mg/d ● B. 500 to 1,000 mg/d ● C. 250 to 500 mg/d ● C. 100 to 200 mg/d ● D. 100 to 300 mg/d

A. 500 to 1,500 mg/d

What is the initial dose of levetiracetam in adults? ● A. 500 mg oral twice daily ● B. 400 mg oral twice daily ● C. 300 mg oral twice daily ● D. 250 mg oral twice daily ● E. 100 mg thrice daily

A. 500 mg oral twice daily

What is the maximum daily dose of levetiracetam for adults? ● A. 1,000 mg ● B. 1,500 mg ● C. 2,000 mg ● D. 3,000 mg ● E. 1 g

D. 3,000 mg

When given for epilepsy, what is the usual target dosage of gabapentin? ● A.100 mg/d ● B. 1,000 mg/d ● C. 500 to 1,000 mg/d ● D. 800 to 1,800 mg/d ● E. 5 g/d

D. 800 to 1,800 mg/d

The maximum eﬀect of topiramate for seizures control is observed at what dose? ● A. 100 mg/d ● B. 200 mg/d ● C. 400 mg/d ● D. > 600 mg/d ● E. 1 g/d

D. > 600 mg/d

The anticonvulsant eﬀect of lamotrigine is due to presynaptic inhibition of release of what? ● A. Calcium ● B. Potassium ● C. Albumin ● D. Glutamate ● E. Serotonin

D. Glutamate

Fetal hydantoin syndrome is associated with which of the following? ● A. Valproic acid ● B. Levetiracetam ● C. Lamotrigine ● D. Phenytoin ● E. Benzodiazepines

D. Phenytoin

Ethosuximide is drug of choice for what? ● A. Absence seizures ● B. GTC seizures ● C. Myoclonic seizures ● D. Tonic seizures ● E. Atonic seizures

A. Absence seizures

Aside from some specific agents for some particular seizure types, there is generally no significant diﬀerence in eﬃcacy of antiseizure medication. Hence, choices are made on the basis of tolerability, adverse eﬀects, eﬀects on comorbidities, drug–drug interactions, or availability of dosing form. What is the goal of antiseizure medication? ● A. Seizure control which is seizure-free time for at least 3 years for an epileptic patient and no or minimum chances of seizure in future ● B. Seizure control which is reduction of seizure frequency and severity to very low ● C. Seizure control which is reduction of seizure frequency and severity so that the patient can live normal life without epilepsy-related limitations ● D. Seizure control which is reduction of seizure frequency and severity so that the patient can live normal life without epilepsy-related limitations with minimal or no drug toxicity ● E. None of above

D. Seizure control which is reduction of seizure frequency and severity so that the patient can live normal life without epilepsy-related limitations with minimal or no drug toxicity

In person with focal onset seizure with or without retained awareness and with or without secondary generalization, the indicated antiseizure medications (ASM) are carbamazepine, phenytoin, phenobarbital, and primidone. Which of the follow-ing is the drug indicated in absence seizure? ● A. Ethosuximide ● B. Valproic acid ● C. Clonazepam ● D. Methsuximide ● E. All of the above

E. All of the above

Valproic acid, lamotrigine, levetiracetam, and zonisamide are considered as broad spectrum ASM. A patient presenting with low platelet counts in neurosurgical OPD with primary generalized tonic clonic seizures is to be started with ASM. Which ASM is indicated in this patient as first-line medication? ● A. Valproic acid ● B. Phenytoin ● C. Levetiracetam ● D. Phenobarbital ● E. Lamotrigine

A. Valproic acid

Following statements regarding antiseizure medication are true except? ● A. A given medication dose is increased until seizures are controlled or side eﬀects become intolerable ● B. Monotherapy is tried with diﬀerent drugs before resorting to two drugs together as 80% of epileptics can be controlled with monotherapy ● C. When more than two ASMs are needed, it is considered that the patient is having nonepileptic seizure ● D. An ASM is discontinued if GGT levels exceed normal levels ● E. When first evaluating a patient on multiple drugs, the most sedating ones are withdrawn first

D. An ASM is discontinued if GGT levels exceed normal levels

Oral loading dose of phenytoin is 300 mg every 4 hours until 17 mg/kg is given while IV loading dose is 18 mg/kg slow IV. Maintenance dose in adults is 200 to 500 mg/d and 4 to 7 mg/kg/d in children. It is diluted only in normal saline and given at slow rate of 20 mg/min or maximum up to 40 mg/min because of risk of which following side eﬀect? ● A. Hypotension ● B. Arrythmias ● C. Laryngeal spasm ● D. Acute kidney failure ● E. Both A and B

E. Both A and B

Carbamazepine is indicated for focal onset seizures, generalized tonic clonic seizures with or without secondary generalization, and trigeminal neuralgia. Dosage range in adults is 600 to 2,000 mg/d, while in children it is 20 to 30 kg/mg/d but it is started at low dosage and increased slowly like 200 mg per oral every day for 1st week, then 200 mg twice a day for next week, then 200 mg thrice a day for next week, and so on. Which test is necessary before starting carbamazepine therapy? ● A. CBC ● B. Platelet count ● C. Serum iron ● D. Liver enzymes ● E. A, B, and C

E. A, B, and C

Levetiracetam is used as an adjunctive therapy for partial onset seizures with secondary generalization in patients 4 years of age and older. It is also used in myoclonic seizure (juvenile myoclonic epilepsy) and generalized tonic clonic seizures. It can cause somnolence, fatigue, dizziness, asthenia, or infection as side eﬀect. What is the maximum safe recommended dose of this medication per day? ● A. 2,000 mg ● B. 2,500 mg ● C. 3,000 mg ● D. 3,500 mg ● E. 4,000 mg

C. 3,000 mg

What is the most eﬀective ASM for generalized genetic epilepsies with generalized tonic clonic seizures, which is also eﬀective for all focal onset seizures, and generalized onset seizures including absence seizure and myoclonic seizures? (Adult dosage range of this drug is 600 to 3,000 mg/d, while in children it is 15 to 60 mg/kg/d, while it is contraindicated in pregnancy or in patients below 2 years of age.) ● A. Levetiracetam ● B. Valproate ● C. Phenytoin ● D. Phenobarbital ● E. Lamotrigine

B. Valproate

After cessation of ASM, which of the following factors are found to aﬀect the likelihood of relapse after seizure-free interval of 2 years? ● A. Seizure type: more relapse in patients with generalized seizures as compared to partial seizures ● B. More the number of seizures before control is attained, the more the chance of relapse ● C. Use of multiple ASM is related with more chance of relapse ● D. EEG class 4 has worst prognosis for relapse ● E. All of the above

E. All of the above

For most women of child-bearing potential, monotherapy with the lowest dose of carbamazepine is indicated. What is the second indicated drug in such patients if therapy with carbamazepine is ineﬀective? ● A. Levetiracetam ● B. Valproic acid ● C. Phenytoin ● D. Lamotrigine ● E. Phenobarbital

B. Valproic acid

What is the most common cause of first type seizures in pediatric age group? ● A. Febrile ● B. Hydrocephalus ● C. Electrolyte imbalance ● D. Vascular event ● E. Idiopathic

A. Febrile

Prophylactic antiepileptic drugs reduce the incidence of late post-traumatic epilepsy by how much? ● A. One-fourth ● B. One-third ● C. Half ● D. No eﬀect ● E. Increase the incidence by drug resistance

D. No eﬀect

What is the most common cause of nonepileptic seizures? ● A. Psychogenic ● B. Basilar migraine ● C. Cardiac arrhythmias ● D. Cyclic vomiting syndrome ● E. Dyskinesias

A. Psychogenic

According to the definition of status epilepticus, seizure lasting more than how many minutes warrants management according to status epilepticus protocol? ● A. 1 ● B. 3 ● C. 5 ● D. 30 ● E. 60

C. 5

What is the most common cause of status epilepticus? ● A. Low levels of antiepileptic drugs ● B. Metabolic ● C. Hypoxia ● D. Tumor ● E. Cerebrovascular event

A. Low levels of antiepileptic drugs

What is the first-line treatment for status epilepticus? ● A. Benzodiazepine ● B. Fosphenytoin ● C. Phenytoin ● D. Valproic acid ● E. Phenobarbital

A. Benzodiazepine

What is the drug of choice for myoclonic status epilepticus? ● A. Benzodiazepine ● B. Fosphenytoin ● C. Phenytoin ● D. Valproic acid ● E. Phenobarbital

D. Valproic acid

Antiseizure medication can be tapered oﬀ in which of the following cases after 7 days? ● A. Penetrating brain injury ● B. Development of late PTS ● C. Prior seizures history ● D. Patient undergoing craniotomy ● E. Linear skull fracture

E. Linear skull fracture

The etiologies of first-time seizure include neurologic insult (stroke, head trauma, CNS infection, birth asphyxia), underlying CNS abnormality (congenital, degenerative, CNS tumor, hydrocephalus, AVM), acute systemic metabolic disturbance (electrolyte disorders, alcohol withdrawal, cocaine toxicity, opioids, phenothiazine antiemetics), and idiopathic. Which of the following neurologic insult is the most common cause of first-onset seizure in pediatric population? ● A. CNS infection ● B. Birth asphyxia ● C. Febrile seizures ● D. Idiopathic ● E. Head trauma

C. Febrile seizures

An adult person comes in neurosurgical OPD with history of new-onset seizure in the absence of obvious cause like alcohol withdrawal. His/her MRI with and without contrast and systemic workup come out to be negative. How this adult should be followed up? ● A. No need for further follow-up in absence of seizure ● B. Follow-up after 6 months with repeat MRI which if found negative then no need for further follow-up ● C. Further follow-up if seizure occurs again ● D. Further follow-up after 6 months with repeat MRI, then at 1 year with repeat MRI, and possibly at 2 years to rule out tumor in brain ● E. Start antiepileptic medication and follow-up after 6 months

D. Further follow-up after 6 months with repeat MRI, then at 1 year with repeat MRI, and possibly at 2 years to rule out tumor in brain

Post-traumatic seizures (PTS) can occur in patients after head injury. These can adversely aﬀect the patient as it may elevate ICP, may adversely aﬀect blood pressure or oxygen delivery, and may worsen other injuries. Hence, antiseizure medication (levetiracetam, phenytoin, carbamazepine) should be started within 24 hours in patients meeting high-risk criteria for PTS. These high-risk criteria include the following except? ● A. ASD hematoma, EDH, ICH, or open depressed skull fracture with parenchymal injury ● B. No history of significant alcohol abuse ● C. Seizures within the first 24 hours after injury ● D. Penetrating head injury ● E. GCS less than 10 or cortical contusion

B. No history of significant alcohol abuse

Antiseizure medications (ASMs) are given for 1 week and then tapered as it prevents from early-onset post-traumatic seizure (within 7 days of injury). However, ASMs are continued for 6 to 12 months in patients after head injury in which of the following conditions? ● A. Penetrating head injury ● B. Development of late PTS (a seizure lasting more than 7 days following head injury) ● C. Prior seizure history ● D. Patients undergoing craniotomy ● E. All of the above

E. All of the above

Alcohol withdrawal seizure can occur within 7 to 30 hours of cessation or reduction of alcohol intake in habituated drinkers. These seizures are treated with diazepam or lorazepam if they continue for 3 to 4 minutes or phenytoin. What is the loading dose (mg/kg) of phenytoin for such patients? ● A. 16 ● B. 18 ● C. 20 ● D. 22 ● E. 24

B. 18

Diﬀerentials of nonepileptic seizures include psychologic disorders (anxiety, dissociative, psychonic, impulse control, attention deficit), cardiovascular disorders (syncope, cardiac ar- rythmias, TIAs), migraine syndrome, movement disorders (tremors, dyskinesias, tic), parasomnias with sleep-related disorders, and others like malingering or cognitive disorders. Following are features associated with nonepileptic seizure disorder except? ● A. Manifestations altered by distraction ● B. Intermittent arrhythmic and out-of-phase convulsive activity ● C. Nonfluctuating intensity and severity during seizure ● D. Bilateral motor activity with preserved consciousness ● E. Absence of stereotypy

C. Nonfluctuating intensity and severity during seizure

A febrile seizure is said to be a seizure in infants or children associated with fever with no defined cause and unaccompanied by acute neurologic illness. Which statement is most appropriate regarding management of these seizures? ● A. There are recommendations for prescribing antiseizure medication in these cases ● B. Phenobarbital can be used with no eﬀect on kid's IQ ● C. Valproate has no serious risks in the kids younger than 2 years ● D. Recurrence of febrile seizures in children can be reduced using diazepam 0.33 mg/kg per oral every 8 hours during a febrile episode and continuing until 24 hours after the fever subsides ● E. None of the above

D. Recurrence of febrile seizures in children can be reduced using diazepam 0.33 mg/kg per oral every 8 hours during a febrile episode and continuing until 24 hours after the fever subsides

Status epilepticus is a continuous seizure activity or more than equal to two sequential seizures without full recovery of consciousness between seizures for more than 30 minutes. The goal of treatment in such patients is rapid termination of clinical and electrical seizure activity as continuation of seizure activity for more than 30 minutes can cause permanent neurologic deficit. What is the most common cause of status epilepticus? ● A. Low level of prescribed ASM in a patient with a seizure disorder ● B. Remote symptomatic cause ● C. Stroke ● D. Metabolic disturbance ● E. Hypoxia

A. Low level of prescribed ASM in a patient with a seizure disorder

Treatment of status epilepticus is divided into four phases with phase 1 comprising from 0 to 5 minutes, phase 2 from 5 to 20 minutes, phase 3 from 20 to 40 minutes, and phase 4 from 40 to 60 minutes. First 0 to 5 minutes phase is called as stabilization phase. Following are the components of this phase except? ● A. ABC, that is, assessment and correction of airway, breathing, circulation with adequate oxygenation and arterial blood gases (ABG) monitoring ● B. 50 mL of dextrose 50 is given by IV push before giving 100 mg thiamine IV ● C. Baseline investigation is sent for with start of IV fluid and neurologic examination ● D. Initial additional measures include EEG monitoring if possible, head CT without contrast, correction of any suspected electrolyte imbalance, and CNS sampling for suspected CNS infection with start of antibiotics ● E. General medication for unknown suspected poisoning cases includes naloxone 0.4 mg IV for narcotics, bicarbonate to counter acidosis, and for neonates less than 2 years, pyridoxine 100 mg IV push is given

B. 50 mL of dextrose 50 is given by IV push before giving 100 mg thiamine IV

During the second phase of status epilepticus treatment, which last from 5 to 20 minutes, benzodiazepine is the drug of choice with IM midazolam or IV lorazepam or IV diazepam in dosage of 10 mg (for adult) or 0.1 mg/kg/dose or 0.15 to 0.2 mg/kg/dose given, respectively, once. If all the above meds are unavailable or contraindicated, then IV phenobarbital (15 mg/ kg/dose) or rectal diazepam (0.2 to 0.5 mg/kg) or intranasal midazolam or buccal midazolam can be given. What is the component of the second therapy phase which lasts from 20 to 40 minutes? ● A. IV phosphenytoin ● B. IV valproic acid 40 mg/kg/dose up to max 3,000 mg/dose ● C. IV levetiracetam 60 mg/kg/dose up to max 4,500 mg/dose ● D. IV phenobarbital 15 mg/kg/dose ● E. All of the above

E. All of the above

A 35-year-old young woman presents with left-sided facial pain without any numbness. She had several episodes in the last few months, and her pain triggers after eating, talking, and touching her cheek. MRI shows a vessel compressing cranial nerve V. Which vessel is most commonly involved? ● A. Superior cerebral artery ● B. AICA ● C. PICA ● D. PCA

A. Superior cerebral artery

A 25-year-old young male presented with painful vesicular eruption. It was in dermatomal distribution on one side of thorax. What is the eﬀective treatment for the pain of acute attack? ● A. Epidural ● B. Intercostal block ● C. Antiviral ● D. Both A and B

D. Both A and B

A 60-year-old female with history of breast cancer, with metastasis to bone and pain in right arm, reports 3 weeks of worsening of pain (burning in right hand—brachial plexus neuropathy). She was prescribed tricyclic antidepressant, use of which is limited by which of the following? ● A. Anticholinergic eﬀect ● B. Central eﬀects ● C. Limited pain relief ● D. All of the above

D. All of the above

Ramsay Hunt syndrome is characterized by which of the following triads? ● A. Contralateral facial paralysis, ear pain, and vesicle on face, ear, or in the ear ● B. Recurrent oral ulcers, genital ulcers, and iridocyclitis ● C. Sixth cranial n. palsy, persistent ear discharge, deep-seated retro-orbital pain ● D. Ipsilateral facial paralysis, ear pain, and vesicle on face, ear, or in the ear

D. Ipsilateral facial paralysis, ear pain, and vesicle on face, ear, or in the ear

Which of the following is correct regarding primary otalgia? ● A. More common in children ● B. Mostly unilateral ● C. Cold, air, and water most common triggers ● D. 75% associated with aural symptoms (hearing loss, tinnitus, and vertigo) ● E. All of the above

E. All of the above

A European solider presented to the emergency department after 24 hours of being shot, with classical triad of burning pain, autonomic dysfunction, and trophic change (complex re- gional pain syndrome) on his upper extremity. Which of the following statements is true? ● A. Medical therapy is suﬃcient ● B. Sympathetic block may not help ● C. Guanethidine commonly used intravenous injection ● D. No role of tricyclic antidepressants ● E. All of the above

C. Guanethidine commonly used intravenous injection

There are three major types of pain, namely, nociceptive pain (somatic and visceral), deaﬀerentation pain, and sympathetically maintained pain. Pain which is caused by lesion or compression of peripheral or central nervous system manifesting as sensory signs and symptoms is called as neuropathic pain. Following are peripheral neuropathic pain syndromes except? ● A. Acute and chronic inflammatory demyelinating polyneuropathy ● B. Entrapment neuropathies ● C. Cervical spondylotic myelopathy ● D. Post herpetic neuralgia ● E. Painful diabetic neuropathy

C. Cervical spondylotic myelopathy

Which of the following statements regarding medical treatment of neuropathic pain is true? ● A. Traditional treatment includes narcotic analgesic and tricyclic antidepressants (amitriptyline 75 mg daily) ● B. Gabapentin is eﬀective for post herpetic neuralgia, painful diabetic neuropathy, trigeminal neuralgia, postoperative state, spinal cord injury, cancer, MS, and migraine ● C. Lidocaine patch is applied for 12 hours/d over the most painful area ● D. Tramadol is a centrally acting analgesic which is eﬀective for these patients ● E. All of the above

E. All of the above

Possible pathways for facial pain include trigeminal nerve, facial nerve, and cranial nerve eight. Which of the following is a cause of cephalic neuralgia? ● A. Tic convulsif: otalgia and deep prosopalgia ● B. Diabetic neuritis of oculomotor nerve ● C. Nerve injury to superior or inferior alveolar nerves ● D. Sinusitis of frontal, maxillary, sphenoidal, or ethmoidal sinus ● E. All of the above

A. Tic convulsif: otalgia and deep prosopalgia

Primary otalgia is unilateral in most cases, and it is mostly triggered by cold air or water. It is associated with aural symptoms like hearing loss, vertigo, and tinnitus. Following is true regarding the management of primary otalgia except? ● A. An initial trial with medications used in trigeminal neuralgias like carbamazepine, phenytoin, and baclofen is the first line of defense ● B. Suboccipital exploration of the 7th cranial nerve or lower cranial nerve with MVD has no role in these cases ● C. Ultimate treatment is sectioning of nervus intermedius, the 9th and upper two fibers of 10th nerve, and a geniculate ganglionectomy ● D. Pain relief with nerve block of pharyngeal tonsils suggests glossopharyngeal neuralgia ● E. CT or MRI should be done in any case where no cause is found

B. Suboccipital exploration of the 7th cranial nerve or lower cranial nerve with MVD has no role in these cases

A woman 50 years of age presented with unilateral pain in forehead with tenderness on one side of forehead. The pain is temporarily relieved with nerve block in the region of orbital roof. This condition responds to gabapentin, pregabalin, or topical capsaicin. What is the most likely diagnosis in this case? ● A. Trigeminal neuralgia ● B. Migraine ● C. Supraorbital neuralgia ● D. Hemi crania continua ● E. Trochleitis

C. Supraorbital neuralgia

Herpes varicella zoster virus is responsible for painful vesicular cutaneous eruptions mostly in the area of thorax or in distribution of trigeminal neuralgia. If pain from this condition persists for more than 1 month, then this condition is called post herpetic neuralgia. The recommended medical treatment for this condition includes which of the following? ● A. Acyclovir 800 mg every 4 hours for 7 days ● B. Tricyclic antidepressants ● C. Gabapentin ● D. Lidocaine patch ● E. All of the above

E. All of the above

For post herpetic neuralgia, gabapentin is started at a dose of 300 mg once a day for first day, 300 mg twice a day on second day, and 300 mg thrice a day on third day. Which side eﬀect of gabapentin makes a surgeon to start it at night with slow increments of doses? ● A. Ataxia and fatigue ● B. Drowsiness ● C. Peripheral edema and confusion ● D. Depression ● E. All of the above

B. Drowsiness

Capsaicin patches or lidocaine patches are helpful in post herpetic neuralgia patients. Intrathecal steroid with methylprednisolone plus 3% lidocaine once every week for 4 weeks has shown good to excellent pain relief for up to 2 years. What is the surgical treatment that is uniformly successful in treating post herpetic neuralgias? ● A. Nerve block ● B. Neurectomies ● C. Cordotomy ● D. Rhizotomy ● E. None of the above

E. None of the above

Complex regional pain syndrome also known as causalgia is a disproportionate pain syndrome which can cause burning pain, autonomic dysfunction, or trophic changes. Which of the following is true regarding the cause of this syndrome? ● A. These are high velocity missile injuries (also known as CRPS type 2) ● B. Sympathetic dystrophy or causalgia minor is caused by nonpenetrating injury and is called as CRPS type 1 ● C. It can occur in postoperative patients following carpal tunnel surgery or surgery on lumbar or cervical spine ● D. It can occur because of direct injury to nerve or because of injury to surrounding soft tissue ● E. All of the above

E. All of the above

CRPS mostly aﬀects limbs with hand and foot involvement. Examination (in such patients is diﬃcult because of tenderness) may show vasodilatation, vasoconstriction, or trophic changes. Following are recommended treatment options in these patients except? ● A. Gabapentin ● B. Tricyclic antidepressants ● C. Sympathetic block ● D. Surgical sympathectomy ● E. Spinal cord stimulation

A. Gabapentin

A 16-year-old boy presented to the OPD for follow-up after 2 months of brachial plexus injury. Initially, his reflexes were absent. Which maneuver will help you to further support your finding? ● A. Jendrassick maneuver ● B. Hamlick maneuver ● C. tENS ● D. Stimulation

A. Jendrassick maneuver

A 35-year-old female is suﬀering from wrist drop after getting IM injection from a quack. Her tone is decreased and reflexes are absent. What kind of lesion is this? ● A. Upper motor neuron ● B. Mixed ● C. Myopathic ● D. Lower motor neuron

D. Lower motor neuron

There are three innervations of thumb. Pick up the correct sequence. ● A. Median nerve: opposition; ulnar nerve: adduction; and radial nerve: extension ● B. Median nerve: extension; ulnar nerve: abduction; and radial nerve: extension ● C. Median nerve: flexion; ulnar nerve: abduction; and radial nerve: extension ● D. None of the above

A. Median nerve: opposition; ulnar nerve: adduction; and radial nerve: extension

A patient suﬀered from foot drop after peroneal nerve injury during car accident. Which of the following statements is true regarding nerve injury? ● A. After 24 months of denervation, most muscle cannot recover useful function even with reinnervation ● B. The longer the distance from the injury site to the function unit to be innervated, the earlier the surgical intervention should be considered ● C. There is some degree of continuity in > 60% of nerve injury ● D. All of the above

D. All of the above

A 30-year-old weight lifter presented to the OPD with severe back ache which started right after his workout. Since then his right foot is numb, and he cannot move his lower limb without pain. MRI spine shows disk prolapse at L5–S1. Which is best way to test L5 clinically? ● A. Extensor hallucis longus ● B. Plantar flexion of big toe ● C. Tibialis anterior and extensor digitorum ● D. None of the above

A. Extensor hallucis longus

A young patient came to electrophysiology department for NCS/EMG for carpel tunnel syndrome. Despite complete absence of a median nerve evoked compound muscle action potential from the thenar eminence, the patient had significant preservation of function and minimal muscle atrophy. Activation of the ulnar nerve at both the wrist and elbow generated easily obtainable compound muscle action potentials from the thenar eminence with initial negative onset. This observed preservation of function and electrophysiologic responses are best explained by the presence of what? ● A. Martin Gruber anastomosis ● B. Medial and radial nerve anastomosis ● C. Radial and anterior interosseous nerve anastomosis ● D. Richie–Cannieu anastomosis

D. Richie–Cannieu anastomosis

A young male was referred to the OPD 6 weeks after a fall from the roof. Immediately after the fall he was not able to extend his wrist and unable to extend his fingers, along with weakness of brachioradialis muscle. The most likely radial nerve injury is where? ● A. Axillary area ● B. Spiral groove ● C. At wrist ● D. Forearm

B. Spiral groove

The peripheral nervous system consists of nerve fibers and axons that connect the central nervous system with motor and sensory, somatic, and visceral end organs. Following are included in peripheral nervous system except? ● A. Cranial nerves one and two ● B. Cranial nerves three to twelve ● C. Spinal nerves ● D. Nerves of extremities ● E. Cervical, brachial, and lumbosacral plexus

A. Cranial nerves one and two

Medical Research Council scale (MRC scale) is used for muscle strength grading. A patient presented to neurosurgical OPD with reduced power of right upper limb. The patient can move the limb actively with gravity eliminated. What is MRC grade of this patient? ● A. 1 ● B. 2 ● C. 3 ● D. 4 ● E. 5

B. 2

Muscle stretch reflex grading scale is used for deep tendon reflex. It typically ranges from 0 to 4 + . Which of the following statements is true regarding these reflexes? ● A. 2 + is normal while 3 + is less brisk than normal ● B. 0.5 + means reflex is elicitable only with reinforcement ● C. In the lower extremities, reinforcement consists of having patients clench their teeth ● D. In the upper extremities, reinforcement consists of hooking tip of fingers of right hand into left hand and pulling (Jendrassik maneuver) ● E. 5 + means hyperreflexic with clonus

B. 0.5 + means reflex is elicitable only with reinforcement

Possible etiologies of upper motor neuron (UMN) paralysis are stroke, spinal cord injury, and cervical spondylotic myelopathy while of lower motor neuron (LMN) paralysis are herniated intervertebral disk, nerve entrapment syndrome, polio, and progressive muscular atrophy. Which of the following statements is incorrect regarding diﬀerence between UMN paralysis and LMN paralysis? ● A. Muscle tone in UMN paralysis is initially flaccid then later spastic with clasping resistance while in LMN paralysis it is flaccid ● B. Tendon reflexes are hyperactive in UMN paralysis while these are absent in LMN paralysis ● C. Pathologic reflexes like Babinski and Hoﬀman are present in UMN paralysis while these are absent in LMN paralysis ● D. Atrophy does not occur in UMN paralysis while it does occur in LMN paralysis ● E. Spontaneous spasms may occur in UMN paralysis while in LMN paralysis fibrillations and fasciculations occur

D. Atrophy does not occur in UMN paralysis while it does occur in LMN paralysis

Fasciculations are coarse muscle contractions that are visible to the naked eye while fibrillations are not visible and require EMG to detect. Fasciculations most often occur in diseases involving anterior horn cells which includes which of the following? ● A. Amyotrophic lateral sclerosis ● B. Spinal muscular atrophy ● C. Polio ● D. Syringomyelia ● E. All of the above

E. All of the above

What is the common root value of deltoid muscle (abducts arm from 30 to 90), biceps brachii (flex forearm and supinate forearm), and brachialis (flex forearm)? ● A. C4, c5 ● B. C6, c7 ● C. C5, c6 ● D. C5, c6, c7 ● E. C4, c5, c6

C. C5, c6

C7, C8, and T1 roots collectively supply which muscle in upper extremities? ● A. Flexor pollicis longus (flex distal phalanx of thumb) ● B. Flexor digitorum profundus 1 and 2 (radial part) (flex distal phalanx of digits 2 and 3, also flexes wrist) ● C. Palmaris longus (wrist flexion) ● D. Flexor digitorum superficialis (flexion of middle phalanx of digits 2 to 5, flexes wrist) ● E. All of the above

E. All of the above

Flexion and extension of thumb occurs in the plane of palm while adduction and abduction occur in plane at right angle to the palm and opposition is bringing the thumb across the hand to the little finger. All of the following statements regarding innervation of thumb muscles is correct except? ● A. Median nerve innervates abductor pollicis brevis which causes abduction ● B. Ulnar nerve supplies adductor pollicis which causes adduction of thumb ● C. Radial nerve supplies extensor pollicis brevis and longus which cause extension of thumb ● D. Radial nerve also supplies opponens brevis which causes opposition ● E. Median nerve also supplies flexor pollicis brevis which causes flexion

D. Radial nerve also supplies opponens brevis which causes opposition

Which muscle is supplied by only L5 and S1 nerve roots? ● A. Extensor digitorum longus which causes extension of toes 2 to 5 and foot dorsiflexion ● B. Extensor hallucis longus which causes great toe extension and foot dorsiflexion ● C. Extensor digitorum brevis which causes extension of great toe and toes 2 to 5 ● D. Peroneus longus and brevis which cause planter flexion of pronated foot and eversion ● E. All of the above

E. All of the above

A nerve action potential (NAP) is produced if a normal healthy nerve fiber is stimulated with an electrical stimulus that has an amplitude and duration which exceed nerve fiber threshold. Presence of NAP (regardless of amplitude and la- tency) distal to lesion in continuity in the first few months after injury usually indicates that operative intervention will not be needed. What is the recommended timing to obtain NAP recording? ● A. 2–4 months for relatively focal contusions ● B. 4–5 months for stretch injuries such as brachial plexus ● C. Any time for partial injuries, entrapments, compressive lesions, and tumors ● D. Acutely to identify an area of nerve block ● E. All of the above are true

E. All of the above are true

Pick the false statement about entrapment neuropathy. ● A. It can be either an external force or a force by nearby structure ● B. One or repetitive insult ● C. Most common symptom is pain which frequently occurs at rest and is more severe at night ● D. Never consider the possibility of systemic disease ● E. None of the above

D. Never consider the possibility of systemic disease

A young female presented with pain in occiput with trigger point at the back of head. Any pressure on that reproduces the pain which radiates up along the vertex. She was diagnosed with occipital nerve entrapment. Treatment options for this patient are the following except? ● A. Idiopathic cases are self-limiting ● B. Temporary relief with occipital nerve block ● C. TENS provide no relief ● D. Collar for 2 weeks may help ● E. C and D

E. C and D

A coal mine worker presented with weak grip in his dominant hand. His hands get fatigued easily now with poorly localized paresthesia in index finger and thumb. What are the salient features to diﬀerentiate pronator teres syndrome from carpal tunnel syndrome (CTS)? ● A. Pain in palm rule out CTS ● B. Nocturnal exacerbation ● C. More common than CTS ● D. Involvement of ulnar nerve

A. Pain in palm rule out CTS

A young female with moderate obesity often visits OPD with tingling in the hand, on palmar side of thumb, index finger, middle finger, and radial half of ring finger. Now all of sudden it has become severe. Acute carpel tunnel syndrome is uncommon and is associated with which of the following? ● A. Exertion or trauma ● B. Median artery thrombosis ● C. Hematoma in transverse carpal ligament (TCL) ● D. All of the above

D. All of the above

A young female complains about tingling on palmer side of hand involving the thumb, index, middle, and half of ring finger, which wakes her up at night with painful numbness. Her grip gradually became weak. How much increase in carpel tunnel pressure produces sensory and motor dysfunction? ● A. 40 mmHg ● B. Less than 20 ● C. 30 ● D. 20–30

A. 40 mmHg

Regarding carpel tunnel syndrome, which statement is false? ● A. Most common entrapment neuropathy ● B. Females are 4 times more aﬀected ● C. Pain at palmar area is characteristic of pronator teres syndrome ● D. Neutral position splint relieves only 20% of patients ● E. Steroid injection may improve symptoms in 75% of patients

D. Neutral position splint relieves only 20% of patients

In carpel tunnel syndrome, surgical intervention is recommended for which of the following? ● A. Constant numbness ● B. Symptoms lasting > 1 year ● C. Sensory loss ● D. Thenar atrophy ● E. All of the above

E. All of the above

A 32-year-old male presented to the OPD with discomfort in little finger and medial half of ring finger, with slight hand weakness of 4 + power and mild muscle atrophy. What is the grade of ulnar nerve injury classification of this patient? ● A. Grade 1 ● B. Grade 2 ● C. Grade 3 ● D. Grade 4

B. Grade 2

Which one is a wrong statement about ulnar nerve? ● A. Elbow is most vulnerable for entrapment ● B. Tardy ulnar palsy is due to delayed onset following bony injury at the elbow ● C. NCS shows 4 m/sec faster than median nerve ● D. Early symptoms of ulnar nerve entrapment may be purely motor

D. Early symptoms of ulnar nerve entrapment may be purely motor

A manual labor worker with frequent use of pneumatic drill presented to the OPD with Froment's sign and claw hand deformity with no sensory loss at dorsum of hand. Most probably injury to which of the following has caused the lesion? ● A. Cubital tunnel ● B. Osborne ligament ● C. Guyon's canal ● D. Struther's arcade

C. Guyon's canal

How can radial nerve injury be distinguished from posterior cord injury? ● A. Involvement of all forearm extensor ● B. Patchy sensory loss in the distribution of radial nerve ● C. Sparing of deltoid and latissimus dorsi muscles ● D. Purely motor involvement

Injury to radial nerve proximal to radial groove of humerus will involve which of the following? ● A. Wrist extensors only ● B. Finger extensors only ● C. Triceps only ● D. Triceps, wrist, and finger extensors

A young male was hit by a car while he was riding a bike. He is unable to dorsiflex his ankle and shows weakness of lateral hamstring. Which nerve will be involved in this case? ● A. Common peroneal nerve ● B. Posterior tibial nerve ● C. Deep peroneal nerve ● D. Superficial peroneal nerve

A. Common peroneal nerve

A male presented to the OPD with complains of spontaneous rubbing or massaging of upper lateral aspect of thigh, along with burning dysesthesia and hyperpathia. How can meralgia paresthetica be diﬀerentiated from femoral neuropathy? ● A. Sensory changes on upper lateral aspect ● B. Purely sensory involvement ● C. No motor weakness ● D. None of the above

A. Sensory changes on upper lateral aspect

A patient presented with complaint of pain that is more severe at night, frequently at rest, often with retrograde radiation, causing more proximal lesion to be suspected. The patient also has tenderness at the site of pain. He is suspected to have entrapment neuropathy. What are most common associations with entrapment neuropathy? ● A. Diabetes mellitus and hypothyroidism ● B. Acromegaly and amyloidosis ● C. Carcinomatosis and polymyalgia rheumatica ● D. Rheumatoid arthritis ● E. All of the above

E. All of the above

What is the mechanism of injury in case of entrapment neuropathy? ● A. Chronic compression can cause axonolysis and Wallerian degeneration ● B. Acute compression can compromise axoplasmic flow, which can reduce membrane excitability ● C. There can be ischemia due to vascular supply compromise ● D. Venous stasis can produce ischemia which can lead to edema outside the axonal sheath, which can further exacerbate ischemia ● E. All of the above

E. All of the above

A patient after trauma to back of the head presents with headache mostly in back and myofascial spasm. Diagnosis of occipital nerve entrapment was made which occurs due to entrapment of the great occipital nerve, which is a sensory branch of C2. All of the following are surgical options for treatment of occipital neuralgia except? ● A. Decompression of C2 nerve root if compressed between c1 and c2 ● B. Occipital neurectomy ● C. Occipital nerve block with local anesthetic and steroids ● D. Occipital nerve stimulators ● E. Ganglionectomy

C. Occipital nerve block with local anesthetic and steroids

The median nerve is supplied by nerve roots C5 through T1 and it arises from medial and lateral cords of brachial plexus. In the cubital fossa, it passes just behind the lacertus fibrosus (bicipital aponeurosis) and enters the upper forearm between the two heads of pronator teres and supplies this muscle. Following statements regarding median nerve are correct except? ● A. Anterior interosseous nerve is purely motor branch of median nerve that supplies all but two muscles of finger and wrist flexion ● B. Near the wrist, it lies medial to the tendon of flexor carpi radialis and passes under transverse carpal ligament which also contains the tendons of FDP and FDS deep to the median nerve ● C. The second motor branch of the median nerve arises deep to the TCL and supplies LOAF muscles in the hand ● D. Palmaris cutaneous branch of the median nerve passes under the TCL and provides sensory innervation to the base of thenar eminence ● E. In the case of main trunk injury of the median nerve, a benediction hand is made when trying to make a fist (due to weakness of flexor digitorum 1 and 2)

A patient presents in neurosurgical emergency with complaint of diﬃculty in grasping small objects between the thumb and the index finger with no sensory loss. Pinch sign is positive. EMG assesses pronator quadratus and flexor pollicis longus. What is the diagnosis in this patient? ● A. Anterior interosseus neuropathy ● B. Pronator teres syndrome ● C. Carpal tunnel syndrome ● D. Injury to the main trunk of median nerve ● E. None of the above

A bulky female of 45 years of age presents in the OPD with complaints of pain in the palmer aspect of radial three and half fingers, dorsal side of these same fingers distal to PIP, and radial half of palm. The patient gets awakened at night by painful numbness in the hands. There is also hand weakness especially grip. Phalen's test (wrist flexion to 90 degrees angle reproduces the pain) is positive. What is the diagnosis in this patient? ● A. Cervical radiculopathy ● B. Pronator teres syndrome ● C. Carpal tunnel syndrome ● D. Reflex sympathetic atrophy ● E. De Quervain's syndrome

E. De Quervain's syndrome

In case of carpal tunnel syndrome patient, EMG shows sensory latencies of 3.7 to 4 and 4.1 to 5 in mild and moderate CTS, respectively, while motor latencies are 4.4 to 6.9 and 7 to 9.9 in mild and moderate CTS. Laboratory workup for these pa- tients include all of the following except? ● A. Thyroid hormone levels ● B. CBC ● C. There is no role of serum electrolytes in CTS patients ● D. HbA1c and glucose ● E. Vitamin B12, folate, and MMA

Which statement is true regarding treatment of carpal tunnel syndrome? ● A. NSAIDs, pyridoxine, and diuretics have strong evidence of good eﬃcacy with no obvious benefits of neutral position splint for 2 to 4 weeks or steroid injection of 10 to 25 mg hydrocortisone into carpal tunnel ● B. Surgery is recommended for symptoms less than 1 year duration with no sensory or motor symptoms ● C. Recurrent motor branch which innervates muscles of the thenar eminence is also called as million dollar nerve because its injury can lead to loss of thumb function and a possible malpractice lawsuit ● D. Kaplan's cardinal line runs from the base of the thumb web space to the hook of hamate which has no link with anastomosis between superficial and deep palmer arches ● E. Incision extends from the proximal wrist flexion crease up to Kaplan's cardinal line between digits 3 and 4

Ulnar nerve has components of c7, c8, and T1. It is second most common entrapment neuropathy after CTS. Potential sites of its compression are above the elbow by arcade of Struthers, at the elbow in retroepicondylar groove (ulnar groove), cubital tunnel, at the point of exit from flexor carpi ulnaris, and in the wrist at Guyon's canal. What are the motor findings in ulnar entrapment neuropathy? ● A. Wasting of interossei may occur ● B. Wartenberg's sign: patients may complaint that the little finger does not make it in when they reach into their pocket due to abducted little finger because of the weakness of the third palmer interosseous muscle ● C. Froment's prehensile thumb sign: grasping a piece of paper between the thumb and the extended index finger results in extension of the proximal phalanx of the thumb and flexion of the distal phalanx ● D. Claw deformity of hand: benediction sign when trying to open the fist from closed position, which is fingers 4 and 5 are hyperextended at MCP joints and flexed at the interphalangeal joint ● E. All of the above

What is the most common cause for peripheral neuropathy? ● A. Diabetes ● B. Arteritis ● C. Monoclonal gammopathy ● D. Acute idiopathic polyneuritis ● E. Sjogren syndrome

A. Diabetes

In critical illness polyneuropathy (CIP), CPK levels are normal or mildly elevated while EMG shows what? ● A. Decrease in amplitudes of compound muscle action potentials ● B. Increase in amplitudes of compound muscle action potentials ● C. Amplitudes of compound muscle action potentials remain unchanged ● D. Two spikes per second ● E. Nonlocalizing changes

A. Decrease in amplitudes of compound muscle action potentials

Idiopathic brachial plexitis usually starts to show some improvement by when? ● A. 3rd day ● B. 2nd week ● C. 4th week ● D. 2nd month ● E. 3rd month

C. 4th week

EMG changes can be appreciated in neuralgic amyotrophy of the upper extremity after how long? ● A. 24 hours ● B. 48 hours ● C. 72 hours ● D. 2 weeks ● E. 3 weeks

E. 3 weeks

A diabetic patient presents with abrupt onset of asymmetric pain in back, hip, buttocks, thigh, and leg usually deep aching/burning with superimposed lancinating paroxysms, severe at night. Progressive weakness in proximal and distal muscles was preceded by weight loss. Patellar reflexes are absent. Sensory loss is minimal. EMG findings consistent with demyelination are invariably accompanied by axonal degeneration, with involvement of paraspinals and no evidence of myopathy. These findings are consistent with what? ● A. Guillain Barre syndrome ● B. Bruns-Garland syndrome ● C. Ramsay Hunt syndrome ● D. Tolosa Hunt syndrome ● E. Brachial plexitis

B. Bruns-Garland syndrome

Which of the following is not associated with drug-induced neuropathy? ● A. Metronidazole ● B. Ciprofloxacin ● C. Phenytoin ● D. Thallium ● E. Cisplatin

What is the most common cause of femoral neuropathy? ● A. Diabetes ● B. Nerve entrapment ● C. Intra-abdominal tumor ● D. Femoral artery catheterization ● E. Retroperitoneal hematoma

Peripheral nerves regeneration rate in mm/d is about how much? ● A. 1 ● B. 2 ● C. 3 ● D. 5 ● E. 10

B. 2

According to the Sunderland system for classification of peripheral nerve injury what is neurotmesis? ● A. First degree ● B. Second degree ● C. Third degree ● D. Fourth degree ● E. Fifth degree

In which of the following complete interruption of axons and myelin sheaths takes place and Wallerian degeneration occurs whereas supporting structures including endoneurium remain intact? ● A. Neuropraxia ● B. Axonotmesis ● C. Neurotmesis ● D. Axonolysis ● E. First degree injury of Sunderland system

Lesions of peripheral nerves can produce somatic findings (combination of weakness, sensory disturbance, and reflex changes) and/or autonomic disturbances. Etiologies of nonentrapment peripheral neuropathies include which of the follow- ing? ● A. Diabetes ● B. Alcoholism ● C. Guillain-Barre syndrome ● D. Vasculitis/arteritis ● E. All of the above

E. All of the above

A patient admitted in ICU developed critical illness neuropathy (CIP) also known as neuropathy of critical illness or ICU neuropathy. For evaluation of the patient, blood work with fasting electrolytes, Hgb-A1C, CBC, ESR, CRP, vitamin B12, methylmalonic acid, serum protein electrophoresis with serum immunofixation, and electrodiagnostics (EMG/NCV) are done to rule out the cause of neuropathy. Following are the diagnostic criteria of CIP except? ● A. Presence of sepsis, multiorgan failure, septic inflammatory response syndrome, or respiratory failure ● B. Diﬃculty weaning from ventilator or extremity weakness ● C. EMG showing increased amplitudes of compound muscle action potentials ● D. Widespread muscle denervation potentials ● E. Normal or only mild increase in serum CPK levels

A male patient 30 years of age develops acute onset of intense pain in right upper extremity some days after a viral illness. Weakness of the same limb developed acutely after 2 weeks of pain onset. Arm movements exacerbated the pain. EMG/NCV showed the portion of brachial plexus involved. What is the most probable diagnosis in this patient? ● A. Parsonage Turner syndrome ● B. Immune-mediated brachial plexus neuropathy ● C. Neuralgic amyotrophy of the upper extremity ● D. Brachial plexitis ● E. All of the above

A patient presents to neurosurgical OPD with history of sudden onset of pain in lower extremity with weakness of quadriceps femoris muscle, SLR positive. EMG/NCV was done which showed patchy denervation involving at least two segmental levels with sparing of the paraspinal muscles. What is the most likely diagnosis in this case? ● A. L4 and L5 acute rupture of intervertebral disk ● B. Lumbosacral plexus neuropathy ● C. Rupture of AVM at L4 and L5 level ● D. Sudden compression of lumbosacral plexus ● E. None of the above

About 50% of patients with diabetes mellitus develop neuropathic symptoms or show slowing of nerve conduction velocities on electrodiagnostic studies. What is the most appropriate treatment of diabetic amyotrophy? ● A. Mexiletine started at 150 mg every 8 hours ● B. Gabapentin 1800 to 3600 mg every day ● C. Pregabalin up to a maximum of 100 mg TID ● D. Amitriptyline and fluphenazine ● E. All of the above

E. All of the above

Drugs that are most notorious for peripheral neuropathy include all of the following except? ● A. Thalidomide ● B. Macrolides ● C. Phenytoin ● D. Amitriptyline ● E. Metronidazole

AIDS patients can develop vague numbness, tingling, and sometimes painful feet. There may be reduction of light touch and vibratory sense. What is this condition called? ● A. Distal symmetric polyneuropathy ● B. Mononeuropathy multiplex ● C. Meralgia paresthetica ● D. Lumbar polyradiculopathy ● E. All of the above

A. Distal symmetric polyneuropathy

Following are most common nerves that can be involved in perioperative neuropathy except? ● A. Ulnar nerve ● B. Brachial plexus ● C. Median nerve ● D. Radial nerve ● E. Common peroneal nerve

Which of the following statements is incorrect regarding the Sunderland system of peripheral nerve injury classification? ● A. First degree is physiologic transection with local conduction block with usually complete recovery in 2 to 3 weeks and is also called neuropraxia ● B. Second degree is complete interruption of axons and myelin sheath, with supporting structures including endoneurium intact with recovery at 1 mm/d and is also called axonotmesis ● C. Third degree is endoneurium disrupted with intact epineurium and perineurium and is also called axonotmesis ● D. Fourth degree is interruption of all neural and supporting elements with only epineurium intact and is also called neurotmesis ● E. Fifth degree is complete transection with loss of continuity

A lesion in diencephalon will present with which nystagmus type? ● A. See-saw ● B. Convergence ● C. Retractorius ● D. Downbeat ● E. Upbeat

A. See-saw

Brun's nystagmus is associated with lesion in which of the following? ● A. Medial longitudinal fasciculus ● B. Lateral longitudinal fasciculus ● C. Pontomedullary junction ● D. Midbrain tegmentum ● E. Medulla

C. Pontomedullary junction

Low degree of papilledema with elevation of nasal margin, 360-degree disk swelling (circumferential halo) but no obscuration of major vessels on fundoscopy characterize which modified Frisen grade papilledema? ● A. Grade I ● B. Grade II ● C. Grade III ● D. Grade IV ● E. Grade V

B. Grade II

Homonymous superior quadrantanopsia in the contralateral visual field (“pie in the sky” deficit) is caused by lesion of which of the following? ● A. Optic tract ● B. Optic chiasma ● C. Meyer's loop ● D. Optic radiation ● E. Occipital cortex

C. Meyer's loop

Aneurysm of which of the following most commonly presents with third nerve palsy? ● A. A comm ● B. P comm ● C. ACA ● D. MCA trifurcation ● E. DACA

B. P comm

Which of the following is not a part of complete Horner syndrome? ● A. Miosis ● B. Ptosis ● C. Enophthalmos ● D. Hyperemia of internal ear ● E. Anhidrosis of half of face

D. Hyperemia of internal ear

What is the most common cause of bilateral internuclear ophthalmoplegia in young adults? ● A. Ocular tuberculosis ● B. Herpes zoster ● C. Congenital ● D. Multiple sclerosis ● E. Horner syndrome

D. Multiple sclerosis

Which of the following is not a cause of pupil-sparing oculomotor palsy? ● A. Uncal herniation ● B. Diabetic neuropathy ● C. Atherosclerosis ● D. Giant cell arteritis ● E. Myasthenia gravis

A. Uncal herniation

Which of the following nerves is not involved in superior orbital fissure syndrome? ● A. III ● B. IV ● C. V1 ● D. V2 ● E. VI

D. V2

Mastoiditis with involvement of petrous apex presenting with classic triad of abducens palsy, retro-orbital pain, and draining ear is which of the following? ● A. Raeder's neuralgia ● B. Tolosa Hunt syndrome ● C. Gradenigo's syndrome ● D. Orbital apex syndrome ● E. Inferior orbital fissure syndrome

C. Gradenigo's syndrome

Nystagmus is involuntary rhythmic oscillations of the eyes, usually conjugate, most common form of which is jerk nystagmus in which the direction of the nystagmus is defined by the direction of the fast component. A patient with structural lesion in the posterior fossa at the cervicomedullary junction including Chiari malformation type 1, basilar impression, basilar impression, or syringobulbia has which type of nystagmus? ● A. Upbeat nystagmus ● B. Vestibular nystagmus ● C. Downbeat nystagmus ● D. See-saw nystagmus ● E. Convergence nystagmus

C. Downbeat nystagmus

Papilledema also known as choked optic is optic disk swelling caused by increased intracranial pressure (ICP). Elevated ICP is transmitted through the subarachnoid space of the optic nerve sheath to the region of the optic disk which causes axoplasmic stasis and papilledema. How long it typically takes to develop papilledema after development of a sustained rise in ICP? ● A. 6 to 24 hours ● B. > 6 hours ● C. 12 to 24 hours ● D. 24 to 48 hours ● E. 48 to 72 hours

D. 24 to 48 hours

Papilledema can cause posterior globe flattening or elevation of the optic nerve head and dilatation of the optic nerve sheath (optic nerve sheath hydrops) which can be demonstrated on MRI or CT brain. Following statements regarding modified Frisen scale for papilledema on fundoscopic examination are correct except? ● A. Grade 0 is normal optic disk with minimal swelling of nasal margin, nerve fiber layer is clear, vessels are not obscured, and cup is also not obscured ● B. Grade 1 is minimal papilledema with 230-degree C-shaped swelling of nasal superior and inferior borders with normal temporal margin, and cup, if present, is maintained ● C. Grade 2 is low degree of papilledema with elevation of nasal margin, 360-degree disk swelling, and obscuration of major vessel starting from margins of disk ● D. Grade 3 is moderate degree of papilledema with elevation of entire disk, 360-degree disk swelling, obscuration of greater than or equal to 1 segment of major blood vessel at disk margin, and cup may be obscured ● E. Grade 4 is marked degree of papilledema with nerve fiber layer opaque, 360-degree disk swelling. Vessels obscured at disk margin, not completely obscured at disk surface

C. Grade 2 is low degree of papilledema with elevation of nasal margin, 360-degree disk swelling, and obscuration of major vessel starting from margins of disk

Which of the following is not an etiology of unilateral papilledema? ● A. Compressive lesions like intraorbital meningiomas, optic nerve sheath schwannoma, or optic nerve glioma ● B. Local inflammatory disorders ● C. Foster Kennedy syndrome ● D. Demyelinating disease such as multiple sclerosis ● E. Elevated ICP due to frontal lobe glioma

E. Elevated ICP due to frontal lobe glioma

The normal eye can detect stimuli as far as 60 degrees superiorly, 70 degrees inferiorly, 60 degrees nasally, and 100 degrees temporally. Confrontational method is a bed side test to detect any gross peripheral field defect while Humphrey visual field, octopus perimeters, and Goldmann perimetry are more accurate in detecting visual field defect. Which of the following statements is incorrect? ● A. Optic nerve and knee of Wilbrand injury on one side will cause junctional scotoma of ipsilateral eye (monocular blindness) while superior temporal quadrantanopsia of the same side eye ● B. Injury to optic chiasma causes bitemporal hemianopia ● C. Injury to right optic nerve or optic radiation causes left homonymous hemianopia with macular splitting of the left visual field ● D. Injury to right Meyer's loop causes homonymous left superior quadrantanopsia with macular sparing of left visual field ● E. Partial lesion of right optic radiation or visual cortex causes left homonymous hemianopsia with macular sparing of left visual field

A. Optic nerve and knee of Wilbrand injury on one side will cause junctional scotoma of ipsilateral eye (monocular blindness) while superior temporal quadrantanopsia of the same side eye

Macular splitting can occur both in lesions anterior and posterior to lateral geniculate body (LGB) while macular sparing tends to occur in lesions posterior to LGB. Homonymous hemianopsia with macular sparing usually occurs with the lesion of the optic radiation or infarcts of the primary visual cortex. What is the reason for macular sparing in lesions posterior to LGB? ● A. Input from macula is spread over large portion of optic radiation or primary visual cortex ● B. In some cases the occipital pole receives dual blood supply ● C. Occipital pole receives anomalous blood supply from the MCA ● D. Macula is resistant to lesion in this area ● E. A, B, and C

E. A, B, and C

A patient presents with ipsilateral central scotoma and contralateral superior temporal quadrantanopsia. This condition is also called as anterior chiasmal syndrome. This injury occurs due to which of the following? ● A. Lesion causing this deficit is close to anterior chiasma ● B. Ipsilateral optic nerve injury ● C. Damage to decussating knee of Wilbrand fibers ● D. All of the above ● E. Damage to only optic nerve close to chiasma

D. All of the above

Pupillodilator muscle fibers are sympathetically innervated and are arranged radially in iris while pupilloconstrictor muscles are parasympathetically innervated and are arranged as sphincter in the iris. Which statement is incorrect regarding the path of sympathetic fibers for pupilodilator muscles? ● A. First-order neuron arises in the anteromedial hypothalamus and descends uncrossed in the lateral tegmentum of the midbrain, pons, medulla, and the cervical spinal cord ● B. Second-order neurons arise from intermediolateral cell column of the spinal cord from C8 to T1 (ciliospinal center of Budge-Waller) which are preganglionic fibers. These exit at T1 and ascend in sympathetic chain ● C. Third-order neurons arise from superior cervical ganglion also known as postganglionic and ascend with common carotid artery ● D. Postganglionic fibers follow ICA through carotid canal, carotid sinus where they follow the 6th nerve and accompany V1 into orbit, then these enter ciliary ganglion and supply pupilodilator muscle ● E. Some fibers travel with ophthalmic artery to supply lacrimal gland and Muller's muscle

A. First-order neuron arises in the anteromedial hypothalamus and descends uncrossed in the lateral tegmentum of the midbrain, pons, medulla, and the cervical spinal cord

Pupillary light reflex involves cranial nerves 2, 3, neurons in Edinger-Westphal nucleus, and pretectal nuclear complex. Which of the following is true regarding pupillary light reflex? ● A. Cranial nerve 2 acts as aﬀerent pathway ● B. Cranial nerve 2 fibers of light reflex lead to pretectal nuclear complex of superior colliculus without synapsing in the lateral geniculate body ● C. Edinger-Westphal nucleus is a parasympathetic motor nuclei ● D. Eﬀerent is carried by the 3rd nerve ● E. All of the above

E. All of the above

Light near dissociation is pupillary constriction of convergence but absence of light response. This condition can occur with syphilis, Parinaud's syndrome, oculomotor neuropathy, or Adie's pupil. What is this condition called? ● A. Argyll Robertson pupil ● B. Marcus Gunn pupil ● C. Swinging flashlight pupil ● D. Anisocoria ● E. None of the above

A. Argyll Robertson pupil

For treatment of Meniere's disease, which procedure should be avoided? ● A. Middle ear perfusion with gentamicin ● B. Bilateral selective vestibular neurectomy ● C. Salt restriction ● D. Vestibular suppressants (e.g., Valium, meclizine) ● E. Endolymphatic shunting

B. Bilateral selective vestibular neurectomy

What is the most common cause of Bell's palsy? ● A. Diabetes ● B. Herpes zoster infection ● C. Trauma/basal skull fracture ● D. Tumor ● E. Idiopathic

E. Idiopathic

Which of the following diagnostic studies shows normal latencies in Meniere's disease? ● A. ENG with bithermal caloric stimulation ● B. Audiogram ● C. BAER ● D. CT/MRI ● E. VDRL

C. BAER

For Bell's palsy, if there is complete paralysis of facial nerve at onset, what proportion of patients will have an incomplete recovery despite management? ● A. 30% ● B. 50% ● C. 80% ● D. 90% ● E. 95%

B. 50%

A 70-year-old male presented in OPD with complaints of decreased hearing in right ear. On examination, he is having positive Rinne test (bilaterally AC > BC) and Weber is lateralizing to left ear. What type of hearing loss he is having? ● A. Conductive hearing loss in right ear ● B. Sensorineural hearing loss in right ear ● C. Combined conductive + SNHL ● D. Combined sensorineural hearing loss ● E. Both A and B

B. Sensorineural hearing loss in right ear

Regarding Bell's palsy if treatment is administered, which of the following is the most widely accepted for improvement? ● A. Tarsorrhaphy ● B. Direct brow lift ● C. Transposition of the temporalis muscle ● D. Corticosteroids ● E. Eye ointment and tear drops

D. Corticosteroids

Which of the following is a characteristic of grade IV Bell's palsy? ● A. Eye closure is incomplete ● B. Forehead motion is slight to moderate ● C. A slightly weak mouth movement is noted with maximal eﬀort ● D. Asymmetry is noted at rest ● E. Both A and B

E. Both A and B

Which of the following is not a typical feature of Ménière's disease? ● A. Insidious and progressive hearing loss ● B. Tinnitus ● C. Vertigo ● D. Otolithic crisis of Tumarkin ● E. Fluctuating deafness

A. Insidious and progressive hearing loss

Which of the following is recommended regarding steroid treatment in patients with Bell's palsy? ● A. Corticosteroids treatment in Bell's palsy is only recommended within the first 24 hours after symptom onset ● B. Prednisolone 25 mg per oral, twice daily for 10 days ● C. Antiviral treatment should be given as monotherapy prior to steroid treatment ● D. Antiviral combined with prednisolone ● E. Steroid therapy is given along with ceftriaxone

B. Prednisolone 25 mg per oral, twice daily for 10 days

A patient suﬀers injury to facial nerve during surgery for CP angle space occupying lesion (SOL). Primary repair is done. What is the expected failure rate in these cases? ● A. 13% ● B. 23% ● C. 33% ● D. 43% ● E. 53%

C. 33%

A patient presents with vertigo (sensation of movement usually spinning) in neurosurgical OPD. Following should be kept in mind as diﬀerentials for this disease except? ● A. Inner ear dysfunction which can be caused by labyrinthitis, Meneire's disease, trauma, drugs like aminoglycosides, syphilis, or vertebrobasilar insuﬃciency ● B. Vestibular nerve dysfunction which can include vestibular neuronitis, vestibular nerve compression by meningioma or schwannoma ● C. Brain stem dysfunction because of vertebrobasilar insuﬃciency, basilar artery migraine, or multiple sclerosis ● D. Dysfunction of dorsal proprioceptors as in osteoarthritis ● E. Vascular compression of vestibular nerve which can cause disabling positional vertigo

D. Dysfunction of dorsal proprioceptors as in osteoarthritis

Complete loss of vestibular function from one side is thought to produce transient vertigo due to mismatch of vestibular input from the two ears. Unilateral selective vestibular neurectomy (SVN) may convert the fluctuating or partial loss to a complete cessation of input and facilitate compensation. What surgical approach is adopted for SVN in case of Meniere's disease? ● A. Retrosigmoid approach ● B. Retrolabyrinthine approach ● C. Middle fossa approach ● D. Translabyrinthine approach ● E. All of the above

B. Retrolabyrinthine approach

Three surgical approaches for selective vestibular neurectomy (SVN) are retrolabyrinthine approach (which is anterior to sigmoid sinus and requires mastoidectomy with skeletonization of the semicircular canals and endolymphatic sac), retrosigmoid approach (posterior fossa suboccipital approach which is posterior to sigmoid sinus), and middle fossa approach (which is middle fossa extradural approach). Which approach is more suitable for complete section of vestibular nerve when other approaches fail? ● A. Retrolabyrinthine ● B. Retrosigmoid ● C. Translabyrinthine ● D. Middle fossa ● E. None of the above

D. Middle fossa

A neurosurgeon is going to do selective vestibular neurectomy (SVN). He/she should have following points in mind to have safe surgery and achieve surgical goals except? ● A. The vestibular nerve is the superior half of eighth nerve and is slightly more gray in color than the cochlear nerve (due to less myelin) ● B. Facial nerve is whiter than the eighth nerve complex, lies anterior and superior to eighth nerve complex. EMG monitoring is also recommended while direct stimulation during surgery can also confirm facial nerve identification ● C. Any artery present on eighth nerve bundle may be sacrificed ● D. If no cleavage plane is identified between vestibular and cochlear division then superior half of nerve bundle is divided ● E. The ELC lies midway between posterior edge of external auditory meatus and the sigmoid sinus

C. Any artery present on eighth nerve bundle may be sacrificed

A patient presents in OPD with attacks of violent vertigo, nausea, vomiting, diaphoresis, and tinnitus with progressive hearing loss which is fluctuating and low frequency. These attacks last for 5 to 30 minutes. What is most probable diagnosis in this case? ● A. Meniere's disease which is due to increased endolymphatic pressure ● B. Benign positional vertigo ● C. Vestibular neuronitis ● D. Vertebrobasilar insuﬃciency ● E. Disabling positional vertigo

A. Meniere's disease which is due to increased endolymphatic pressure

Diagnostic studies for Meniere's disease are electronystagmography with bithermal caloric stimulation abnormal and audiogram with low frequency hearing loss. Its medical management include reduced intake of salt, diuretics including acetazolamide, vestibular suppressants like diazepam, meclizine HCL, and vasodilators. What is the surgical treatment of Meniere's disease for serviceable hearing loss? ● A. Endolymphatic shunting procedure to mastoid cavity or subarachnoid space ● B. Direct application of corticosteroids to inner ear ● C. Nonselective vestibular ablation like surgical labyrinthectomy, middle ear perfusion with gentamicin ● D. Selective vestibular neurectomy ● E. Both A and D

E. Both A and D

Vestibular nerve section results in relief of vertigo in 90%, unchanged in 5%, and worse in 5%, while 9% incidence of facial paralysis in which 3% is permanent paralysis. On other hand selective vestibular nerve section results in improved hearing in 10%, unchanged in 28%, and worse in 48% while deafness in 14%. In postoperative failure, if ENG shows any vestibular nerve function on operated side then what is the next management step? ● A. Treatment only with medication ● B. Radiofrequency is done ● C. Chemotherapy is given ● D. Reoperation is considered ● E. All of the above

D. Reoperation is considered

Which of the following statements regarding path of facial nerve is incorrect? ● A. The seventh nerve exits the brain stem at the pontomedullary junction and enters the supero-anterior portion of internal auditory canal to enter the geniculate ganglion ● B. The first branch from geniculate ganglion is greater superficial petrosal nerve which passes to the pterygopalatine ganglion, innervates nasal mucosa, palatine mucosa, and lacrimal gland of eye ● C. The second branch is branch to stapedius muscle, and lesions proximal to this produce hyperacusis ● D. Third is chordae tympani which joins the facial nerve bringing sensations from posterior one-third of tongue, and along chordae tympani are fibers to submandibular and sublingual glands ● E. Facial nerve exits skull through stylomastoid foramen where it enters parotid gland and splits into five terminal branches

D. Third is chordae tympani which joins the facial nerve bringing sensations from posterior one-third of tongue, and along chordae tympani are fibers to submandibular and sublingual glands

What is the recommended management option for Bell's palsy? ● A. General supportive measure ● B. Eye protection and steroid prednisolone 25 mg per oral BID for 10 days ● C. Acyclovir ● D. Surgical decompression ● E. All of the above

B. Eye protection and steroid prednisolone 25 mg per oral BID for 10 days

In WHO classification of CNS tumors 2021, what does suﬃx NOS (not otherwise specified) indicate? ● A. Nonconcordance between histological and molecular types ● B. Noncanonical results ● C. New emerging tumor type ● D. Molecular testing not done or uncertain

A. Nonconcordance between histological and molecular types

Which of the following is no more a subtype of adult type diﬀuse gliomas? ● A. Astrocytoma IDH mutant, grade 2 ● B. Astrocytoma IDH wildtype, grade 2 ● C. Astrocytoma IDH mutant, grade 3 ● D. Astrocytoma IDH mutant, grade 4

B. Astrocytoma IDH wildtype, grade 2

According to WHO 2021 classification, which lineage of pituitary tumors secretes GH > PRL and a subunit? ● A. Mammosomatotroph pitNET ● B. Mature plurihormonal PIT1 lineage pitNET ● C. Immature PIT1 lineage pit NET ● D. Acidophil stem cell pitNET ● E. Mixed somatotroph–lactotroph PitNET

A. Mammosomatotroph pitNET

During the first 6 months of life, infratentorial tumors comprise what percentage of all intracranial tumors? ● A. 15% ● B. 27% ● C. 53% ● D. 74% ● E. 60%

B. 27%

A 45-year-old male presented with abulia, dementia, personality changes, apraxia, hemiparesis, and dysphasia. These suggest lesion localized in which area? ● A. Left frontal ● B. Right temporal ● C. Parietal ● D. Occipital ● E. Posterior fossa

A. Left frontal

A 40-year-old female presented with auditory and olfactory hallucinations, déja vu, memory impairment, and right superior quadrantanopsia. These suggest lesion localized in which area? ● A. Right frontal ● B. Left temporal ● C. Parietal ● D. Occipital ● E. Posterior fossa

B. Left temporal

What is the most common supratentorial tumor in children as well as in adults? ● A. Astrocytoma ● B. Medulloblastoma ● C. Craniopharyngioma ● D. Teratoma ● E. Choroid plexus papilloma

A. Astrocytoma

What is the most common brain tumor in neonates? ● A. Astrocytoma ● B. Medulloblastoma ● C. Craniopharyngioma ● D. Teratoma ● E. Choroid plexus papilloma

D. Teratoma

A middle-aged patient presented with first-time seizures. CT of brain showed brain tumor. Dexamethasone 10 mg was given intravenously. What will be the usual adult maintenance dose? ● A. 10 mg postoperatively 12 hourly ● B. 6 mg postoperatively 6 hourly ● C. 4 mg postoperatively 6 hourly ● D. 2 mg postoperatively 8 hourly ● E. Repeat 10 mg as needed

B. 6 mg postoperatively 6 hourly

An old patient undergoes excision of glioblastoma multiforme. What is the alkylating agent used in chemotherapy? ● A. Carmustine ● B. Lomustine ● C. Temozolamine ● D. Vincristine ● E. Procarbazine

C. Temozolamine

During histochemical analysis of a tumor specimen, which agent is used to stain myelin? ● A. Ziehl Neelsen ● B. Gram stain ● C. Reticulin stain ● D. Luxol fast blue ● E. Periodic acid Schiﬀ

D. Luxol fast blue

A patient presented with headache, seizures, and monoplegia. What is the tumor marker for meningioma? ● A. Glial fibrillary acidic protein ● B. Epithelial membrane antigen ● C. S-100 ● D. Chromogranin A ● E. Synaptophysin

B. Epithelial membrane antigen

Ki-67 is expressed in all phases of cell cycle except? ● A. G0 ● B. G1 ● C. G2 ● D. S1 ● E. S2

A. G0

Any metastatic small-cell tumors to the brain staining positive for neuroendocrine stains are almost all due to primaries in which of the following? ● A. Liver ● B. Bone ● C. Pancreas ● D. Lungs ● E. Prostate

D. Lungs

A patient presents in neurosurgical OPD with complaints of auditory and olfactory hallucinations, déjà vu, memory impairment, and contralateral superior quadrantanopsia. What is the most likely part of brain involved by brain tumor in this patient? ● A. Frontal lobe ● B. Parietal lobe ● C. Temporal lobe ● D. Occipital lobe ● E. Posterior fossa

C. Temporal lobe

Tumor headache is classically described as headache which is worse in morning, often exacerbated by coughing, straining, bending forward, and associated with nausea and vomiting with temporarily relieved by vomiting. Following are etiologies of tumor headache except? ● A. Increased ICP which may be due to tumor mass eﬀect, hydrocephalus, mass eﬀect from associated edema, or mass effect from associated hemorrhage ● B. Invasion or compression of brain sensitive structures like dura, vessels, periosteum, and cranial nerves with sensory functions ● C. Diﬃculty vision which may be because of diplopia due to direct compression of third, fourth, or fifth cranial nerves, abducens palsy from increased ICP ● D. Extreme hypotension resulting from increased ICP ● E. Psychogenic due to stress from loss of functional capacity

D. Extreme hypotension resulting from increased ICP

A pediatric patient presents in OPD with complaints of headache, nausea, vomiting, papilledema, vertigo, diplopia, ataxia of extremities, dysmetria, and intention tremors. What is the most likely location of this tumor within brain of this patient? ● A. Lesion of cerebellar vermis ● B. Lesion of cerebellar hemisphere ● C. Brain stem lesion ● D. Supratentorial lesion ● E. Lesion in cerebellopontine angle

B. Lesion of cerebellar hemisphere

A patient presents with posterior fossa tumor and the surgeon decides to place a VP shunt in this patient and operate after 2 weeks. Which of the following is a theoretical risk of using this approach? ● A. Placing shunt is a lifelong commitment whereas not all patients with hydrocephalus from posterior fossa tumor requires shunting ● B. Seeding of peritoneum with malignant tumor cells is possible, for example, with medulloblastoma ● C. Shunt infection can occur prior to surgery ● D. Definitive treatment is delayed and there are chances of upward transtentorial herniation ● E. All of the above

E. All of the above

An adult patient presents to you with MRI of brain showing multiple brain lesions, and round ring enhancing between gray and white matter with history of weight loss. Steroids are started in this patient with a dosage of 10 mg IV loading dose and 6 mg every 6 hours postoperatively or IV (pediatric loading dose is 0.5 to 1 mg/kg IV then 0.25 to 0.5 mg/kg/d divided every 6 hours). What is the most likely diagnosis in this patient? ● A. Cerebral abscesses ● B. Cerebral metastasis ● C. Multiple gliomas ● D. Neurofibromatosis type 1 ● E. None of the above

B. Cerebral metastasis

A patient with brain tumor with no previous history of seizure has to undergo craniotomy for excision of tumor. Prophylactic antiseizure medication (ASM) was started in this patient before surgery. Post surgery, the patient is stable with no distressing event. How long should ASM be continued in this patient? ● A. For 2 years ● B. Taper oﬀ ASM starting 2 weeks postoperatively ● C. Taper oﬀ ASM starting 1 week postoperatively ● D. Continue for 2 months ● E. There is no need to give postoperative ASM in this patient

C. Taper oﬀ ASM starting 1 week postoperatively

Procarbazine, CCNU (lomustine), and vincristine combination therapy is used as either an adjunct (following radiotherapy) or concomitant (simultaneously with radiotherapy) therapy in a number of tumors. What factors make these chemotherapeutic agents eﬃcient instead of presence of blood–brain barrier? ● A. Some CNS tumors may partially disrupt BBB ● B. Lipophilic agents, for example, nitrosoureas, may cross BBB more easily ● C. Selective intraarterial, for example, intracarotid or intervertebral, injection produces higher local concentration which increases penetration ● D. The BBB may be iatrogenically disrupted, for example, by giving mannitol or may be bypassed by intrathecal administration ● E. All of the above

E. All of the above

A patient underwent craniotomy for brain tumor. CT of brain without contrast was done after 6 to 12 hours of surgery to assess for acute complications like intraparenchymal hematoma, subdural or epidural hematoma, or for hydrocephalus or pneumocephalus. When should MRI with and without contrast be done in this patient? ● A. It should be delayed at least up to 30 days ● B. It should be done within 2 to 3 days ● C. It should be done after 6 months ● D. It should be done only if symptoms appear ● E. Both A and B

E. Both A and B

Stains for organisms include tissue gram stains for bacteria, periodic acid-Schiﬀ stain for fungi, Ziehl-Neelsen stain for acid fast bacilli like tuberculosis, and special stains like Luxol fast blue stains for demyelinating lesions which stain myelin sheath. Which statement is incorrect regarding immunohistochemical stain response of diﬀerent tumors? ● A. Oligodendroglioma stains positive for glial fibrillary acidic protein (GFAP) ● B. Ependymoma, choroid plexus, chordoma, and oligodendroglioma stains positive for S-100 ● C. Chordoma, craniopharyngioma, carcinoma, and PitNET stain positive for both CAM 5.2 (cytokeratins) and EMA epi thelial membrane protein ● D. Meningioma stains positive for EMA ● E. All of the above statements are correct

E. All of the above statements are correct

The gene locus for von Recklinhausen's disease is on which chromosome? ● A. 17 ● B. 22 ● C. 25 ● D. 19 ● E. Chromosome X

A. 17

A patient presented with six café au lait spots, each ≥ 15 mm in greatest diameter along with two painful neurofibromas. Lisch nodules in NF1 are present in which of the following? ● A. Nail bed ● B. Scalp hair ● C. Iris ● D. Proximal convoluted renal tubules ● E. Lips

C. Iris

A patient presented with bilateral hearing loss and seizures. Bilateral vestibular schwannomas and multiple meningiomas are hallmark of which of the following? ● A. NF1 ● B. NF2 ● C. Tuberous sclerosis ● D. Sturge Weber syndrome ● E. Neurocutaneous melanosis

B. NF2

Which of the following is a diagnostic criterion of schwannomatosis? ● A. Germline pathogenic NF2 mutation ● B. First-degree relative with NF2 mutation ● C. Pathologically proven meningioma and germline SMARCB1 pathogenic mutation ● D. Schwannomas in previous field of radiation therapy ● E. > 1 pathologically proven schwannoma/meningioma and LOH chromosome 22

C. Pathologically proven meningioma and germline SMARCB1 pathogenic mutation

Which of the following is true regarding schwannomatosis versus NF2? ● A. Most NF2 patients have chronic pain ● B. Life expectancy of schwannomatosis is worse than NF2 ● C. Surgery may occasionally be associated with worsening of global pain ● D. Pain is localized and associated with the mass

C. Surgery may occasionally be associated with worsening of global pain

Subependymal giant cell astrocytoma (SEGA) is associated with tuberous sclerosis (TSC). What is the most common location of SEGA in TSC? ● A. Posterior fossa ● B. Frontal lobe ● C. Sphenoid wing ● D. Foramen of Monro ● E. Torcular Herophili

D. Foramen of Monro

Which of the following is not a major diagnostic criterion for tuberous sclerosis (TSC)? ● A. > 3 hypomelanocytic macules > 5 mm diameter ● B. > 3 angiofibromas ● C. > 2 ungual fibroma ● D. > 4 pits in dental enamel ● E. > 2 angiomyolipomas

D. > 4 pits in dental enamel

Intracerebral calcifications are most frequently associated with which of the following? ● A. NF1 ● B. NF2 ● C. Tuberous sclerosis ● D. Neurocutaneous melanosis ● E. Von Ricklinghausen's disease

C. Tuberous sclerosis

Infantile myoclonus in tuberous sclerosis respond well to which of the following? ● A. Chemotherapy ● B. Radiotherapy ● C. Mannitol ● D. Steroids ● E. Hormone replacement therapy

D. Steroids

According to diagnostic criteria, von Hippel-Lindau (VHL) should be suspected in an individual who has all of the following, except? ● A. Retinal angioma at a young age ● B. Spinal or cerebellar hemangioblastoma ● C. Adrenal or extra-adrenal pheochromocytoma ● D. RCC if the individual is > 47 years old ● E. Endolymphatic sac tumors

D. RCC if the individual is > 47 years old

For a patient with or at risk of von Hippel-Lindau (VHL) what is the recommended guideline for retinal examination at 16 + years? ● A. Every 3 months ● B. Every 6 months ● C. Annually ● D. Every 5 years ● E. Every 10 years

B. Every 6 months

A patient presented with right hemiparesis, hemiatrophy, and homonymous hemianopia along with left port-wine facial nevus. What is the most probable diagnosis? ● A. NF1 ● B. NF2 ● C. Tuberous sclerosis ● D. Sturge Weber syndrome ● E. Neurocutaneous melanosis

D. Sturge Weber syndrome

Neuroectodermal defect during morphogenesis involving melanoblasts of skin and pia mater originating from neural crest cells leads to which of the following? ● A. NF1 ● B. NF2 ● C. Tuberous sclerosis ● D. Sturge Weber syndrome ● E. Neurocutaneous melanosis

E. Neurocutaneous melanosis

Which of the following is an autosomal dominant syndrome with benign colonic polyposis, colorectal carcinomas, and tumors of brain with medulloblastoma being the mostly associated brain tumor? ● A. Neurocutaneous melanosis ● B. Brain tumor polyposis syndrome 1 ● C. Brain tumor polyposis syndrome 2 ● D. Turcot syndrome ● E. Li Fraumeni's syndrome

C. Brain tumor polyposis syndrome 2

Li Fraumeni's syndrome is caused by germ line mutations in which of the following? ● A. TP 53 ● B. 17q ● C. 19p ● D. Chromosome X ● E. 22

A. TP 53

An 8-year-old patient presents in neurosurgical OPD with greater than or equal to six café au lait spots (hyperpigmented oval light brown skin macules which are present in greater than 99% cases of NF1), each greater than or equal to 5 mm in greatest dimension on his/her body, greater than or equal to two neurofibromas, and freckling (hyperpigmentation) in the axillary or intertriginous (inguinal) areas. You diagnosed this case as NF1 or Von Recklinghausen's disease. What other abnormalities can lead to diagnosis of this disease? ● A. Optic pathway glioma/pilocytic astrocytoma ● B. More than or equal to two Lisch nodules which are pigmented iris hamartomas that appear as translucent yellow/brown elevations that tend to become more numerous with age ● C. Distinctive osseous abnormalities like sphenoid dysplasia or thinning of long bone cortex with or without pseudarthrosis ● D. A first-degree relative (parent, sibling, or oﬀspring) with NF1 by above criteria ● E. All of the above

E. All of the above

A patient presents to your OPD as diagnosed case of NF1 with optic nerve gliomas. Focal, resectable, and symptomatic lesions should be surgically removed. MEK inhibitors are approved for use in 2 to 18 years of age for symptomatic, inoperable plexiform neurofibromas. How is the patient counselled regarding management of optic nerve gliomas? ● A. Unlike optic gliomas in the absence of NF1, these are rarely chiasmal, are often multiple, and hence have a better prognosis ● B. Most are nonprogressive and should be followed ophthalmologically and with serial imaging (MRI or CT) ● C. Surgical intervention probably does not alter visual impairment ● D. Surgery is reserved for special situations like large disfiguring tumor or pressure on adjacent structures ● E. All of the above

E. All of the above

A patient presents with seizures, multiple intradural spinal tumors, retinal hamartomas, and increased antigenic nerve growth factor. He/she is diagnosed as neurofibromatosis type 2 (NF2) which is also called as MISME syndrome (multiple inherited schwannomas, meningiomas, and ependymomas). All of the following are essential diagnostic criteria of NF2 except? ● A. Unilateral vestibular schwannoma and more than two plexiform neurofibromas ● B. Bilateral vestibular schwannomas on MRI or CT of brain ● C. A first-degree relative (sibling, parent, or oﬀspring) with NF2 and unilateral vestibular schwannomas or any two of meningioma, schwannoma, glioma, or posterior subcapsular opacity ● D. Unilateral vestibular schwannoma and any two of meningioma, schwannoma, glioma, or posterior subcapsular opacity ● E. Multiple meningiomas and unilateral vestibular schwannoma or any two of ependymoma, schwannoma, or posterior subcapsular opacities

A. Unilateral vestibular schwannoma and more than two plexiform neurofibromas

A patient presents with bilateral vestibular schwannomas with intact hearing in both ears. Surgery is planned and MRI of cervical spine is done prior to surgery to rule out any intraspinal tumor. Which statement regarding surgical treatment of this patient is not true? ● A. Smaller tumor is removed first and if hearing is serviceable in the operated side, then the other tumor should be operated ● B. If hearing is not preserved in the operated side, then the other tumor is followed with serial imaging as long as possible ● C. If there is need to operate on the second VS after failure to preserve hearing in the first VS surgery, then subtotal resection is done ● D. If bilateral VS are present and both are asymptomatic then there is no need to operate ● E. Stereotactic radiosurgery is an alternative treatment option

D. If bilateral VS are present and both are asymptomatic then there is no need to operate

Schwannomatosis typically presents with multiple schwannomas (spinal, cutaneous or cranial) and less frequently with meningiomas (cranial and spinal) with a propensity for spinal and paraspinal nerves and less frequently for meninges. What is the essential criterion for schwannomatosis? ● A. More than or equal to two schwannomas (nonintradermal and pathologically confirmed) and no bilateral VS on highquality MRI ● B. One schwannoma or meningioma and one aﬀected first degree relative ● C. Germline SMAECB1 and LZTR1 mutations ● D. Loss of heterozygosity/deletion of chromosome 22 and two diﬀerent NF2 mutations ● E. Both A and B

E. Both A and B

Schwannomatosis (SWN) presents with chronic pain and pain associated with mass. MRI of entire neuraxis and molecular genetic testing of the biopsied mass should be done. Which statement regarding treatment of SWN is correct? ● A. Surgery should be done for asymptomatic lesions as these can progress to malignant tumor ● B. Surgery for symptomatic schwannomas may relieve local pain ● C. Medical therapy with methotrexate is recommended ● D. There is no chance of worsening of global pain after surgery ● E. Life expectancy is reduced in these patients

B. Surgery for symptomatic schwannomas may relieve local pain

A patient presents with history of myoclonus which converted to generalized tonic clonic seizure. This patient also has facial adenomas which are present since childhood. CT of brain shows intracerebral calcifications primarily located in subependymal region in the walls of lateral ventricles, and these protrude into ventricles causing candle guttering. Treatment of this disease is observation, with surgery (transcallosal or ventriculoscopic approach) reserved for symptomatic lesions like subependymal giant cell astrocytoma (SEGA). What is a major criterion for diagnosis of this condition (tuberous sclerosis complex)? ● A. More than or equal to three hypomelanotic macules more than or equal to 5 mm diameter ● B. More than or equal to three angiofibromas or fibrous cephalic plaque ● C. More than or equal to two ungual fibroma ● D. Shagreen patch ● E. All of the above

E. All of the above

A 60-year-old patient presents to your OPD as diagnosed case of hemangioblastoma of cerebellum, renal cell carcinoma, and pheochromocytoma. Other tumors that are associated with this condition are endolymphatic sac tumors, pancreatic neuroendocrine tumors and cysts, broad ligament cystadenomas, and epididymal cystadenomas. Treatment options for this condition include belzutifan, surgical resection of symptomatic lesion, or stereotactic radiosurgery. What subtype von Hippel-Lindau (VHL) disease is this? ● A. Type 1 ● B. Type 2A ● C. Type 2B ● D. Type C ● E. Type D

C. Type 2B

Li Fraumeni's syndrome is associated with CNS tumors like primarily SHH activated medulloblastomas, IDH wild-type high-grade gliomas, and choroid plexus carcinomas. Essential criterion for this condition is pathogenic germline alteration in the TP 53 gene. What is the classic criterion for diagnosis of this disease? ● A. Sarcoma before the age of 45 years ● B. A first-degree relative with any cancer before the age of 45 years ● C. A first- or second-degree relative with any cancer before the age of 45 years, sarcoma at any age ● D. All of the above ● E. A first-degree relative with cancer after the age of 55 years with sarcoma as well

D. All of the above

A patient presents with history of seizures, left-sided body weakness, right-sided facial port-wine nevus in the distribution of 1st division of trigeminal nerve, and growth hormone deficiency. On investigations, this patient shows curvilinear double parallel lines or tram tracking on plain skull X-rays, increased intraocular pressure, and leptomeningeal angiomatosis. What is the most probable diagnosis in this case? ● A. Sturge-Weber syndrome ● B. Tuberous sclerosis ● C. Turcot syndrome ● D. Cowden syndrome ● E. Carney syndrome

A. Sturge-Weber syndrome

Diﬀuse gliomas are most common primary intra-axial brain tumors. Astrocytes typically appear in the white matter (e.g., centrum semiovale) and traverses through white matter tract. A patient presenting in OPD with history of seizure, his/her CT of brain showing low density lesion, MRI showing abnormal signal on T2WI with no enhancement, and no or very little mass eﬀect is diagnosed as astrocytoma, IDH mutant, grade 2 tumor. Following are the most common locations for this tumor except? ● A. Temporal lobe ● B. Occipital lobe ● C. Posterior frontal lobe ● D. Right anterior parietal lobe ● E. Left anterior parietal lobe

B. Occipital lobe

Gliomas can spread through CSF pathways (subarachnoid seeding) in 10 to 25% of high-grade gliomas and rarely may spread systemically. Gliomas can also spread from one part of brain to other part through white matter track. Which of the following statements is incorrect? ● A. Bilateral frontal lobe involvement occurs through genu or body of corpus callosum ● B. Bilateral occipital or parietal lobes involvement occurs through splenium of corpus callosum ● C. Midbrain involvement of tumor occurs through internal capsule ● D. Simultaneous frontal and temporal occurrence of tumor occurs through uncinate fasciculus ● E. Interthalamic adhesions cause bilateral thalamic gliomas

C. Midbrain involvement of tumor occurs through internal capsule

A patient presents in neurosurgical OPD with signs and symptoms of raised ICP. CT of brain was done which showed brain tumor in frontal lobe and solid enhancement on contrast. Microvascular proliferation was seen, and Ki-67 and MIB-1 index was found to be more than 7 to 9% in this tumor on biopsy, which showed it was astrocytoma, IDH mutant, grade 4 tumor. What is the essential diagnostic criterion for IDH mutant tumors? ● A. A diﬀusely infiltrating tumor with IDH1 codon 132 or IDH2 codon 172 missense mutation with loss of nuclear ATRX expression or ATRX mutation ● B. A diﬀusely infiltrating tumor with IDH1 codon 132 or IDH2 codon 172 missense mutation with exclusion of combined whole arm deletions of 1p and 19q ● C. Astrocytic diﬀerentiation by morphology with TP 53 mutation ● D. IDH mutation with Aa and B ● E. A and B

E. A and B

Significant prognosticators for gliomas are postoperative residual tumor volume and preoperative tumor size. Following factors show worse prognosis of glioma except? ● A. Age more than 40 years ● B. No neurologic deficit prior to surgery ● C. Largest tumor diameter of more than or equal to 6 cm ● D. Tumor crossing midline ● E. Subtotal resection

B. No neurologic deficit prior to surgery

A patient presents with seizure in your clinic for the first time. You advised plain CT of brain which showed hypodense to isodense lesion with calcifications. MRI of brain showed poor demarcation with T1 hypointense lesion and hyperintense on T2 with no enhancement on contrast. The essential diagnostic criteria for diagnosis of oligodendroglioma is a diffusely infiltrating tumor WITH IDH1 codon 132 or IDH2 codon 172 missense mutation WITH combined whole arm deletions of 1p and 19 q. Following features in this patient will characterize this lesion as grade 3 tumor except? ● A. Low mitotic activity ● B. High cellularity ● C. Marked cytological atypia ● D. Pathologically microvascular proliferation ● E. Necrosis with and without palisading

A. Low mitotic activity

A patient presents with signs of raised ICP in your clinic. You advised urgent MRI which showed lesion with large area of central necrosis and ring enhancement pattern on contrast. Most common locations of these lesions are temporal lobe, parietal lobe, frontal lobe, and occipital lobe in descending order. All of the following are included in essential diagnostic criteria for glioblastoma IDH wildtype except? ● A. DNA methylation of glioblastoma ● B. A diﬀusely infiltrating astrocytic glioma ● C. IDH wildtype and H3 wildtype ● D. Microvascular proliferation or necrosis ● E. TERT promoter mutation or EGFR gene amplification or +7/−10 chromosome copy number alteration

A. DNA methylation of glioblastoma

High grade diﬀuse gliomas (WHO grades 3 and 4) show improved prognosis with which of the following? ● A. Preoperative performance status, for example, Karnofsky score (KPS) more than 60 ● B. Old-aged patients ● C. Nonmethylation of MGMT promotor gene ● D. IDH1 mutation ● E. All of the above

D. IDH1 mutation

Gross total resection (GTR) is complete removal of enhancing portions of the tumor and any nonenhancing portion contained within, as verified by postoperative contrast imaging within 48 hours of surgery, or removal of high intensity lesion or very low intensity on T1 MRI in case of nonenhancing lesion. Surgical goals in case of diﬀuse glioma surgery are to obtain adequate tissue for histological and molecular study and cytoreduction or maximal safe resection with the goal of GTR when feasible. What are relative contraindications of surgery? ● A. Extensive dominant lobe high grade glioma ● B. Lesion with significant bilateral involvement ● C. Karnofsky score less than 70 ● D. Multicentric glioma ● E. All of the above

E. All of the above

A patient presents with diﬀuse astrocytoma grade 2 near eloquent area of brain. The extent of resection of this tumor can be increased or complication can be decreased by using which method? ● A. Image guidance or stereotactic radiosurgery ● B. Preoperative mapping like functional MRI or diﬀusion tensor imaging ● C. Intraoperative mapping with awake craniotomy ● D. Intraoperative dyes like indocyanine green and 5 amino levulinic acid ● E. All of the above

E. All of the above

Adjuvant radiotherapy or chemotherapy in patients with grade 2 glioma is recommended only if there is subtotal resection or early progression. Which of the following is true regarding Stupp regimen for diﬀuse glioma patients? ● A. It consists of radiotherapy (fractionated focal radiation) at a dose of 2 Gy per fraction once daily 5 days per week over a period of 6 weeks, for a total dose of 60 Gy ● B. A + Chemotherapy is given such as temozolomide at a dose of 75 g/m2/d, 7 days per week till end of radiotherapy ● C. B + After first 6 weeks of chemotherapy and radiotherapy 4 weeks gap is given and then chemotherapy is given in 6 cycles each consisting of 5 days of TMZ repeated every 28 days at a dose of 150 mg/m2/d for first cycle then 200 mg/m2/ d for rest of cycles ● D. C + Cytoreductive surgery ● E. All of the above

C. B + After first 6 weeks of chemotherapy and radiotherapy 4 weeks gap is given and then chemotherapy is given in 6 cycles each consisting of 5 days of TMZ repeated every 28 days at a dose of 150 mg/m2/d for first cycle then 200 mg/m2/ d for rest of cycles

Hypermethylation of the MGMT promoter gene (consists of > 30% methylation) causes reduced levels of MGMT activity. Tumors with reduced levels of MGMT activity are more susceptible to cytotoxic eﬀects of temozolomide. This leads to longer overall survival of these patients from 15.2 months in those without hypermethylation of MGMT to how long? ● A. 20.2 months ● B. 22.2 months ● C. 25.2 months ● D. 28.2 months ● E. 30.2 months

C. 25.2 months

Pseudoprogression is an increase in contrast enhancing areas on postoperative MRI after giving Stupp regimen due to radiation necrosis or tumor cells killed by chemotherapy. Hence, such enhancing areas within first 6 months of Stupp regimen are considered pseudoprogression. Which of the following studies is helpful with very low specificity and sensitivity to diﬀerentiate pseudoprogression from tumor recurrence? ● A. MR perfusion ● B. DWI with higher ADC ● C. MR spectroscopy ● D. PET ● E. All of the above

E. All of the above

A young child presents with seizure that is refractory to medication. Histopathology showed monomorphic cells with bland round, ovoid, or spindle nuclei. Which of the following is an essential diagnostic criterion for diﬀuse astrocytoma, MYB or MYBL1-altered tumors? ● A. Diﬀuse astrocytoma without histological features of anaplasia and no mutation in IDH or H3 genes and structural variant of MYB or MYBL1 ● B. A or diﬀuse astrocytoma without anaplasia and no mutations in IDH or H3 genes and DNA methylation profile aligned with diﬀuse astrocytoma, MYB or MYBL1 altered ● C. B + absence of LIG2 ● D. B + absence of MAP2 expression ● E. All of the above

B. A or diﬀuse astrocytoma without anaplasia and no mutations in IDH or H3 genes and DNA methylation profile aligned with diﬀuse astrocytoma, MYB or MYBL1 altered

A young patient presents with intractable partial epilepsy. On imaging there is nonenhancing cortical based lesion in temporal lobe with total cure with gross total excision. On histopathology, it shows glioma with diﬀuse growth architecture and a focal angiocentric pattern and monomorphic spindled cells with immunophenotypic and/or ultrastructural evidence of astrocytic and ependymal diﬀerentiation. What is the most likely diagnosis in this scenario? ● A. Polymorphous low-grade neuroepithelial tumor of the young ● B. Angiocentric glioma ● C. Diﬀuse glioma MYB- or MYBL1-altered ● D. Diﬀuse low grade glioma MAPK-pathway altered ● E. None of the above

B. Angiocentric glioma

A young patient presents with seizure with tumor present in right-sided temporal lobe on imaging. He is diagnosed with polymorphous low-grade neuroepithelial tumor of the young. Following are included in essential diagnostic criteria of this patient except? ● A. Diﬀuse growth pattern (at least regionally) and oligodendroglioma-like components (although these may be minor) ● B. A and few (if any) mitotic figure and regional CD34 expres- sion by tumor cells and by ramified neural cells in associated cerebral cortex and IDH wildtype ● C. B and unequivocal expression of BRAF p.V600E on immunohistochemical assessment ● D. C and conspicuous calcifications with absence of 1p/19q codeletion ● E. B and molecular diagnostic evidence of BRAF p.V600E mutations, FGFR2 or FGFR3 fusions, or potentially other MAPK pathway–driving genetic abnormalities

D. C and conspicuous calcifications with absence of 1p/19q codeletion

Appearance of diﬀuse low-grade glioma, MAPK pathway altered, may resemble pilocytic astrocytoma complete with cystic elements but these tumors tend to be more extensive on T2 flair images. Which of the following is an essential diagnostic criterion for these tumors? ● A. Diﬀuse glioma with absence or minimal mitotic activity and neither microvascular proliferation nor necrosis ● B. Genetic alteration in the MAPK pathway ● C. IDH wildtype and H3 wildtype ● D. Absence of homozygous deletion of CDKN2A ● E. All of the above

E. All of the above

Following are correct regarding diﬀuse midline glioma H3 K27 M altered in children except? ● A. Involvement tends to be in brainstem, pons, or bithalamic ● B. The patient presents with the triad of multiple cranial nerve palsies, long tract signs, and ataxia or obstructive hydrocephalus ● C. Biopsy lacks clinical utility in these patients ● D. Given the high rate of leptomeningeal spread, imaging of the entire neuraxis is recommended ● E. Pontine lesions enlarge pons which is low intensity on T1 and high intensity on T2

C. Biopsy lacks clinical utility in these patients

Which of the following is not included in essential diagnostic criteria of diﬀuse midline glioma, H3 K27M-altered? ● A. A diﬀuse glioma ● B. Results from molecular analysis enable diﬀerentiation of the H3.1 or H3.2 p.K28 (K27)-mutant subtype from the H3.3 p.K28 (K27) mutant type ● C. Loss of H3 p.K28me3 (K27me3) (immunohistochemistry) ● D. Midline location ● E. Presence of a pathogenic mutation or amplification of EGFR (for the EGFR mutant type)

B. Results from molecular analysis enable diﬀerentiation of the H3.1 or H3.2 p.K28 (K27)-mutant subtype from the H3.3 p.K28 (K27) mutant type

A young patient presents with seizures and focal neurologic deficit. Imaging shows hemispheric lesion that is typically contrast enhancing and multifocal. The tumor shows to be a cellular infiltrative tumor with mitotic activity AND H3.3 p.G35 R or p.G35 mutation AND hemispheric location AND methylation profile of diﬀuse hemispheric glioma, H3 G34-mutant. What is most likely diagnosis? ● A. Diﬀuse pediatric type high-grade glioma, H3 wildtype and IDH wildtype ● B. Infant type hemispheric glioma ● C. Diﬀuse hemispheric glioma, H3 G34-mutant ● D. Diﬀuse midline glioma ● E. None of the above

C. Diﬀuse hemispheric glioma, H3 G34-mutant

What is an essential diagnostic criterion for diﬀuse pediatric type high-grade glioma, H3 wildtype and IDH wildtype? ● A. A diﬀuse glioma with mitotic activity occurring in a child or a young adult ● B. Absence of mutation in IDH1 or IDH2 ● C. Few (if any) mitotic figures ● D. Absence of mutation in H3 genes ● E. Methylation profile aligned with pHGG RTK1, pHGG RTK2, or pHGG MYCN ● F. All of the above

F. All of the above

Following are features of infant type hemispheric glioma except? ● A. Presentation is usually in the first year of life ● B. It presents with agitation, lethargy, and enlarged head circumference ● C. Spine images are not recommended in addition to brain images ● D. Infants with high-grade glioma fare better than older children ● E. Histology consists of well-demarcated cellular tumors involving brain parenchyma and leptomeninges

C. Spine images are not recommended in addition to brain images

What is an essential diagnostic criterion of infant type hemispheric glioma? ● A. Cellular astrocytoma ● B. Presentation in early childhood ● C. Cerebral hemispheric lesions ● D. Presence of typical tyrosine kinase abnormality ● E. All of the above

E. All of the above

Pilocytic astrocytomas are the most common astrocytic tumors in children which are WHO grade 1 tumor. The most common location for these tumors is cerebellar hemisphere. These are usually cured with complete surgical resection. What is the recommendation for postoperative radiotherapy in such tumors? ● A. It is never indicated ● B. Indicated for all postoperative cases ● C. Indicated for nonresectable recurrence ● D. C + used for malignant recurrence of tumor ● E. Depends on histopathological report

D. C + used for malignant recurrence of tumor

All of the following are diﬀerentiating points between pilocytic astrocytoma and pilomyxoid astrocytoma except? ● A. Pilocytic astrocytoma (PCA) shows biphasic compact and loose growth patterns while pilomyxoid astrocytoma (PMA) shows monomorphic pattern with loose cells ● B. PCA shows high proliferative index, while PMA shows low cellularity ● C. PCA may or may not have Rosenthal fibers and eosinophilic granular bodies while PMA often lack Rosenthal fibers and eosinophilic bodies ● D. PMA carries a higher rate of recurrence, worse outcome, and more tendency for CSF dissemination as compared to PCA ● E. PCA most commonly occurs in cerebellar hemisphere, while PMA occurs mostly in hypothalamic and chiasmatic regions

B. PCA shows high proliferative index, while PMA shows low cellularity

Which of the following statements regarding location of pilocytic astrocytoma (PCA) is incorrect? ● A. 42% of PCAs are found in cerebellum ● B. Percentage of PCAs in cerebral hemisphere is 35% and these most commonly occur in children ● C. Optic gliomas and hypothalamic gliomas constitute 9% ● D. PCAs arising in the optic nerves are called optic gliomas, and they are the most common site in neurofibromatosis type 1 ● E. Percentage of brainstem PCAs is 9%

B. Percentage of PCAs in cerebral hemisphere is 35% and these most commonly occur in children

Feature of pilocytic astrocytoma (PCA) on CT or MRI is usually a contrast enhancing mural nodule (especially cerebellar PCAs) with cystic component with little or no surrounding edema. What is the recommended treatment option for PCA? ● A. Excision of nodule along with cyst wall in every case ● B. In tumors with false cyst where cyst wall also enhances, only cyst wall needs to be removed ● C. Both false cyst and mural nodule need to be removed ● D. Cyst wall does not need to be removed in any case ● E. Radiotherapy and chemotherapy are recommended in all postoperative cases

C. Both false cyst and mural nodule need to be removed

Pilocytic astrocytomas (PCAs) constitute 15% of primary brain tumors in patients aged less than 19 years and 27 to 40% of pediatric posterior fossa tumors. Optic pathway gliomas account for 2% of gliomas in adults and 7% in children. What is the most common location of optic pathway glioma in sporadic cases (other than in NF1)? ● A. Optic nerve ● B. Optic disc ● C. Optic chiasma ● D. Optic nerve with involvement of hypothalamus ● E. Both optic nerves simultaneously

C. Optic chiasma

A patient presents with nonspecific visual deficit without proptosis. This patient also has pituitary dysfunction and signs of hydrocephalus. MRI of brain shows mild to moderately enhancing chiasmal tumor. What is the recommended treatment for optic pathway glioma tumors? ● A. Chiasmal tumors are good for biopsy only with CSF shunting and removal of exophytic component ● B. Tumors involving optic nerve are removed using transcranial approach with excision of nerve from the globe all the way back to the chiasma ● C. Transorbital (Kronlein) approach is not appropriate for optic nerve tumors since the tumor may be left in the nerve stump ● D. Radiotherapy and chemotherapy are recommended for chiasmal tumors, multicentric tumors, postoperatively if tumor is found in chiasmal stump end of resected nerve, and malignant tumor ● E. All of the above

E. All of the above

A young patient presents with macrocephaly, signs of hydrocephalus, cachexia, and diabetes insipidus. On imaging, a tumor involving the third ventricle and chiasmal region is found. What is the most probable diagnosis in this case? ● A. Chiasmal glioma ● B. Third ventricular tumor ● C. Hypothalamic glioma ● D. Brainstem glioma ● E. Optic nerve glioma

C. Hypothalamic glioma

Upper brainstem tumors are usually lower grade tumors and present with cerebellar findings and hydrocephalus while lower brainstem tumors are usually higher grade with signs and symptoms of multiple lower cranial nerve deficits and long tract findings. Following are the patterns that can be identified on MRI in brainstem gliomas except? ● A. Diﬀuse gliomas, which are midline malignant, H3 k27 mutant, are seen mostly in pediatrics ● B. Cervicomedullary gliomas are mostly low-grade astrocytomas ● C. Ventrally exophytic brainstem gliomas are low-grade gliomas ● D. Focal gliomas are mostly limited to medulla and most are low-grade astrocytomas ● E. Dorsally exophytic gliomas may be low-grade gliomas

C. Ventrally exophytic brainstem gliomas are low-grade gliomas

Following statements are most appropriate regarding management of brainstem gliomas except? ● A. There is no role of biopsy in nonexophytic infiltrating brainstem gliomas ● B. Exophytic brainstem gliomas with broad attachment to the floor of 4th ventricle precludes complete excision, and survival can be increased in such cases with excision ● C. Surgery in malignant astrocytomas is without benefit; however, biopsy and shunting for hydrocephalus are recommended ● D. Tumors that demonstrate recurrent growth after resection remain histologically benign and are amenable to resection ● E. Radiations are given in 45 to 55 Gy dosage over a 6-week period, 5 days per week, and steroids added to radiotherapy can cause symptomatic improvement in 80% patients

A. There is no role of biopsy in nonexophytic infiltrating brainstem gliomas

A patient presents with diplopia, visual field deficits, nystagmus, Parinaud's syndrome, ataxia, and seizures along with hydrocephalus. MRI of brain showed a mass projecting dorsally from the quadrigeminal plate. Diagnosis of tectal glioma is made, which is also referred to as the smallest tumor in the body that can lead to death of the patient. What is the management option for these tumors? ● A. VP shunting ● B. Endoscopic third ventriculostomy ● C. Endoscopic aqueductoplasty ● D. Stereotactic radiosurgery ● E. All of the above

E. All of the above

A patient presents with history of seizures, and imaging showed a contrast enhancing lesion in temporal region with dural tail. On histopathology, astrocytes with pleomorphic tumor cells were found including large multinucleated cells, spindle cells, xanthomatous cells, and eosinophilic granular bodies. What is the most probable diagnosis in this patient? ● A. Meningioma ● B. Pleomorphic astrocytoma ● C. Pleomorphic xanthoastrocytoma ● D. Subependymal giant cell astrocytoma ● E. None of the above

C. Pleomorphic xanthoastrocytoma

A young patient presents with signs and symptoms of raised ICP. Imaging showed a tumor arising from the walls of lateral ventricles next to foramen of Monro. Gross total resection of the tumor was done with everolimus given to reduce the tumor size and progression. This tumor has strong association with tuberous sclerosis. What is the most likely diagnosis in this patient? ● A. Choroid plexus papilloma ● B. Intraventricular meningioma ● C. Subependymal giant cell astrocytoma ● D. Chordoid glioma ● E. Astroblastoma

C. Subependymal giant cell astrocytoma

An 11-year-old boy presents with history of seizures. His CT scan shows calcified lesion in temporal lobe. MRI confirms space occupying lesion (SOL) in temporal lobe. The patient underwent surgery and histopathological diagnosis came out to be ganglioglioma. What is the preferred treatment in case of recurrence? ● A. Re-resection ● B. Radiotherapy ● C. Chemotherapy ● D. Observation

A. Re-resection

Desmoplastic infantile astrocytoma may recur with anaplastic transformation how long after initial resection? ● A. 3 to 5 years ● B. 8 to 10 years ● C. 12 to15 years ● D. 18 to 20 years

B. 8 to 10 years

What is the most common location for dysembryoplastic neuroepithelial tumor (DNET)? ● A. Temporal lobe ● B. Parietal lobe ● C. Occipital lobe ● D. Basal ganglia ● E. Cerebellum

A. Temporal lobe

Which type of rosettes is demonstrated in rosette forming glioneural tumor? ● A. Homer Wright rosettes ● B. Flexner Wintersteiner rosettes ● C. Ependymal rosettes ● D. Neurocyte forming rosette

D. Neurocyte forming rosette

Which of the following is not an essential diagnostic criterion for central neurocytoma? ● A. Intraventricular localization ● B. ODG like monomorphic cells ● C. Synaptophysin expression ● D. No sign of malignancy ● E. Methylation profile

D. No sign of malignancy

Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease) is a cerebellar lesion with features of both a malformation and a low-grade neoplasm, and causes diﬀuse enlargement of cerebellar folia. It has the propensity to progress and recur after surgery and is strongly associated with which of the following? ● A. Down syndrome ● B. Klinefelter syndrome ● C. Neurofibromatosis I ● D. Li Fraumeni syndrome ● E. Cowden syndrome

E. Cowden syndrome

A patient 16 years of age presents with seizure. CT imaging of brain shows hypodense area in temporal lobe with calcifications. T1WI MRI showed hypointense area while T2WI showed hyperintense area. Maximal safe resection of this tumor was done. On histopathology an intra-axial low-grade glioneuronal tumor was noted in combination with neoplastic ganglion and glial cells. Which of the following statements is incorrect regarding ganglioglioma? ● A. The most common location of this tumor is temporal lobe ● B. These are benign tumors with 97% recurrence-free survival of 7.5 years ● C. Radiotherapy is recommended in all postoperative cases ● D. These are slow-growing well-diﬀerentiated tumor that primarily aﬀects children and young adults ● E. It is a WHO grade 1 tumor

C. Radiotherapy is recommended in all postoperative cases

On histopathology of a tumor, irregular groups of large, mature ganglion cells were noted WITH matrix resembling normal neuropil sometimes more coarsely fibrillar or vacuolated. Diagnosis of gangliocytoma was made. What is the most common location of this tumor? ● A. Frontal lobe ● B. Hypothalamus ● C. Third ventricle ● D. Temporal lobe ● E. Parietal lobe

D. Temporal lobe

Histopathology of a tumor showed biphasic morphology with dominant desmoplastic leptomeningeal component admixed with a neuroepithelial component containing astrocytic cells only or containing astrocytes or neuronal cells. This lesion is mostly found in cerebral hemisphere of infants often as large cystic lesions frequently attached to the dura. What is the mostly likely diagnosis in this patient? ● A. Gangliocytoma ● B. Desmoplastic infantile ganglioglioma ● C. Desmoplastic infantile astrocytoma ● D. Both A and C ● E. Dysembryoplastic neuroepithelial tumor

D. Both A and C

A patient 15 years old presents with seizures that are not controlled with medication. CT of brain shows hypodense lesion with distinct margins in the temporal lobe of brain. MRI shows hypointense lesion on T1WI and hyperintense lesion on T2WI with nodular enhancement. Histopathology shows cortical glioneuronal tumor with presence of specific glioneuronal elements. What is the WHO grade of dysembryoplastic neuroepithelial tumor (DNT)? ● A. Grade 1 ● B. Grade 2 ● C. Grade 3 ● D. Grade 4 ● E. Grade N/A

A. Grade 1

Following are essential criteria for diﬀuse glioneuronal tumor with oligodendroglioma-like features and nuclear clusters (DGONC) (WHO grade N/A) except? ● A. Methylation profile of DGONC ● B. Nuclear clusters of small to medium sized cells exhibiting oligodendroglioma like morphology ● C. Monosomy of chromosome 14 ● D. Strong expression of both OLIG2 and synaptophysin ● E. Absence of widespread GFAP expression

C. Monosomy of chromosome 14

A patient presents with dysplastic cerebellar gangliocytoma with gangliocytic lesion enlarging cerebellar folia and densely packed ganglion cells of various sizes and matrix resembling normal neuropil. What can be the likely presentation of such tumors? ● A. Signs and symptoms of cerebellar mass ● B. Dysmetria ● C. Hydrocephalus ● D. Cranial nerve deficits ● E. All of the above

E. All of the above

A patient presents in neurosurgical OPD with complaints of seizure and headache. Biopsy of the tumor showed multinodularity with absence of mitotic activity with tumor cell/matrix vacuolation with tumor cell immunoreactivity for synaptophysin. What is the most common location of multinodular and vacuolating neuronal tumor? ● A. Frontal lobe ● B. Temporal lobe ● C. Parietal lobe ● D. Spinal cord ● E. Occipital lobe

Temporal lobe

A patient presents in emergency with signs and symptoms of increased intracranial pressure. On CT of brain an intraventricular lesion was found with calcifications and cystic degeneration, heterogeneously isointense on T1 and hyperintense on T2. MR spectroscopy shows high glycine peak. Which of the following is a recommended treatment for central neurocytoma? ● A. Total resection is often curative with no need of postoperative radiotherapy ● B. Subtotal resection can be followed by stereotactic radiosurgery especially if MIB 1 labelling is more than 2 to 4% ● C. Chemotherapy with alkylating agents, platinum-based agents, or vincristine can be done for recurrent or inoperable tumors ● D. After treatment the patient should have long-term followup imaging surveillance for recurrence ● E. All of the above

E. All of the above

Extraventricular neurocytoma is a variant of central neurocytoma which can be found in all of the following locations except? ● A. Cerebral parenchyma ● B. Brainstem ● C. Thalamus ● D. Corpus callosum ● E. Cerebellum

D. Corpus callosum

Cerebellar liponeurocytoma is a WHO grade 2 tumor which was formerly called lipomatous medulloblastoma. Which of the following statements is correct regarding cerebellar neurocytoma prognosis? ● A. These tumors do not have a favorable prognosis ● B. Subtotal resection has a 15% recurrence rate ● C. Postoperative radiation has no eﬀect on progression-free survival ● D. All recurrences were restricted to the posterior fossa ● E. None of the above

D. All recurrences were restricted to the posterior fossa

Supratentorial ependymomas (WHO grades 2 and 3) are well-circumscribed supratentorial glioma with uniform cells having round nuclei that form focal pseudorosettes or ependymal rosettes embedded in a fibrillary matrix. These are thought to arise from radial glial cells. Which of the following statements is correct regarding treatment of these tumors? ● A. Postoperative radiotherapy has no role in these tumors ● B. WHO grade is the most important prognosticator of survival in adults and children ● C. The outcome is worse for infratentorial than for supratentorial ependymomas in adults ● D. Gross total removal is the most important prognosticator of overall survival in adults and children ● E. Gross total removal cannot be achieved in most of the cases

D. Gross total removal is the most important prognosticator of overall survival in adults and children

Supratentorial tumors with morphological and immunohistochemical features of ependymoma and ZFTA fusion positive are WHO grade 2 or 3 tumors. What is the most common location for these tumors? ● A. Frontal lobe ● B. Parietal lobe ● C. Both A and B ● D. Thalamus ● E. Hypothalamus/third ventricular region

C. Both A and B

Supratentorial tumor with morphological and immunohistochemical features of ependymoma with YAP1 fusion positive seem to be restricted to young children. What is the most common location for these tumors? ● A. Thalamus ● B. Hypothalamus ● C. Within or adjacent to the lateral ventricle ● D. Temporal lobe ● E. In spinal cord

C. Within or adjacent to the lateral ventricle

A patient presents in neurosurgical emergency with signs of raised intracranial pressure, diplopia, and facial weakness. MRI of brain was done and posterior fossa ependymoma (WHO grade 2 or 3) was diagnosed radiographically. Following statements are correct regarding this tumor except? ● A. These are mostly found in the floor of the fourth ventricle ● B. Ependymomas have the potential to spread through CSF to the entire neuraxis, a process known as seeding resulting in so-called drop mets ● C. There is no need to do imaging of the entire neuraxis ● D. Extension through the foramina of Luschka or Magendie may be seen ● E. The tumor enhances with IV contrast except for cystic portion or areas of necrosis

C. There is no need to do imaging of the entire neuraxis

Which of the following statements is incorrect regarding treatment of posterior fossa ependymoma? ● A. The goal of surgery is maximal possible resection of intracranial portion without causing neurological deficit, while gross total resection may not be possible in many cases because of extensive involvement of the floor of the fourth ventricle ● B. Lesions in the fourth ventricle region are approached via midline suboccipital craniectomy ● C. Traditional radiotherapy is 45 to 48 Gy to tumor bed, while for spinal drop mets, 30 Gy is given to the entire spinal axis with boost to regions showing drop mets ● D. There is very small risk of recurrence following subtotal resection ● E. 5-year survival in pediatric population is 20 to 30%, while it is 80% in adults

D. There is very small risk of recurrence following subtotal resection

Posterior fossa group A (PFA) ependymoma and posterior fossa group B (PFB) ependymoma are both WHO grade 2 or 3 tumors. PFA ependymomas most frequently occurs in preadolescents, while group B ependymoma mostly occurs in adults and adolescents. The prognosis of both these tumors depends on the extent of resection. Which statement is correct regarding location of these tumors? ● A. Both of these tumors arise from the floor of the fourth ventricle ● B. Group B tumors arise from the floor, while group A tumors arise from the roof or lateral wall ● C. Group B tumors arise from the roof or lateral wall, while group A tumors arise from the floor of the fourth ventricle ● D. Both tumors arise from the roof of the fourth ventricle ● E. Location of these tumors is variable in every patient

B. Group B tumors arise from the floor, while group A tumors arise from the roof or lateral wall

Spinal ependymomas are intramedullary spinal gliomas with vast majority of these tumors being grade 2 (grade 3 tumors being rare) and most common location at cervical and cervicothoracic levels. Which of the following are the imaging characteristics of these tumors? ● A. Hypointense on T1 and T2 with no enhancement and often eccentric and poorly demarcated ● B. Hypointense on T1 and hyperintense on T2 with contrast enhancement and often eccentric and poorly demarcated ● C. Hypointense on T1 and hyperintense on T2 with contrast enhancement, and more centrally located and more readily defined ● D. Hypointense on T1 and hypointense on T2 with no contrast enhancement, and more centrally located and more readily defined ● E. Hyperintense on T1 and T2 with contrast enhancement, and more centrally located and more readily defined

C. Hypointense on T1 and hyperintense on T2 with contrast enhancement, and more centrally located and more readily defined

Myxopapillary ependymomas are WHO grade 2 tumors that essentially only occur at conus medullaris or filum terminale and are diﬀerentiated from metastatic carcinoma, paragangliomas, schwannomas, chordomas, and myxoid chondrosarcomas by using GFAP staining. Complete excision of these tumors is often not possible due to invasion of the spinal cord parenchyma and/or CSF dissemination. What is the prognosis of these tumors? ● A. Poor prognosis with 10-year survival less than 50% ● B. Unfavorable prognosis with 10-year survival less than 90% ● C. Favorable prognosis with 10-year survival more than 50% in adults and children ● D. Poor prognosis with 10-year survival less than 10% ● E. Favorable prognosis with 10-year survival of more than 90%

E. Favorable prognosis with 10-year survival of more than 90%

Subependymomas are WHO grade 1 tumors which are mostly incidentally found as nonenhancing, well-demarcated intraventricular mass and are followed up with serial imaging if asymptomatic. What is the most common location for these tumors? ● A. Fourth ventricle ● B. Third ventricle ● C. Lateral ventricles ● D. Sylvian aqueduct ● E. Frontal horn of lateral ventricle

A. Fourth ventricle

Spinal ependymoma, MYCN-amplified, is a rare intramedullary tumor, mostly found in cervical and thoracic cord region and shows the same imaging characteristics as other ependymomas. These tumors have poor prognosis and early spread throughout the neuraxis is frequent. What is the recurrence rate of these tumors which are reported up till now? ● A. 100% ● B. 80% ● C. 70% ● D. 60% ● E. 50%

A. 100%

Ependymomas are believed to arise from bipolar progenitor cells that are thought to be a major source of neurons in the developing nervous system. What are these cells also called? ● A. Radial glial cells ● B. Progenitor cells ● C. Axial cells ● D. Circumferential cells ● E. CD4 positive cells

A. Radial glial cells

All of the following molecular genetic studies are done to subclassify ependymomas except? ● A. ZFTA fusion ● B. YAP1 fusion ● C. P65 ● D. L1CAM ● E. BRAF

E. BRAF

Posterior fossa group B ependymomas are mostly WHO grade II/III tumors but have a worse prognosis as they can often be not removed completely due to their propensity to invade which of the following? ● A. Cerebral cortex ● B. Basal cranial nerves ● C. Obex ● D. Mid brain ● E. White matter tracts

C. Obex

Ependymomas rank 2nd only to medulloblastomas in radiosensitivity. Radiotherapy is administered after surgical excision. What is the dose to tumor bed for cranial lesions? ● A. 30 Gy ● B. 35 to 38 Gy ● C. 3D conformal XRT with 59.4 Gy to tumor bed + 1 cm margin ● D. 62 Gy ● E. 65 Gy

C. 3D conformal XRT with 59.4 Gy to tumor bed + 1 cm margin

A young child was operated on for ependymoma. After 2 weeks, CSF cytology was performed that showed evidence of drop mets. What will be the radiotherapy dose to the entire spine in this case? ● A. 30 Gy ● B. 35 to 38 Gy ● C. 45 to 48 Gy ● D. 62 Gy ● E. 65 Gy

A. 30 Gy

Which of the followings is a distinctive, WHO grade 2 ependymoma variant that essentially only occurs in the filum terminale with the histological features of papillary, with microcystic vacuoles and mucosubstance? ● A. Clear cell ● B. Tanycytic ● C. Myxopapillary ● D. RELA fusion positive ● E. Anaplastic

C. Myxopapillary

When should the first follow-up image be obtained after initial discovery of subependymoma to rule out rapid growth? ● A. 1 month ● B. 3 months ● C. 6 months ● D. 1 year ● E. 5 years

B. 3 months

A 2-year-old patient presents with nausea, vomiting, macrocephaly, and seizures. Imaging showed iso- to hyperdense lesion on CT of brain in the ventricle which is hypointense on T1WI and hyperintense on T2WI multilobulated intraventricular mass classically with projecting fronds and densely enhancing. What are the most common locations of this tumor in descending order? ● A. Third ventricle, then fourth ventricle, then lateral ventricles ● B. Lateral ventricles, then third ventricle, then fourth ventricle ● C. Lateral ventricles, then fourth ventricle, then third ventricle ● D. Third ventricle, then lateral ventricle, then fourth ventricle ● E. None of the above

C. Lateral ventricles, then fourth ventricle, then third ventricle

Atypical choroid plexus papillomas are WHO grade 2 tumors which are located intraventricularly and demonstrate choroid plexus diﬀerentiation by histopathological and immunophenotypic features and are characterized by absence of criteria qualifying for diagnosis of choroid plexus carcinoma and which of the following? ● A. More than or equal to 1 mitosis/HPF of 0.23 mm2 ● B. More than or equal to 2 mitosis/HPF of 0.23 mm2 ● C. More than or equal to 3 mitosis/HPF of 0.23 mm2 ● D. More than or equal to 4 mitosis/HPF of 0.23 mm2 ● E. More than or equal to 5 mitosis/HPF of 0.23 mm2

B. More than or equal to 2 mitosis/HPF of 0.23 mm2

Choroid plexus carcinoma is a WHO grade 3 tumor that invades the adjacent brain and primarily occurs in the lateral ventricles of children. These tumors are prone to metastasize through CSF. What is the percentage of these tumors metastasized thorough CSF at the time of diagnosis? ● A. 41% ● B. 31% ● C. 51% ● D. 21% ● E. 11%

D. 21%

Following are the histopathological features of choroid plexus carcinoma except? ● A. Increased cellularity ● B. Nuclear pleomorphism ● C. Blurring of the papillary pattern with poorly structured sheets of tumor cells ● D. Mitoses usually more than or equal to 2 mitoses/10 HPF of 0.23 mm2 ● E. Necrotic areas

D. Mitoses usually more than or equal to 2 mitoses/10 HPF of 0.23 mm2

Imaging of the entire neuraxis is recommended in choroid plexus carcinoma due to high incidence of drop mets. Which of the following are features of choroid plexus carcinoma on MRI? ● A. Heterogenous signals on T1 and T2 with irregular enhancing margins suggesting subependymal invasion ● B. Edema in adjacent brain ● C. Vascular flow voids in 55% of images ● D. Often hydrocephalus is evident ● E. All of the above

E. All of the above

Which of the following statements is correct regarding treatment of choroid plexus carcinoma? ● A. There is no benefit of surgery in malignant choroid plexus tumors ● B. Chemotherapy benefits all of these patients ● C. Radiotherapy was not shown to improve survival in patients undergoing surgery ● D. Adjuvant chemotherapy should be given to all the patients ● E. All of the above

C. Radiotherapy was not shown to improve survival in patients undergoing surgery

Atypical choroid plexus papillomas are more likely to recur than their grade 1 counterpart. What is the median age of diagnosis of these tumors? ● A. 6 to 8 months ● B. 8 to 10 months ● C. 10 to 12 months ● D. 12 to 14 months ● E. 14 to 16 months

C. 10 to 12 months

Which statement is incorrect regarding treatment and prognosis of grade 1 choroid plexus papilloma? ● A. Choroid plexus papillomas are often cured surgically with total removal ● B. The operation may be diﬃcult because of fragility of the tumor and bleeding from the choroidal arteries ● C. Persistence with second or even third operation is recommended as 5-year survival rate of 84 to 97% can be achieved ● D. Postoperative subdural collections after transcortical tumor excision may occur because of persistent ventriculosubdural fistula which may require subdural peritoneal shunt ● E. Malignant degeneration is common with role of chemotherapy and radiation for these tumors

E. Malignant degeneration is common with role of chemotherapy and radiation for these tumors

What is the common location of choroid plexus papilloma? ● A. Usually infratentorial in adults ● B. A + Supratentorial in children ● C. Supratentorial in adults ● D. C + Infratentorial in children ● E. Equally present supratentorially and infratentorially in adults and children

A. Usually infratentorial in adults

Hydrocephalus in choroid plexus papilloma may result from which of the following? ● A. Overproduction of CSF ● B. Obstruction of CSF outflow ● C. Communicating hydrocephalus due to CSF-borne particulates ● D. Does not occur in choroid plexus papilloma ● E. A, B, and C

E. A, B, and C

A 2-year-old child presented with signs of raised ICP and CT showed hydrocephalus and a densely enhancing multilobulated mass with projecting fronds, within left lateral ventricle. What is the most probable diagnosis? ● A. Ependymoma ● B. Medulloblastoma ● C. Astroblastoma ● D. Choroid plexus papilloma ● E. Ganglioglioma

D. Choroid plexus papilloma

What is the usual extraventricular location of choroid plexus papilloma? ● A. Middle fossa ● B. Cribriform plate ● C. CP angle ● D. Ambient cistern ● E. Suprasellar region

C. CP angle

What is the treatment of postoperative ventriculo-subdural collection in choroid plexus papilloma (CPP) surgery? ● A. Craniotomy and evacuation ● B. Subdural drain ● C. Subdural–peritoneal shunting ● D. Subdural–subarachnoid shunting

C. Subdural–peritoneal shunting

About 40% of choroid plexus carcinomas are associated with which syndrome? ● A. Cowden syndrome ● B. von Hippel Lindau syndrome ● C. Li Fraumeni syndrome ● D. Turcot syndrome ● E. Gardner syndrome

C. Li Fraumeni syndrome

Absence of which mutation is associated with a good prognosis in choroid plexus carcinoma? ● A. TP53 ● B. BRAF ● C. ATRX ● D. PTEN ● E. EGFR

A. TP53

A 4-year-old patient presents with history of nausea, vomiting, headache, lethargy, and irritability which developed during the course of 6 to 10 weeks. CT of brain showed a hyperdense contrast-enhancing mass in the posterior fossa with calcifications while MRI showed hypo- to isointense lesion on T1, heterogenous lesion on T2. A diagnosis of medulloblastoma (grade 4) tumor is made. Where does this tumor arise in the posterior fossa most commonly? ● A. Floor of the fourth ventricle near fastigium ● B. From the roof of the fourth ventricle ● C. In the cerebellum, they arise in the vermis near the apex of the roof of the fourth ventricle ● D. In the cerebellum, these arise from cerebellar hemisphere ● E. From the floor of the fourth ventricle near the foramen of Magendie

C. In the cerebellum, they arise in the vermis near the apex of the roof of the fourth ventricle

Medulloblastomas which were formerly called as primitive neuroectodermal tumors (PNET) are now considered under umbrella term embryonal tumors and account for 68% of embryonal tumors. About 10 to 35% of these tumors are seeded to spinal canal at the time of diagnosis and warrants imaging of the entire neuraxis. Which statement is correct regarding treatment of these tumors? ● A. Invasion or attachment to the floor of the fourth ventricle at the brainstem in the region of facial colliculus often limits its excision ● B. Opening of foramen magnum with removal of C1 and C2 arches is often required for midline cerebellar medulloblastomas ● C. 30 to 40% of children require permanent VP shunt postoperatively ● D. Postoperative radiotherapy (54 gray to primary tumor and 18 gray to craniospinal axis) and chemotherapy are needed in almost all patients ● E. All of the above

E. All of the above

Which statement is incorrect regarding poor prognosticators of medulloblastoma? ● A. Poor Karnofsky performance scale score ● B. Inability to perform gross total resection ● C. MDB classification with SHH activated, TP53 wild type with classic histology ● D. Disseminated disease at the time of diagnosis ● E. Younger age of patient for some cell types (< 3 years)

C. MDB classification with SHH activated, TP53 wild type with classic histology

Which of the following is the most common histologic variant of medulloblastoma which constitutes 72% of medulloblastomas, is predominant in children, is found in all four molecular medulloblastomas, is composed of densely packed undiﬀerentiated small round cells with mild-to-moderate nuclear pleomorphism, and has high mitotic count? ● A. Classic type ● B. Desmoplastic/nodular ● C. MDB with extensive nodularity ● D. Large cell/anaplastic MDB ● E. Both A and B

A. Classic type

A patient is found to have medulloblastoma (MDB) around the foramen of Luschka, arising from the brain stem on imaging. These MDBs account for 15% of adults MDBs with male to female ratio of 1:2 with predominant histologic variant of classic type. In spite of being grade 4, the prognosis for children is excellent with surgery and adjuvant therapy in contrast to poor prognosis in adults. Which molecular type of MDB is this? ● A. WNT activated ● B. SHH activated TP53 wild type ● C. SHH activated TP53 mutant ● D. MDB non-WNT/non-SHH ● E. Group 4 MDB

B. SHH activated TP53 wild type

Putative cell of origin of SHH activated, TP53 wild type MDB is cerebellar granule neuron cell precursors of the external granule cell layer and cochlear nucleus. What is the predominant histologic variant in SHH activated, TP53 wild type MDB? ● A. Extensive nodularity ● B. Classic ● C. Large cell/anaplastic ● D. Desmoplastic/nodular ● E. Both A and B

D. Desmoplastic/nodular

SHH activated, TP 53 mutant MDB is mostly a high-risk tumor. What is the most common histologic variant in these tumors? ● A. Extensive nodularity ● B. Classic ● C. Large cell/anaplastic ● D. Desmoplastic/nodular ● E. Both A and B

C. Large cell/anaplastic

Atypical teratoid or rhabdoid tumors are WHO grade 4 embryonal tumors that occur primarily in infants and children. Where in the CNS this tumor can be found? ● A. Cerebral hemispheres ● B. Suprasellar region ● C. Cerebellar hemispheres ● D. Brainstem ● E. Anywhere along the neuraxis

E. Anywhere along the neuraxis

Cribriform neuroepithelial tumors are embryonal tumors that are highly cellular and characterized by presence of cribriform strands and ribbons. What is the location of these tumors? ● A. Brainstem ● B. Cerebellum ● C. Cerebellopontine angle ● D. Near any of the ventricles (third, fourth, or lateral) ● E. Anywhere along the neuraxis

D. Near any of the ventricles (third, fourth, or lateral)

Embryonal tumor with multilayer rosettes is a WHO grade 4 aggressive tumor with mean overall survival of 12 months. This tumor is most commonly found in cerebral hemispheres. Which age group does this tumor aﬀects? ● A. 5 to 10 years ● B. 10 to 14 years ● C. Less than 4 years ● D. After 50 years ● E. None of the above

C. Less than 4 years

A child presented with obstructive hydrocephalus. Which of the following is false regarding medulloblastoma? ● A. CT: noncontrast—typically hyperdense and most lesions enhance with contrast ● B. MRI: T1WI—hypo- to isointense ● C. MRI: T2WI—heterogeneous due to tumor cysts, vessels, and calcifications ● D. MRS: decreased choline and elevated NAA ● E. Spinal imaging: MRI with IV gadolinium or CT/myelography with water-soluble contrast should be done to rule out drop mets

D. MRS: decreased choline and elevated NAA

What is the 5-year event-free survival (EFS) of very high-risk group of medulloblastoma based on histology and molecular profiling? ● A. > 90% ● B. 75–90% ● C. 50–85% ● D. < 50%

D. < 50%

Gross nodular seeding in cerebellar subarachnoid space is designated as which stage in postoperative staging system? ● A. m0 ● B. m1 ● C. m2 ● D. m3 ● E. m4

C. m2

Regarding histological/morphological variants of medulloblastoma, what is the most common variant? ● A. Classic ● B. Large cell ● C. Nodular ● D. With extensive nodularity ● E. Anaplastic

A. Classic

Regarding histologic/morphologic variants of medulloblastoma, which of the following is associated with Gorlin syndrome in early childhood? ● A. Classic ● B. Large cell ● C. Nodular ● D. With extensive nodularity ● E. Anaplastic

C. Nodular

Regarding molecular genetics based clusters of medulloblastoma, a midline lesion that is typical in older childhood with male:female = 1:2. Predominant histologic variant is classic. Putative cell of origin is lower rhombic lip progenitor cells and frequently shows genetic mutations in CTNNB1, DDX3X, and TP53. This will be categorized as which of the following? ● A. WNT activated ● B. SHH activated, TP53 mutated ● C. SHH activated, TP53 wild type ● D. Non-WNT, non-SHH group III ● E. Non-WNT, non-SHH group IV

A. WNT activated

What percentage of children require permanent VP shunt placement following post fossa resection? ● A. 10 to 20% ● B. 20 to 30% ● C. 30 to 40% ● D. 50 to 60% ● E. 60 to 70%

C. 30 to 40%

What is the common location of cribriform neuroepithelial tumors (CRINET)? ● A. Suprasellar ● B. Near the ventricles ● C. CP angle ● D. Subchiasmatic ● E. Pineal region

B. Near the ventricles

Pineal region tumors are relatively rare and are more common in children than in adults. Germinoma is the most common tumor at this location followed by astrocytoma and teratoma. Which of the following is correct regarding the boundaries of pineal region? ● A. Bounded dorsally by the splenium of the corpus callosum and tele choroidea ● B. Bounded ventrally by the quadrigeminal plate and midbrain tectum ● C. Bounded rostrally by the posterior aspect of the third ventricle ● D. Bounded caudally by the cerebellar vermis ● E. All of the above

E. All of the above

Pineal region tumor in an adult of more than 40 years of age is most likely which of the following? ● A. Germinoma ● B. Meningioma or glioma ● C. Teratoma ● D. Ependymoma ● E. Glial cyst (pineal cyst)

B. Meningioma or glioma

In order to proceed for the management of pineal region mass, MRI of brain with and without contrast is needed. MRI of cervical, thoracic, and lumbar spine with IV contrast is also needed to see any drop metastasis. Which of the following statements is incorrect regarding the treatment of pineal region tumors? ● A. Pineal cyst of less than 2-cm size must be excised with surgery ● B. If there is hydrocephalus, then transventricular biopsy should be taken to see histopathology of tumor ● C. If there is no hydrocephalus, then open biopsy or stereotactic biopsy or navigation-assisted endoscopic biopsy should be taken ● D. If the histopathology comes out to be germinoma, then treatment is radiotherapy and chemotherapy ● E. For all tumors other than germinoma, the option is resection followed by adjuvant therapy

A. Pineal cyst of less than 2-cm size must be excised with surgery

Two main stereotactic trajectories for pineal region tumors include anterolateral (low frontal) approach below the internal cerebral vein and the second is posterolateral trans-parieto-occipital approach. Which of the following vessels is at risk of damage during pineal region surgery? ● A. Vein of Galen ● B. Basal vein of Rosenthal ● C. Internal cerebral veins ● D. Posterior medial choroidal artery ● E. All of the above

E. All of the above

The pineal gland is a neuroendocrine gland that secretes melatonin in response to photic stimulation of the retina by the bright light, and also regulates secretion of some pituitary hormones. It is outside the blood–brain barrier and is supplied by choroidal branches of the posterior cerebral artery. The tumors which are treated with surgical resection include following except? ● A. Radioresistant tumors like malignant non-germinoma GCTs ● B. Meningiomas and teratomas ● C. Pineacytoma ● D. Germinoma ● E. Well-encapsulated tumors

D. Germinoma

Among the following surgical approaches for the pineal region tumors, which is the most common? ● A. Midline infratentorial supracerebellar approach of Horsley and Krause as refined by Stein ● B. Occipital transtentorial approach ● C. Transventricular (requires dilated ventricles and large eccentric lesions) ● D. Lateral paramedian infratentorial ● E. Transcallosal ● F. Paramedian infratentorial supracerebellar approach

A. Midline infratentorial supracerebellar approach of Horsley and Krause as refined by Stein

Complications from surgery for pineal region tumors include new visual field deficits, epidural fluid collection, infection, and cerebellar ataxia. What is the surgical mortality rate of pineal region tumor surgery? ● A. 10 to 15% ● B. 15 to 20% ● C. 5 to 10% ● D. 20 to 25% ● E. 25 to 30%

C. 5 to 10%

A patient's biopsy after operation for pineal region tumor shows immature neuroepithelial cells with irregular hyperchromatic nuclei and a high nuclear to cytoplasm ratio, poorly defined cell borders, and a high Ki 67 index (23 to 50%). What is the most likely diagnosis in this case? ● A. Pineoblastoma ● B. Pineocytoma ● C. Parenchymal tumor of intermediate diﬀerentiation ● D. Pineoblastomacytoma ● E. All of the above

A. Pineoblastoma

What is the WHO grade of papillary tumor of papillary region? ● A. Grade 1 ● B. Grade 2 ● C. Grade 3 ● D. Grade 4 ● E. Both grades 1 and 2

E. Both grades 1 and 2

What is the ideal way of getting diagnosis of a pineal region tumor? ● A. Tumor markers ● B. Giving radiation and seeing response ● C. Taking biopsy ● D. Giving chemotherapy ● E. None of the above

C. Taking biopsy

Pineal region is the area of the brain bounded dorsally by the splenium of the corpus callosum and the tela choroidea, ventrally by the quadrigeminal plate and midbrain tectum, rostrally by the posterior aspect of the third ventricle, and caudally by which of the following? ● A. Cerebellar vermis ● B. Pons ● C. Medulla oblongata ● D. Sylvian fissure ● E. Obex

A. Cerebellar vermis

What is the most common tumor in pineal region in adults (> 40 years)? ● A. Germinoma ● B. Pineal cysts ● C. Meningioma ● D. Paraganglioma

C. Meningioma

Pineocytoma is a rare, well-diﬀerentiated tumor of the pineal parenchyma consisting of pineocytomatous rosettes of uniform cells and pleomorphic cells having gangliocytic diﬀerentiation, and typically aﬀects adults with average age at diagnosis of 43 years. What is the WHO grade for pineocytomas? ● A. I ● B. II ● C. III ● D. IV ● E. Not specified

A. I

Test dose radiation practice for management of pineal region masses was that if a pineal region tumor enhanced uniformly and had the classic appearance of a germinoma on MRI, a test dose of radiation was given, and if the tumor shrank then the diagnosis of germinoma was virtually certain and radiotherapy (XRT) was continued without surgery. What was the XRT dose? ● A. 2 Gy ● B. 3 Gy ● C. 4 Gy ● D. 5 Gy ● E. 10 Gy

D. 5 Gy

Pineal cysts do not require surgical removal unless they become large enough to cause obstructive hydrocephalus. What is the size at that time usually? ● A. > 2 cm ● B. > 3 cm ● C. > 4 cm ● D. > 5 cm ● E. > 1 cm

A. > 2 cm

Surgical treatment of pineal region tumors should be limited to all of the following except? ● A. Radioresistant, for example, nongerminomatous GCT ● B. Well-encapsulated tumors ● C. Benign tumors ● D. Pineocytoma ● E. Germinoma

E. Germinoma

What is the surgical mortality of pineal region tumors? ● A. 1 to 3% ● B. 3 to 5% ● C. 5 to 10% ● D. 10 to 15% ● E. 15 to 20%

C. 5 to 10%

What is the postoperative 5-year survival rate of pineal tumors? ● A. 65 to 70% ● B. 70 to 86% ● C. 86 to 91% ● D. 91 to 94% ● E. 94 to 98%

C. 86 to 91%

Which of the following computer-assisted cisternal endoscopic approach (CACE) permits visualization of neurovascular structures and avoids traversing brain parenchyma? ● A. Supracerebellar infratentorial ● B. Supracerebellar supratentorial ● C. Infracerebellar infratentorial ● D. Infracerebellar supratentorial ● E. Transtympanic

A. Supracerebellar infratentorial

Which of the following is the approach to the pineal region tumor extending into corpus callosum and third ventricle? ● A. Occipital transtentorial ● B. Transcallosal ● C. Lateral paramedian infratentorial ● D. Transventricular ● E. Approach of Horsley and Krause

B. Transcallosal

Schwannoma (WHO grade 1) is a benign nerve sheath tumor exclusively or primarily comprised of diﬀerentiated neoplastic Schwann cells with peak incidence in the fourth to sixth decades of life. Which statement is correct regarding location of these tumors? ● A. They commonly arise from peripheral nerves in the skin and subcutaneous tissue of the head and neck, or on the flexor surfaces of the limbs ● B. Intracranially they frequently involve the vestibular division of cranial nerve eight ● C. Intracranially these can also arise from cranial nerve five or other cranial nerves ● D. In the spine, these tumors form dumbbell shaped tumors as they extend out through the neural foramen which constricts them ● E. All of the above

E. All of the above

A 10-year-old patient presents with multiple cutaneous lesions that are typically soft, mobile, and asymptomatic. On histopathology, these tumors are found to be composed of neoplastic Schwann cells interspersed with normal cells and infiltrative low cellularity spindle cell neoplasm that is associated with a variably myxoid to collagenous stroma with mixed cell population. What is the most likely diagnosis in this patient? ● A. Schwannomatosis ● B. Neurofibroma ● C. Perineuroma ● D. Neurofibromatosis type 1 ● E. Hybrid nerve sheath tumors

B. Neurofibroma

A perineuroma is a benign WHO grade 1 tumor which is comprised exclusively of neoplastic perineural cells. Soft tissue perineuroma arises in deep soft tissues and presents as a soft tissue mass with nonspecific symptoms. Which of the following is not included in the essential diagnostic criteria of perineuroma? ● A. Slender spindle cells with bipolar cytoplasmic processes ● B. Storiform and/or whorled architecture ● C. Positive for at least one perineural antigen (EMA, claudin-1, GLUT1) ● D. Always associated with nerve fiber ● E. Negative for S100

D. Always associated with nerve fiber

Hybrid nerve sheath tumors are also known as benign peripheral nerve sheath tumor not otherwise specified (NOS). On histopathology, there are intermingled features of two types of benign nerve sheath tumors and immunohistochemical staining for each component. What is the most common type of hybrid nerve sheath tumor? ● A. Neurofibroma/schwannoma ● B. Schwannoma/perineuroma ● C. Neurofibroma/perineuroma ● D. Schwannoma/meningioma ● E. None of the above

B. Schwannoma/perineuroma

Malignant melanocytic nerve sheath tumors are aggressive tumors that usually arise from a spinal or autonomic nerve. What neoplastic cells are found in this tumor? ● A. Cells with melanocytic features ● B. A + Schwann cells ● C. A + Neurofibroma cells ● D. A + Astrocytes ● E. A + Meningeal cells

B. A + Schwann cells

Malignant peripheral nerve sheath tumors originate from peripheral nerve or a pre-existing benign nerve sheath tumor or in patient with NF-1 (approximately 50% are associated with NF-1). Following are features of this tumor which are associated with a favorable prognosis except? ● A. Smaller tumor size ● B. Female gender and Hispanic white race ● C. No metastasis at presentation ● D. GTR ● E. No association with prior XRT

B. Female gender and Hispanic white race

A patient presents with low-back pain and radiculopathy. A surgery is performed, and the tumor attached to filum terminale is excised. On preoperative imaging, it showed serpiginous dilated and ectatic blood vessels with a cap sign. On histology, it showed uniform, round or polygonal chief cells congregate in lobules (Zellballen) encompassed by a barely discernible single layer of susentacular (type 2) cells. What is the most probable diagnosis in this patient? ● A. Schwannoma ● B. Myxopapillary ependymoma ● C. Cauda equina neuroendocrine tumor ● D. Neurofibroma ● E. Malignant filum terminale neuroma

C. Cauda equina neuroendocrine tumor

A 35-year-old patient presents with decreasing hearing in right ear, tinnitus in the same ear, diﬃculty in maintaining balance, abnormal corneal reflex, and nystagmus. Weber test was found lateralizing to the left ear. Further testing showed pure tone audiogram threshold to be less than or equal to 50%, while speech discrimination score was more than or equal to 50% (serviceable hearing). MRI of brain showed oval enhancing tumor centered on IAC and trumpeting sign which was 2.2 cm in size and extended to the brainstem surface without displacing it. What is the Koos grading of this tumor? ● A. Grade 1 ● B. Grade 2 ● C. Grade 3 ● D. Grade 4 ● E. Grade 5

C. Grade 3

A patient presents with facial nerve palsy. On examination, this patient has obvious but not disfiguring asymmetry of face, slight to moderate motion of forehead, complete closure of eye with eﬀort, and slightly weak mouth with maximal eﬀort. What is the House and Brackmann facial nerve grading in this patient? ● A. Grade 1 ● B. Grade 2 ● C. Grade 3 ● D. Grade 4 ● E. Grade 5

C. Grade 3

A patient presents with vestibular schwannoma in your clinic. What are the treatment options in this patient? ● A. Surgery through translabyrinthine approach ● B. Surgery through retrosigmoid approach ● C. Surgery through subtemporal approach ● D. Stereotactic radiosurgery ● E. All of the above

E. All of the above

All of the following are desirable for diagnosis of schwannoma except? ● A. Verocay bodies ● B. Subcapsular lymphocytes ● C. Hyalinized blood vessels ● D. Lattice-like pattern of CD34 staining ● E. Loss of SMARCB1 expression

D. Lattice-like pattern of CD34 staining

What is the most common subtype of hybrid nerve sheath tumors? ● A. Schwannoma/perineurioma ● B. Neurofibroma/schwannoma ● C. Neurofibroma/perineurioma

A. Schwannoma/perineurioma

Which of the following features is not associated with a favorable prognosis for MPNST? ● A. Smaller tumor size ● B. Female gender ● C. GTR ● D. No mets at presentation ● E. No association with prior XRT

B. Female gender

Which of the following tumors of cauda equina demonstrate dilated ectatic blood vessels with a cap sign on T2MRI or GRASS MRI? ● A. Myxopapillary ependymoma ● B. Astrocytoma ● C. Cavernoma ● D. Cauda equina NET ● E. AVM

D. Cauda equina NET

A 45-year-old female presented in OPD department with sudden hearing loss (SHL). Treatment options of SHL include all of the following except? ● A. 60-mg prednisone QID × 10 days ● B. Famciclovir 500 mg TID × 10 days ● C. Intravenous heparin ● D. Conservative ● E. Thrombolytic therapy

B. Famciclovir 500 mg TID × 10 days

On histopathology of vestibular schwannomas along with Antoni A and Antoni B fibers, which of the following can be present? ● A. Psammoma bodies ● B. Verocay bodies ● C. Langerhans cells ● D. Calcifications ● E. Neovascularization

B. Verocay bodies

On the first postoperative day of cerebellopontine angle space occupying lesion (CPA SOL) of a patient, you observed the patient had obvious asymmetry of face, slight to moderate movement of forehead, and complete eye closure with eﬀort. What is the facial nerve palsy grading according to House and Brackmann? ● A. I ● B. II ● C. III ● D. IV ● E. V

C. III

For auditory brainstem responses (ABR), which of the following is true? ● A. Diagnosis of hearing loss ● B. Assess the inferior vestibular nerve ● C. Assess the superior vestibular nerve ● D. Prognosticator for hearing preservation ● E. Cannot be assessed in deaf ear

D. Prognosticator for hearing preservation

After gross total excision of vestibular schwannoma, when should MRI be done? ● A. Within 72 hours after surgery ● B. At 3 months ● C. At 6 months ● D. At 1 year ● E. At 5 years

D. At 1 year

In Koos grading system, what is the grade of a vestibular schwannoma (VS) protruding into CPA, not reaching brainstem, with approximate volume of 0.6 cc? ● A. I ● B. II ● C. III ● D. IV ● E. V

B. II

What is the normal diameter of IAC? ● A. 0.5 to 0.8 cm ● B. 0.8 to 1 cm ● C. 0.3 to 0.5 cm ● D. 0.4 to 1 cm ● E. 0.1 to 0.5 cm

A. 0.5 to 0.8 cm

In which of the following, serviceable hearing is unlikely to be preserved postoperatively? ● A. Preoperative SDS < 75% ● B. Preoperative PTA loss > 25 dB ● C. Preoperative BAER has abnormal wave morphology ● D. Tumor > 2 to 2.5 cm diameter ● E. All of the above

E. All of the above

Malignant peripheral nerve sheath tumors are usually associated with which of the following? ● A. NF2 ● B. NF1 ● C. Li Fraumeni syndrome ● D. Tuberous sclerosis ● E. Terson syndrome

B. NF1

Which of the following structures can be sacrificed in retrosigmoid approach for CPA tumor resection? ● A. Facial nerve ● B. Greater occipital nerve ● C. Petrosal vein ● D. Superior cerebellar artery ● E. PICA

C. Petrosal vein

What is the 7th cranial nerve preservation rate in retrosigmoid removal of vestibular schwannoma (VS) when the tumor is > 2 cm? ● A. 26 to 30% ● B. 30 to 50% ● C. 50 to 76% ● D. 70 to 86% ● E. 80 to 96%

C. 50 to 76%

A patient presents with a long history of headache. MRI shows isointense lesion on T1 while hypointense on T2, signal voids showing calcifications, densely enhancing broad-based attachment to dura, and a dural tail. Hyperostosis of the adjacent bone and homogenously hyperdense lesion on CT of brain are also seen. Meningiomas are the most common primary intracranial tumors in adults, representing 36.4% of CNS tumors that arise from meningothelial cells of arachnoid membrane. Following statements are correct regarding these tumors except? ● A. Most are slow growing benign tumors, with 32% of incidentally discovered meningiomas showing no growth over 3 years follow-up ● B. They are most commonly located along falx, convexity and sphenoid bone ● C. Psammoma bodies are round, microscopic, calcified structures having a concentric laminated appearance ● D. Chordoid and clear cell subtypes have been assigned grade 1 tumor with low rate of recurrence ● E. The most common subtypes of meningioma are meningothelial, fibrous, and transitional

D. Chordoid and clear cell subtypes have been assigned grade 1 tumor with low rate of recurrence

On histopathology, a meningioma is found to have syncytia-like lobules of epithelioid cells with some nuclei appearing to have holes and pseudoinclusions. Cells resemble arachnoid cap cells mimicking meningothelial hyperplasia which was found adjacent to the optic glioma. Whorls and psammoma bodies are less frequent in this tumor. What is the most likely subtype of this meningioma? ● A. Meningothelial meningioma ● B. Fibrous meningioma ● C. Transitional meningioma ● D. Psammomatous meningioma ● E. Angiomatous meningioma

A. Meningothelial meningioma

The most common locations of meningioma in brain are parasagittal accounting for 20.8%, then convexity (15.2%) and then tuberculum sellae which accounts for 12.8%, and fourth is the sphenoid wing meningioma which accounts for 11.9%. Which of the following statements is correct regarding sphenoid wing meningioma? ● A. It has three basic categories depending on the origin from the sphenoid wing ● B. Behavior and treatment of lateral sphenoid wing (pterional) meningioma are similar to convexity meningioma ● C. Middle third meningiomas are also called as alar meningioma ● D. Medial (clinoidal) meningiomas tend to encase the ICA and the MCA as well as cranial nerves in the region of the superior orbital fissure and the optic nerve. Total removal of this type is often not possible ● E. All of the above are correct

E. All of the above are correct

A patient presents with anosmia, ipsilateral optic atrophy, and contralateral papilledema (Foster Kennedy syndrome). On further evaluation, the patient is found to have apathy, abulia, urinary incontinence, and memory disturbances. Plain MRI of brain and with IV contrast found olfactory groove meningioma. Why is embolization contraindicated in these tumors? ● A. It is very diﬃcult to embolize these tumors ● B. It gets 70 to 80% of blood supply from anterior cerebral artery which is not embolized due to risk of ophthalmic artery embolization and blindness ● C. It gets 70 to 80% of blood supply from anterior ethmoidal artery which is not embolized due to risk of ophthalmic artery embolization and blindness ● D. Embolization of perforating arteries can lead to loss of olfaction in these cases ● E. Wrong embolization poses high risk of brain stem damage

C. It gets 70 to 80% of blood supply from anterior ethmoidal artery which is not embolized due to risk of ophthalmic artery embolization and blindness

Parasagittal meningiomas are grouped based on AP direction of the superior sagittal sinus and their location along this direction. Anterior meningiomas are found between ethmoidal plate and coronal suture (44%), middle meningiomas are found between coronal suture and lambdoidal suture accounting for 33%, while posterior meningiomas are found between lambdoid suture and torcular Herophili accounting for 23%. Which of the following statements is not correct regarding Sindou grading system for meningioma invasion of superior sagittal sinus? ● A. Type 1 meningiomas are attached to the lateral wall of sinus ● B. Type 2 meningiomas cause invasion of lateral recess, while type 3 meningiomas cause invasion of lateral wall ● C. Type 4 meningiomas cause invasion of roof of sinus ● D. In type 5, there is incomplete occlusion of sinus with contralateral wall spared ● E. In type 6, there is total sinus occlusion with invasion of contralateral wall

D. In type 5, there is incomplete occlusion of sinus with contralateral wall spared

A patient presents with gradual onset of bitemporal hemianopia, and on examination he is found to have a tumor which originated in the middle cranial fossa, and the site of origin is only 2 cm posterior to that of the olfactory groove meningioma. Which of the following statements is correct regarding these tumors and anatomy of region from where these arise? ● A. This tumor is tuberculum sellae meningioma ● B. Tuberculum sellae is the bony elevation between the chiasmatic sulcus and the sella turcica ● C. Planum sphenoidale, which is the anterior margin of charismatic sulcus, is the demarcation between anterior and middle cranial fossa ● D. Posteriorly growing tuberculum sellae meningioma can mimic pituitary macroadenoma ● E. All of the above

E. All of the above

Which of the following is an environmental risk for development of meningioma? ● A. Ionization radiation such as children exposed to ionizing radiation while treating leukemia ● B. Hormonal exposure such as in women receiving hormonal replacement therapy ● C. Mutation of NF2 tumor suppresser gene on chromosome 22q12.2 ● D. Those which are associated with NF2 or NF1 ● E. Mutations in AKT1, TRAF7, and SMO

A. Ionization radiation such as children exposed to ionizing radiation while treating leukemia

Meningiomas characteristically have external carotid artery feeders. What are some notable exceptions where they are fed from the ICA? ● A. Low frontal median meningiomas, for example, olfactory groove, are fed from ethmoidal branches of the ophthalmic artery ● B. Suprasellar meningiomas may also be fed by large branches of the ophthalmic arteries ● C. Parasellar meningiomas tend to be fed from the ICA while secondary vascular supply may be derived from pial branches of the anterior, middle, and posterior cerebral arteries ● D. Petroclival meningiomas are supplied via the artery of tentorium also known as artery of Bernasconi and Cassinari ● E. All of the above

E. All of the above

A patient with meningioma is symptomatic, with significant growth on serial imaging (more than 2.5 mm increase in diameter over 1 year) and marked perilesional edema. Excision was done. Postoperative images showed residual tumor with histology report showing anaplastic lesion. Hence, this patient was given postoperative radiotherapy and further treated with MRI-guided laser interstitial thermal therapy. Which of the following is incorrect regarding Simpson grading system for removal of meningioma? ● A. Grade 1 is macroscopically complete removal with excision of dural attachment and abnormal bone, not including sinus resection when involved, with recurrence rate of 9% ● B. Grade 2 is macroscopically complete removal with endothermy coagulation of dural attachment, with recurrence of 19% ● C. Grade 3 is macroscopically complete removal without resection or coagulation of dural attachment or its extradural extension, with recurrence of 29% ● D. Grade 4 is partial removal, removing tumor in situ, with recurrence rate of 44% ● E. Grade 5 is simple decompression with biopsy

A. Grade 1 is macroscopically complete removal with excision of dural attachment and abnormal bone, not including sinus resection when involved, with recurrence rate of 9%

Anterior third parasagittal meningiomas can be approached through bicoronal flap with safe division of sinus in this location while posterior to coronal suture, the sinus cannot be divided because of risk of venous drainage compromise. Meningiomas are often very bloody. What is the general principle of meningioma surgery? ● A. Early interruption of arterial blood supply of tumor ● B. Avoid excessive retraction of brain by doing internal decompression of tumor ● C. Dissecting the capsule from the brain ● D. Removal of attached bone and dura when possible ● E. All of the above

E. All of the above

Meningiomas are the most common primary intracranial tumors that arise from meningothelial cells of which of the following? ● A. Dura ● B. Arachnoid ● C. Pia ● D. Ependymal lining of ventricles ● E. Radial cells

B. Arachnoid

What are the round, microscopic, calcified structures having concentric, laminated appearance, possibly formed as a tumoricidal defense mechanism, found in meningiomas called? ● A. Psammoma bodies ● B. Mallory bodies ● C. Michaelis Gutmann bodies ● D. Schiller Duval bodies ● E. Verocay bodies

A. Psammoma bodies

Multiple meningiomas are associated with which of the following? ● A. Neurofibromatosis II ● B. Tuberous sclerosis ● C. Von Hippel Lindau syndrome ● D. Li Fraumeni syndrome ● E. Sturge Weber syndrome

A. Neurofibromatosis II

What is the grading criteria for WHO grade 2 meningiomas? ● A. 1–4 mitotic figures in 10 consecutive HPF each of 0.16 mm2 ● B. 4–19 mitotic figures in 10 consecutive HPF each of 0.16 mm2 ● C. 20–25 mitotic figures in 10 consecutive HPF each of 0.16 mm2 ● D. 25–30 mitotic figures in 10 consecutive HPF each of 0.16 mm2

B. 4–19 mitotic figures in 10 consecutive HPF each of 0.16 mm2

What is the most common location of meningiomas? ● A. Parasagittal ● B. CP angle ● C. Olfactory groove ● D. Intrasylvian ● E. Tuberculum sellae

A. Parasagittal

Which of the sphenoid wing meningiomas tend to encase ICA and MCA frequently? ● A. Clinoidal ● B. Middle ● C. Alar ● D. Pterional ● E. En masse

A. Clinoidal

What is the Sindou grade of a meningioma with invasion of lateral recess of superior sagittal sinus? ● A. I ● B. II ● C. III ● D. IV ● E. V

B. II

Regarding meningioma surgical excision, what is the Simpson grade of macroscopically complete excision with endothermy coagulation of dural attachment? ● A. I ● B. II ● C. III ● D. IV ● E. V

B. II

Proposed criteria for significant growth of meningiomas includes all of the following except? ● A. 2–5 mm increase in diameter over 1 year ● B. 1 cc increase in volume in 1 year ● C. 15% increase in volume over 1 year ● D. 5 cc increase in volume in 1 year

D. 5 cc increase in volume in 1 year

Postoperative radiotherapy is not recommended for which of the following? ● A. WHO grade 1 meningiomas with subtotal resection (STR) ● B. Anaplastic meningioma regardless of extent of resection (EOR) ● C. New atypical meningiomas with STR ● D. Recurrent atypical meningioma regardless of EOR

A. WHO grade 1 meningiomas with subtotal resection (STR)

Which approach provides ideal extended exposure to medial sphenoid wing meningiomas? ● A. Subfrontal approach ● B. Bicoronal approach ● C. Conventional pterional approach ● D. Fronto-temporo-orbito-zygomotomy (FTOZ) ● E. Anterior transpetrosal approach

D. Fronto-temporo-orbito-zygomotomy (FTOZ)

A patient presented in neurosurgery OPD with brain imaging showing meningioma-like features. Surgery was done and histopathology showed variably cellular tumor composed of spindled to ovoid cells arranged around a branching and hyalinized vasculature and variable stromal collagen deposition and STAT6 nuclear expression. This tumor has a high rate of recurrence as late as decades. What is the most likely diagnosis? ● A. Atypical meningioma ● B. Pleomorphic astrocytoma ● C. Solitary fibrous tumor ● D. Carcinomatous fibrous tumor ● E. None of the above

C. Solitary fibrous tumor

A patient is found to have tumor with tightly packed capillary-sized and cavernous vessels with a single layer of benign epithelial cells. This tumor is found most commonly in the spine followed by skull and uncommonly in the brain parenchyma, nerve roots, and cauda equina with a predilection for thoracic and lumbar region (often multiple). Following are the imaging findings in a patient with hemangioma except? ● A. Polka-dot sign is found on axial images ● B. Corduroy pattern is found on longitudinal images ● C. Calcifications are very rare in these tumors ● D. T2 MRI shows mixed signal internally ● E. The surrounding hemosiderin ring is low density on T2 with blooming artifact

C. Calcifications are very rare in these tumors

A patient presents with nausea, vomiting, headache, cerebellar signs, and obstructive hydrocephalus. CT of brain showed low density cystic lesion with contrast enhancing nodule in the posterior fossa, MRI showed serpentine vascular signal voids with cystic lesion, and blood workup showed polycythemia. These tumors are most common primary intra-axial tumors in the adult posterior fossa and 30% of these occur as part of Von Hippel-Lindau (VHL) disease. What is the most likely diagnosis in this patient? ● A. Hemangioma ● B. Pilocytic astrocytoma ● C. Epidermoid tumor ● D. Hemangioblastoma ● E. Dermoid tumor

D. Hemangioblastoma

Which of the following statements is incorrect regarding treatment of hemangioblastoma? ● A. Gross total resection with removal of mural nodule and leaving cyst wall is optimal treatment (unless there is evidence of thickened wall cysts with tumor on MRI or direct visualization during surgery) ● B. Preoperative embolization may help reduce the vascularity ● C. Prognosis is excellent following GTR and is better for cases with VHL than for sporadic cases ● D. In case of cystic brainstem hemangioblastoma, solid tumor of the nodule is removed under microscope ● E. Radiotherapy is helpful for multiple small deep lesions, inoperable brainstem hemangioblastomas, for patients who are not surgical candidate, although it does not prevent regrowth following subtotal excision

C. Prognosis is excellent following GTR and is better for cases with VHL than for sporadic cases

A 50-year-old male patient presents with backache, pain in legs, sphincter disturbance, and nerve root symptoms. CT and MRI show centrally located bony destruction with an anterior soft tissue mass (with small calcifications) which is confined anteriorly by soft presacral fascia. This lesion is hypointense on T1, hyperintense on T2, and contrast enhancing. A firm fixed mass is palpable between rectum and sacrum on rectal examination. What is the most likely diagnosis in this patient? ● A. Spinal chordoma ● B. Chondrosarcoma ● C. Osteosarcoma ● D. Osteochondroma ● E. Osteoblastoma

A. Spinal chordoma

Chordomas are rare tumors which arise from remnant of the primitive notochord (which normally diﬀerentiates into the nucleus pulposus of the intervertebral disks). Histologically these tumors are considered low grade but still why are these primary malignant tumors? ● A. Diﬃculty of total removal ● B. A high recurrence rate of 85% following surgery ● C. They metastasize in 5 to 20% of cases ● D. Locally aggressive and osteo-destructive ● E. All of the above

E. All of the above

Four types of chordomas have been identified, namely, conventional, chondroid, dediﬀerentiated, and poorly diﬀerentiated. Which statement is incorrect regarding location of chordoma? ● A. Chordoma can arise anywhere along entire the neuraxis where there is remnant of notochord ● B. 35% occur cranially in the spheno-occipital region (clivus) ● C. 53% occur in the spine at the sacrococcygeal region ● D. 3% are intracranial tumors while 1% are primary spine tumors ● E. Less commonly, these may occur in the spine above sacrum

D. 3% are intracranial tumors while 1% are primary spine tumors

What is the most appropriate management options regarding chordomas? ● A. For chordomas caudal to the third sacral segment, a posterior approach with wide en block excision and postoperative radiation is usually the best option ● B. Decompression is best avoided because entering the mass serves to spread the tumor (surgically induced metastasis) which will then regrow ● C. Chordomas located in the C2 are usually not amenable to en bloc resection ● D. If s2 nerve roots are the most caudal nerve roots spared, there is 50% chance of normal bladder and bowel function while above this, most will have impaired bladder control and bowel problems ● E. All of the above

E. All of the above

Which statement is correct regarding radiotherapy in chordoma patients? ● A. Radiotherapy does prevent recurrence when done in conjunction with palliative or debulking surgery ● B. It is indicated only for residual, recurrent, or inoperable cases ● C. Best results are obtained only with en bloc excision combined with high dose radiotherapy ● D. Proton beam therapy alone or combined with high-energy X-ray photon therapy is more eﬀective than conventional radiotherapy and is readily available ● E. Early radiation has no eﬀect on longer survival

C. Best results are obtained only with en bloc excision combined with high dose radiotherapy

Chemotherapeutic agent imatinib (a tyrosine kinase inhibitor) has some antitumor eﬀect in chordoma. Which subtype of chordoma has worse prognosis? ● A. Dediﬀerentiated ● B. Poorly diﬀerentiated ● C. Conventional ● D. Chondroid ● E. Both A and B

E. Both A and B

Radiologically, a solitary fibrous tumor may be mistaken for which tumor? ● A. Astrocytoma ● B. Meningioma ● C. Osteosarcoma ● D. Fibrous dysplasia ● E. Oligodendroglioma

B. Meningioma

What is the characteristic of WHO grade 3 solitary fibrous tumor? ● A. Hypocellular tumor, < 2.5 mitosis/10 HPF ● B. Dense tumor, > 2.5 mitosis/mm2 (> 5 mitosis/10 HPF) ● C. Dense tumor, > 5 mitosis/mm2 (> 10 mitosis/10 HPF) ● D. > 2.5 mitosis/mm2 (> 5 mitosis/10 HPF) with tumoral necrosis ● E. Dense tumor, > 5 mitosis/mm2 (> 10 mitosis/10 HPF) with tumoral necrosis

D. > 2.5 mitosis/mm2 (> 5 mitosis/10 HPF) with tumoral necrosis

Which of the following is the most common site of hemangiomas? ● A. Skull ● B. Spine ● C. Brain parenchyma ● D. Nerve roots ● E. Cauda equina

B. Spine

Which of the following is the most common primary intraaxial tumor in the adult posterior fossa? ● A. Pilocytic astrocytoma ● B. Ependymoma ● C. Choroid plexus papilloma ● D. Hemangioblastoma ● E. Medulloblastoma

D. Hemangioblastoma

If the cases of intracerebral hemorrhage (ICH) are carefully examined, which tumor is likely to be found as an underlying cause? ● A. AVM ● B. Hemangiomas ● C. Hemangioblastoma ● D. Cavernoma ● E. Hemangioblastoma

C. Hemangioblastoma

Which of the following is the most common cyst pattern seen in hemangioblastoma? ● A. No associated cyst ● B. Peritumoral cyst alone ● C. Intratumoral cyst ● D. Peritumoral and intratumoral cysts

B. Peritumoral cyst alone

Which of the following is not an angiographic pattern seen in hemangioblastoma? ● A. Vascular mural nodule on side of avascular cyst ● B. Vascular lesion surrounding avascular cyst ● C. Solid vascular mass ● D. Multiple, separate vascular nodules ● E. Avascular cyst surrounding vascular lesion

E. Avascular cyst surrounding vascular lesion

Chordoma originates from which of the following layers? ● A. Notochord ● B. Neural crest cells ● C. Mesoderm ● D. Ectoderm ● E. Endoderm

A. Notochord

A patient underwent sacrectomy for chordoma. The s2 nerve root was spared. What is the chance of preservation of bladder and bowel control? ● A. 10% ● B. 20% ● C. 30% ● D. 40% ● E. 50%

E. 50%

A patient presents to neurosurgical OPD with MRI showing hyperintensity of leptomeninges with subarachnoid spaces on T1WI and same lesions hypointense on T2WI, with multifocal nodularity and invasion of Virchow-Robin (perivascular) spaces. On histology, there are diﬀuse, multifocal, primary me- ningeal melanocytic lesion with invasion of CNS parenchyma, marked cytological atypia, absence of mitotic activity, and necrosis. What is the location of diﬀuse meningeal melanocytic neoplasms in the brain? ● A. Temporal lobes ● B. Cerebellum ● C. Pons ● D. Medulla ● E. All of the above

E. All of the above

A well-developed, solid, noninfiltrating tumor arises from the leptomeninges, characterized by epithelioid, fusiform, polyhedral, or spindled melanocytes with no evidence of anaplasia, necrosis, or increased mitoses. These lesions are most commonly found in cervical and thoracic spine. What is the most likely diagnosis in this scenario? ● A. Meningeal melanoma ● B. Meningeal melanocytoma ● C. Meningeal melanomatosis ● D. Meningeal melanocytosis ● E. None of the above

B. Meningeal melanocytoma

All of the following are included in the essential diagnostic criteria of meningeal melanoma except? ● A. Circumscribed/localized primary melanocytic neoplasm in the meninges ● B. Mitotic count more than 1.5 mitosis/mm2 ● C. Demonstration of GNAQ, GNA11, PLCB4, or CYSLTR2 mutation ● D. Necrosis ● E. Marked cytological atypia

C. Demonstration of GNAQ, GNA11, PLCB4, or CYSLTR2 mutation

Meningeal melanomas are pleomorphic, anaplastic, mitotically active, and often invade CNS tissue and may demonstrate necrosis. These tumors may spread through CSF pathways, and occasionally systemic metastases outside the CNS do occur. Which statement regarding management of these tumors is correct? ● A. These are not aggressive tumors ● B. With GTR, prognosis is better than with cutaneous melanoma metastatic to the CNS ● C. These tumors respond well to radiotherapy ● D. Peak age of these tumors is in 4th decade ● E. All of the above

B. With GTR, prognosis is better than with cutaneous melanoma metastatic to the CNS

Germ cell tumors primarily aﬀect children and adolescents with peak age of 10 to 14 years. Which of the following statements is incorrect regarding these tumors? ● A. 80 to 90% of these tumors occur in the midline usually in the suprasellar and pineal region ● B. These tumors are not sensitive to radiotherapy ● C. Simultaneous suprasellar and pineal region lesions is diagnostic of so-called synchronous germ cell tumors which constitutes 13% of germ cell tumors ● D. In the pineal region, these tumors occur predominantly in males while in females germ cell tumors are more common in the suprasellar region ● E. All intracranial germ cell tumors are malignant and may metastasize via CSF and systemically

B. These tumors are not sensitive to radiotherapy

Germ cell tumors can (but not always) give rise to tumor markers in the CSF. Which of the following statements is correct? ● A. Choriocarcinoma and 50% of germinoma show elevated levels of beta human chorionic gonadotropin levels in the CSF ● B. Yolk sac tumors, embryonal carcinoma, and occasionally teratoma show elevated levels of alpha-fetoprotein ● C. Intracranial germinomas show elevated serum or CSF levels of placental alkaline phosphate ● D. Many of these tumors are mixed cell type and many pineal region tumors do not give rise to markers ● E. All of the above

E. All of the above

A patient presents with tumor that has germ cell tumor with components exhibiting diﬀerentiation along at least two of the three somatic tissue lines (ectoderm, endoderm, mesoderm) and fully diﬀerentiated adult type histology and absence of germ cell tumor components. What is the most likely diagnosis in this patient? ● A. Mature teratoma ● B. Immature teratoma ● C. Teratoma with somatic type malignancy ● D. Germinoma ● E. Embryonal carcinoma

A. Mature teratoma

Which statement is incorrect regarding germinoma? ● A. These contain large undiﬀerentiated-appearing cells resembling primordial germ cells with ample glycogen-rich clear cytoplasm containing round, vesicular, and centrally placed nuclei ● B. Necrosis is common in these tumors ● C. Mitotic activity is variable ● D. The cells are arranged in sheets, lobules, or cords with trabeculae ● E. Some may have syncytiotrophoblastic giant cells

B. Necrosis is common in these tumors

Which of the following is not included in the essential diagnostic criteria of embryonal carcinoma? ● A. A germ cell tumor with large epithelioid cells ● B. Cytokeratin expression ● C. CD30 or OCT4 expression ● D. Absent or only focal nonmembranous KIT expression ● E. Absence of hCG expression, absence of AFP expression, and absence of other germ cell tumor components

B. Cytokeratin expression

Which of the following is a feature if choriocarcinoma? ● A. Only syncytiotrophoblasts are seen ● B. Only cytotrophoblastic elements are visible ● C. Both syncytiotrophoblasts and cytotrophoblasts are seen ● D. It shows APD expression ● E. There is CD30 expression

C. Both syncytiotrophoblasts and cytotrophoblasts are seen

How are primary CNS melanocytic tumors distinguished from other pigmented CNS neoplasms like melanotic nerve sheath tumors? ● A. Location ● B. Negri bodies ● C. Tissue histology ● D. Molecular analysis ● E. Alkylation profiling

D. Molecular analysis

What is the most common location of germ cell tumors in females? ● A. Suprasellar ● B. Pineal region ● C. CP angle ● D. Cisterna magna ● E. Interpeduncular cistern

A. Suprasellar

Regarding germ cell tumors, which CSF tumor marker is expressed in almost 100% of choriocarcinomas? ● A. Beta HCG ● B. ALP ● C. PLAP ● D. AFP ● E. INF

A. Beta HCG

What is the diagnostic criterion for mature teratoma? ● A. A germ cell tumor with components exhibiting diﬀerentiation along all three somatic tissue lines ● B. Fully diﬀerentiated ● C. Dediﬀerentiated ● D. Fetal histology ● E. Presence of embryonal germ cell components

B. Fully diﬀerentiated

Absence of all of the following is essential for diagnosis of choriocarcinoma except? ● A. KIT ● B. AFP ● C. CD30 ● D. OCT4 ● E. β-HCG

E. β-HCG

Section 2 Flashcards

(452 cards)