Functional

Question 1

A 65-year-old male presents with resting tremor, rigidity, postural instability, and bradykinesia. Which of the following symptoms is most likely to also be present?
a. Dry mouth.
b. Hyposmia.
c. Hypertension.
d. Diabetes.
e. Diarrhea.

Answer 1

Hyposmia.
The patient exhibits cardinal features of Parkinson’s disease (PD). Common non-motor symptoms of PD include loss of sense of smell (hyposmia) and taste, neuropsychiatric problems, mood disorders, sleep disturbances, constipation, bladder problems, orthostatic hypotension, and excessive salivation. Medication side effects with levodopa can include impulsive behavior and hallucinations.

Question 2

Stimulation in what peri-Sylvian region most likely cause speech arrest during awake speech mapping?
a. Pars orbitalis.
b. Gyrus supramarginalis.
c. Pars opercularis.
d. Gyrus angularis.
e. Pars triangularis.

Answer 2

Pars opercularis.
The pars opercularis is the region where stimulation is most likely to cause speech arrest. Broca’s area is typically adjacent to the inferior precentral sulcus or pars opercularis. Awake speech mapping is crucial to minimize neurological deficits during tumor resection in this area.

Question 3

Which inclusion-based feature is classic for Parkinson’s disease?
a. Ubiquitin-positive inclusions.
b. Lewy bodies.
c. Ballooned neurons.
d. Neurofibrillary tangles.
e. Tau-positive inclusions.

Answer 3

Lewy bodies.
Lewy bodies, which are intracytoplasmic eosinophilic neuronal inclusions, are classic for Parkinson’s disease, found in persisting pigmented neurons in the substantia nigra. Neurofibrillary tangles are found in Alzheimer’s disease, among others. Ubiquitin-positive inclusions are seen in multiple systems atrophy and motor neuron disease. Tau-positive inclusions are characteristic of several neurodegenerative diseases. Ballooned neurons are found in Pick’s disease and Corticobasal degeneration.

Question 4

A 55 year-old woman has intractable seizures treated with levetiracetam, oxcarbazepine, and vagus nerve stimulation (VNS). She presents with increased seizure activity after 2 days of painful coughing and odynophagia, preventing her from taking her anticonvulsant medications. Examination reveals a hoarse voice. What is the most appropriate initial intervention?
A Obtain electroencephalogram.
B Throat cultuate and administer antibiotics.
C Increase VNS frequency.
D Inactivate VNS.
E Start phenytoin.

Answer 4

Inactivate VNS.
Throat pain, hoarseness, dysphagia, and coughing can be side-effects of VNS therapy, potentially acutely after increased output settings or subacutely with chronic therapy. The increased seizure activity may be due to difficulty swallowing medications because of VNS complications. The device can be temporarily turned off using a magnet.

Question 5

What is the approximate seizure freedom rate in patients following non-lesional resective frontal lobe epilepsy surgery?
a. 0-10%
b. 20-30%
c. 60-70%
d. 40-50%
e. 80-90%

Answer 5

40-50%.
Seizure freedom rates (Engel Class I) following frontal lobe epilepsy surgery are approximately 40-50%. A meta-analysis reported an average rate of 45%. This is lower than the 60-70% rate for temporal lobe epilepsy. Negative prognostic factors include normal imaging and generalized scalp EEG abnormalities.

Question 6

In the Hassler terminology, the ventrolateral thalamus is subdivided into three regions, including which of the following:
a. Ventral oralis posterior (VOP), ventral intermediate (VIM), and ventral caudalis (VC).
b. Ventral lateral (VL), ventral intermediate (VIM), and ventral posterior lateral (VPL).
c. Ventral oralis anterior (VOA), ventral oralis posterior (VOP), and ventral caudalis (VC).
d. Ventral oralis anterior (VOA), ventral oralis posterior (VOP), and ventral intermediate (VIM).
e. Ventraloralis anterior (VOA), ventral intermiate (VIM), and ventral caudalis (VC).

Answer 6

Ventral oralis anterior (VOA), ventral oralis posterior (VOP), and ventral intermediate (VIM).
The Hassler classification divides the ventrolateral nucleus of the thalamus into VOA, VOP, and VIM, arranged anterior to posterior. VIM and VOP are primary targets for tremor suppression. VOA has been targeted for medically refractory dystonia. The ventral caudalis (VC) nucleus is posterior to these and receives sensory input.

Question 7

A 25-year-old man presents to clinic with progressive worsening of twisting movement in his torso. His symptoms began as right foot and leg cramps during late teens and progressed to include arms, neck, and torso. He is otherwise healthy and has an uncle with similar symptoms. What is the most likely diagnosis?
a. Secondary focal/segmental dystonia
b. Idiopathic scoliosis
c. Isolated focal/segmental dystonia
d. Isolated generalized dystonia
e. Secondary generalized dystonia

Answer 7

Isolated generalized dystonia.
The patient has generalized dystonia (trunk and at least 2 other limbs). Adolescent onset with progressive worsening is typical for isolated (primary) dystonia. Some dystonias have a strong familial component and may involve DYT gene mutations.

Question 8

A 45-year-old male has a five-year history of progressive right arm intention tremor. A similar condition is present in his father and paternal uncle. The tremor improves with alcohol ingestion, but has become increasingly refractory to propanolol, mysoline, as well as topamax. There is no rigidity, bradykinesia, or gait abnormality present. What is the most appropriate neurosurgical intervention?
a. Globus pallidus deep brain stimulator
b. Thalamic deep brain stimulator
c. Subthalamic nucleus deep brain stimulator
d. C6,7 selective rhizotomy
e. Stereotactic pallidotomy

Answer 8

Thalamic deep brain stimulator.
The patient has clinical signs of benign essential tremor. For medically refractory essential tremor, the preferred target is the Ventral Intermediate (Vim) nucleus of the thalamus. This can be achieved via deep brain stimulation (DBS) or lesioning (thalamotomy). DBS offers adjustability and reversibility but has hardware-related risks.

Question 9

A 40 year-old male presents with a 3-year history of uncontrollable writhing movement of all four extremities and recent memory decline. He has a family history of early-onset dementia and suicide in male relatives. MRI reveals atrophy of the caudate nucleus bilaterally. What developmental abnormality would you expect to find?
a. Chromosome 14-21 translocation
b. Expanded GAA repeats
c. Parkin gene mutation
d. Expanded CAG repeats
e. FMR1 gene mutation

Answer 9

Expanded CAG repeats.
The patient has Huntington’s disease, characterized by choreiform movements, psychiatric symptoms, and caudate nucleus degeneration. It’s an autosomal dominant disease with trinucleotide CAG expansion. Parkin gene mutations are linked to juvenile Parkinson’s. Chromosome 14:21 translocation can cause Trisomy 21. GAA repeats are associated with Friedreich’s Ataxia, and FMR1 mutations with Fragile X syndrome.

Question 10

Epilepsy is generally defined as:
a. two or more provoked seizures.
b. one unprovoked seizure with a remote history of febrile seizure
c. two or more unprovoked seizures.
d. one provoked seizure.
e. one unprovoked seizure.

Answer 10

Two or more unprovoked seizures.
Epilepsy is defined as a condition with recurrent, unprovoked seizures. Two or more unprovoked seizures meet this definition. About 25% of those with a first seizure will have a second within 2 years.

Question 11

A patient treated with deep brain stimulation (DBS) for generalized dystonia presents to your clinic 1 month after DBS with complaints that it is not effective. What would be the most appropriate next step?
a. Counsel patient.
b. Recommend genetic testing.
c. Recommendation implantation of additional leads.
d. Explant due to surgical failure.
e. Offer revision surgery.

Answer 11

Counsel patient.
Clinically significant improvement from GPi DBS for dystonia may not be noted for months, with some studies suggesting improvement at the three-month mark. Patients should be counseled that improvement might take months. Genetic testing, if done, should generally precede surgery.

Question 12

When considering targets for deep brain stimulation for a patient with advanced Parkinson’s disease, what is true about the expected benefits of stimulation of the subthalamic nucleus (STN) over the globus pallidus internus (GPi)?
a. Fewer neurocognitive side effects.
b. Superior motor outcomes in patients under age 50.
c. Greater medication reduction.
d. Greater longevity of therapy.
e. Greater reduction in overall disability score.

Answer 12

Greater medication reduction.
STN DBS is associated with greater medication reduction (30-40%) compared to GPi DBS (15-20%) in Parkinson’s disease patients. However, STN DBS often carries an increased risk of neurocognitive sequelae like impaired visuomotor processing and depression. Randomized trials suggest equal motor efficacy and impact on disability scores for both targets.

Question 13

A 23 year-old woman with complex partial seizures was initially treated with phenytoin, then switched to carbamazepine, and is currently on levetiracetam after failing the first two therapies. After a seizure-free period of six months upon starting levetiracetam, she now has recurrence of 2-3 seizures/month. What is the most appropriate next step in her management?
a. Evaluate for vagus nerve stimulation.
b. Add oxcarbazepine therapy with the levetiracetam.
c. Add phenytoin and oxcarbazepine to the current therapy with levetiracetam.
d. Switch to monotherapy with oxcarbazepine.
e. Evaluate for resective epilepsy surgery.

Answer 13

Evaluate for resective epilepsy surgery.
The patient meets criteria for drug-resistant epilepsy after failing two first-line anti-epileptic therapies. She is unlikely to become seizure-free with more medication trials. Evaluation for resective epilepsy surgery is the most appropriate next step, as chances of seizure-freedom are significantly higher if she is a candidate. VNS is an option if not a surgical candidate or if surgery fails.

Question 14

A 25 year-old female with medically refractory epilepsy undergoes vagus nerve stimulation (VNS). What is the expected clinical response to VNS in general?
a. 50% reduction in seizures in 50% of patients
b. Increase in seizure frequency in 50% of patients
c. No reduction in seizures but increased quality of life in most patients
d. 90% reduction in seizures in 90% of patients<
e. Complete cure of epilepsy in most patients

Answer 14

50% reduction in seizures in 50% of patients.
VNS is an approved therapy for medically refractory epilepsy in patients not suitable for resection or who have failed it. VNS typically results in about a 50% reduction in seizures in 50% of patients at 2 years. It does not cure epilepsy.

Question 15

The MOST common clinical feature of mesial temporal lobe seizures is:
a. Ictal bicycling movements.
b. Ipsilateral dystonic posturing.
c. Visual auras.
d. Ictal oral automatisms.

Answer 15

Ictal oral automatisms.
Oral automatisms (lip smacking, chewing, swallowing) are very common during mesial temporal lobe seizures. Bicycling movements are more common in frontal lobe seizures. Gustatory, olfactory, or epigastric auras are common in mesial temporal lobe seizures, not visual auras. Dystonic posturing is common but reliably contralateral.

Question 16

A 50 year-old man has a 15 year history of worsening bilateral (right worse than left) hand tremor that is worse with movement, such as writing and dressing. The tremor is partially alleviated with alcohol. He does not have a resting tremor or rigidity. Propranolol reduces the tremor, but side effects limit its utility. What is the most likely diagnosis?
a. Dyskinesia.
b. Orthostatic Tremor.
c. Essential Tremor.
d. Parkinson’s Disease.
e. Dystonic Tremor.

Answer 16

Essential Tremor.
The patient shows signs of Essential Tremor (ET), which is an action tremor, often bilateral but asymmetric, exacerbated by movement, and improved with alcohol and propranolol. For medically refractory ET, DBS of the VIM nucleus of the thalamus is the treatment of choice.

Question 17

A 34-year-old man with left temporal lobe epilepsy experiences a two-minute episode involving loss of consciousness and tonic stiffening of all four limbs, followed by relatively symmetric rhythmic convulsive jerking of the limbs. What type of seizure is this patient experiencing?
a. Simple partial.
b. Absence.
c. Complex partial.
d. Secondarily generalized.
e. Primary generalized.

Answer 17

Secondarily generalized.
The patient is having a secondarily-generalized tonic-clonic seizure. Convulsions can be primary-generalized or secondarily-generalized from a regional onset (like temporal lobe epilepsy). The ILAE now terms secondarily-generalized seizures as “focal to bilateral tonic-clonic” seizures.

Question 18

Patients who continue to seize after temporal lobectomy for temporal lobe epilepsy still experience an overall 70% reduction in seizure frequency. What sub-type of temporal lobe seizures in this patient population have an even higher rate of seizure control following temporal lobectomy?
a. Consciousness impairing seizures.
b. Consciousness sparing seizures.
c. Seizures with aura.
d. Seizures without aura.
e. Focal seizures.

Answer 18

Consciousness impairing seizures.
Patients failing temporal lobectomy but still having seizures experience larger reductions in consciousness-impairing seizures (73% for complex partial, 78% for generalized tonic-clonic) compared to consciousness-sparing seizures (65%). This results in an overall 70% decrease in total seizure frequency. Consciousness-impairing seizures are linked to increased morbidity.

Question 19

A 17-year-old cerebral palsy patient with spasticity undergoes intrathecal baclofen pump placement with significant objective improvement. 15 months later, he presents to the emergency department with recurrent spasticity. He is otherwise medically well, afebrile and without signs or symptoms of infection. What is the next most appropriate step in management?
a. Temporary intrathecal catheter placement for baclofen injection.
b. Radiopaque dye injection through the catheter access port.
c. Surgery for pump replacement.
d. Interrogation of the pump and review the programming.
e. AP and lateral radiographs of intrathecal catheter.

Answer 19

Interrogation of the pump and review the programming.
The first step in evaluating recurrent spasticity in a patient with a baclofen pump is to interrogate the pump and review its programming. Programming and refill errors, as well as catheter-related complications, are common causes of malfunction. AP/lateral radiographs are the initial radiographic evaluation for catheter issues.

Question 20

What is the best initial treatment for a 9 year old patient with diffuse bilateral dystonia secondary to cerebral palsy?
a. Selective dorsal rhizotomy.
b. Intramuscular botulinum injection.
c. Intrathecal baclofen.
d. Deep brain stimulation.
e. Section of Filum Terminale.

Answer 20

Intrathecal baclofen.
Intrathecal baclofen is the most effective treatment for widespread dystonia secondary to cerebral palsy among the options. Oral baclofen is a mainstay, and if ineffective, intrathecal administration via a pump is often beneficial. Selective dorsal rhizotomy is for spasticity, not dystonia. Intramuscular botulinum toxin is for isolated dystonia and can be problematic for widespread involvement.

Question 21

A 32 year-old male has intractable seizures localized to the left language-dominant supplementary motor area. What neurological deficit will most likely result following surgical resection?
a. Anomia and finger agnosia.
b. Temporary mutism.
c. Temporary paresis on the left.
d. Left inferior quadrantopsia.
e. Alexia without agraphia

Answer 21

Temporary mutism.
Resections in the language-dominant SMA often result in transient post-operative muteness, contralateral paresis (right-sided in this case), and neglect. These deficits usually resolve within days or weeks. Anomia and finger agnosia suggest Gerstmann’s Syndrome (dominant parietal damage).

Question 22

During the creation of a burr hole for a deep brain stimulator, an awake, sedated patient develops the sudden onset of coughing, hypotension, and hypoxia. This clinical presentation is most suggestive of which complication?
a. Aspiration pneumonia.
b. Tension pneumocephalus.
c. Seizure activity.
d. Intracranial hemorrhage.
e. Air embolism.

Answer 22

Air embolism.
Sudden vigorous coughing, unexplained hypoxia, and hypotension during burr hole creation in an awake patient may indicate venous air embolism. Precordial Doppler monitoring can aid early detection. Patient positioning and coughing are important predictors.

Question 23

Which of the following are the cardinal motor symptoms of Parkinson disease (PD) that are most amenable to treatment with deep brain stimulation (DBS)?
a. Dyskinesia, dystonia and freezing of gait.
b. Freezing of gait, rigidity and tremor.
c. Dyskinesia, bradykinesia and rigidity.
d. Bradykinesia, rigidity and tremor.
e. Dystonia, dyskinesia and tremor.

Answer 23

Bradykinesia, rigidity and tremor.
The cardinal motor symptoms of Parkinson’s disease (PD) are bradykinesia, rigidity, and tremor. These are the motor symptoms most responsive to DBS. Other symptoms like freezing of gait are typically less responsive to DBS. Dyskinesia is a side effect of levodopa, not a cardinal PD symptom, though DBS can improve it.

Question 24

A 74 year old man presents with progressive cognitive decline over one year, occasional visual hallucinations, poor attention, short-term memory loss, and bilateral upper extremity rigidity. What is the most likely diagnosis?
a. Dementia with Lewy bodies.
b. Alzheimer’s disease.
c. Multi-infarct dementia.
d. Wilson’s disease.
e. Pick’s disease.

Answer 24

Dementia with Lewy bodies.
Dementia with Lewy bodies (DLB) is characterized by dementia, extrapyramidal signs (rigidity), and psychosis (visual hallucinations). It’s the second most common degenerative dementia. Alzheimer’s is less likely due to the concomitant extrapyramidal signs and psychosis. Multi-infarct dementia has a stuttering progression. Pick’s disease is a frontotemporal dementia with early behavioral changes.

Question 25

A 50-year-old woman has 3 years of periodic right hand tremor aggravated by stress and caffeine, and improved with a glass of wine. The tremor impairs her ability to write, button her clothes, and drink. She is otherwise healthy and takes no medications. Her MRI is normal. What is the most appropriate next step in management? a. GPi deep brain stimulation. b. Propranalol. c. Reassurance that this is normal aging. d. Thalamic deep brain stimulation. e. Thalamotomy.

Answer 25

Propranalol. The patient has classic symptoms of essential tremor but hasn't tried medical therapy. Propranolol and primidone are first-line therapies for ET and should be trialed first. Surgical interventions are considered for medically refractory ET.

Question 26

In childhood epilepsy syndromes, patients with Lennox-Gastaut syndrome MOST often have the following EEG findings: a. Normal or discontinuous EEG activity b. Bilateral hypsarrhthmia c. Diffuse EEG slowing d. Triphasic spike and wave activity e. Multifocal slow spike and wave activity

Answer 26

Multifocal slow spike and wave activity. Lennox-Gastaut syndrome, typically affecting children 1-7 years old, is characterized by multifocal seizures and a background EEG with slow spike-wave activity. Hypsarrhythmia is characteristic of infantile spasms (West Syndrome). Triphasic spike and wave activity is seen in CJD.

Question 27

A 60-year-old generally healthy man with Parkinson disease (PD) is considering undergoing deep brain stimulation electrode implantation. He was diagnosed with PD 8 years ago and responded extremely well to levodopa therapy initially. Last year, he began to develop levodopa-related dyskinesias. Which of the following factors is most predictive of his response to deep brain stimulation a. Levodopa responsiveness. b. Age. c. Development of levodopa-induced dyskinesias. d. Duration of disease. e. Lack of medical comorbidities.

Answer 27

Levodopa responsiveness. Responsiveness to levodopa is the best predictor of DBS success for Parkinson's disease. Age and comorbidities might be relative contraindications but don't necessarily preclude DBS. Duration of disease and dyskinesias have not been directly associated with DBS outcomes, though early DBS after dyskinesia onset has shown superiority to medical therapy.

Question 28

A 27-year-old woman with a normal brain MRI experiences seizures characterized by jerking of the left hand which occasionally also spreads to the proximal arm. These episodes are nearly constant throughout the day and are not associated with any impairment of consciousness. Where are this patient's seizures likely originating? a. Supplementary motor area. b. Primary motor cortex. c. Prefrontal cortex. d. Occipital lobe. e. Temporal lobe.

Answer 28

Primary motor cortex. The patient has epilepsia partialis continua (EPC) with seizures likely from the right primary motor cortex (upper extremity area). Motor cortex seizures are commonly simple-partial and may spread along somatotopy ("Jacksonian march"). SMA seizures often show tonic "fencing" posturing.

Question 29

A 12-year-old boy presents with several years of dystonia in the lower extremities and recent development of dystonia in the upper extremities. His symptoms are mild in the morning but worsen as the day progresses. His dystonia does not improve with inactivity, but does improve after a full night's sleep. What is the most appropriate next step in his management? a. Low-dose oral levodopa therapy. b. Intrathecal baclofen therapy. c. Oral baclofen therapy. d. DBS of the globus pallidus interna (GPi). e. DBS of the ventral intermediate nucleus (VIM).

Answer 29

Low-dose oral levodopa therapy. This presentation suggests Dopa-responsive dystonia (DRD) or Segawa syndrome (DYT5), characterized by childhood-onset lower extremity dystonia with diurnal fluctuation. A dopamine response test (low-dose oral levodopa) is essential. DBS of GPi is for severe medication-refractory primary generalized dystonia, and a levodopa trial is required before considering DBS.

Question 30

A 58 year old man develops unilateral ballistic movements of his right upper extremity. An MRI shows a small infarct. What is the most likely location of the lesion? a. Globus pallidus internus. b. Thalamus. c. Putamen. d. Subthalamic nucleus. e. Substantia nigra.

Answer 30

Subthalamic nucleus. The patient has hemiballismus, classically caused by a lesion in the contralateral subthalamic nucleus (STN). A lesion to the STN reduces excitatory signals to GPi, decreasing its inhibition of the thalamus, leading to increased movement.

Question 31

Inferior extension of a therapeutic lesion placed in the posteroventral globus pallidus pars internus may induce which of the following adverse events: a. Hemiballism. b. Sensory loss. c. Ataxia. d. Hemiplegia. e. Visual field deficit.

Answer 31

Visual field deficit. The optic tract passes immediately below the posteroventral globus pallidus pars internus and can be injured during posteroventral pallidotomy, leading to a visual field deficit. Hemiballism is linked to STN lesions. Sensory loss can occur with thalamotomy lesion extension into the ventrocaudal nucleus. Hemiplegia can occur with pallidotomy lesions extending too far posteriorly or medially.

Question 32

Patient with subcortical band heteropia are characterized by: a. Infantile spasms. b. Subependymal giant cell astrocytomas. c. Male predominance. d. X-linked migrational disorder.

Answer 32

X-linked migrational disorder. Subcortical band heteropia is an X-linked migrational disorder that results in a second layer of subcortical gray matter in females. In males, it produces diffuse lissencephaly. Infantile spasms are characteristic of West Syndrome. Subependymal giant cell astrocytomas are characteristic of Tuberous Sclerosis.

Question 33

When performing invasive monitoring for epilepsy, when is stereo-EEG monitoring preferred over subdural grids and strips? a. Desire to reduce the need for continuous antibiotics. b. Greater than average skull thickness. c. Putative involvement of a functional network. d. Need to perform language mapping. e. Desire to perform resection during the same admission.

Answer 33

Putative involvement of a functional network. Stereo-EEG (SEEG) is preferred for investigating the three-dimensional propagation of seizure activity through a functional network, like the limbic network, due to the ability to place depth electrodes throughout. Subdural grids are advantageous for language mapping. Resection during the same admission is more common with subdural grids.

Question 34

Which of the following is the first line treatment for craniocervical dystonia (excluding oromandibular)? a. Botulinum toxin. b. Intrathecal baclofen. c. Selective peripheral denervation. d. Deep brain stimulation. e. Selective dorsal rhizotomy.

Answer 34

Botulinum toxin. Botulinum toxin is the first-line treatment for craniocervical dystonia (excluding oromandibular). It is currently the preferred primary therapy. Pallidal DBS is an effective alternative for failed medical therapy. Selective peripheral denervation is rarer now. Intrathecal baclofen benefits secondary dystonia and spasticity. SDR is for spasticity.

Question 35

Which anti-epileptic agents would be BEST to use in a patient receiving multiple other medications extensively metabolized by the liver? a. Phenobarbital (Luminal). b. Carbamazepine (Tegretol). c. Phenytoin (Dilantin). d. Levetiracetam (Keppra). e. Oxcarbazepine (Trileptal).

Answer 35

Levetiracetam (Keppra). Phenytoin, carbamazepine (and oxcarbazepine), and phenobarbital are potent inducers of hepatic enzymes, which can significantly affect the metabolism of other liver-metabolized drugs. Levetiracetam does not significantly induce hepatic enzymes and is a favorable alternative in such patients.

Question 36

Intraoperative test stimulation is being performed during a subthalamic nucleus deep brain stimulator placement. Stimulation at low voltages evokes dysconjugate gaze. The lead position is likely too far in which direction? a. Anterior. b. Lateral. c. Medial. d. Superficial. e. Posterior.

Answer 36

Medial. Stimulation-induced unilateral ocular deviation (dysconjugate gaze) suggests recruitment of oculomotor (III) fibers, indicating the active contact is too medial. The STN is lateral and dorsal to the oculomotor fibers. Lateral placement can cause contralateral motor symptoms; posterior stimulation can cause sensory symptoms.

Question 37

What language test represents the primary test for intra-operative assessment of language function during cortical stimulation mapping? a. Auditory responsive naming. b. Auditory sentence completion. c. Visual gesture naming. d. Visual object naming. e. Counting.

Answer 37

Visual object naming. Visual object naming is the gold standard for intra-operative language assessment during cortical stimulation mapping. This technique, typically using line drawings, assesses visual object recognition and language function and can reliably guide resection and predict long-term language deficits.

Question 38

Following left anterior temporal lobectomy for epilepsy, a patient develops right hemiplegia, hemisensory loss, and homonymous hemianopia. Which surgical complication is the most likely cause of her symptoms? a. Anterior choroidal artery injury. b. Damage to the cerebral peduncle. c. Middle cerebral artery injury. d. Post-operative hematoma. e. Damage to the thalamus.

Answer 38

Anterior choroidal artery injury. Damage to the anterior choroidal artery, causing an infarct, is a potential complication of temporal lobectomy and can result in contralateral hemiplegia, hemisensory loss, and hemianopia. Careful preservation of pial boundaries during mesial resection is important.

Question 39

An 8 year old boy with cerebral palsy and spastic quadriparesis presents to the emergency room with excessive somnolence. His baclofen pump was adjusted earlier today and his mother notes that he has remarkably little tone now. What is the best pharmacological treatment option? a. Naloxone. b. Physostigmine. c. Baclofen. d. Atropine. e. Flumazenil.

Answer 39

Physostigmine. Physostigmine can counteract the respiratory depressant effects of intrathecal baclofen overdose. Signs of baclofen toxicity include progressive somnolence, reduced muscle tone, and potentially respiratory arrest. Early recognition and airway management are crucial. The pump should be deactivated or emptied.

Question 40

After invasive grid monitoring and mapping of eloquent cortex, surgical resection is recommended for a 19-year-old man with drug-resistant epilepsy. The patient's seizures involve speech arrest with tonic posturing of the upper extremities in a "fencing posture". Where is the likely seizure focus? a. Primary motor cortex. b. Supplementary motor area. c. Primary sensory cortex. d. Mesial temporal lobe. e. Lateral temporal lobe.

Answer 40

Supplementary motor area. Tonic posturing of the limbs, often in a "fencing posture" (ipsilateral elbow extension, contralateral elbow flexion/shoulder abduction), is common in seizures originating in the supplementary motor area (SMA). Motor cortex seizures often have a "Jacksonian march." Temporal lobe seizures often involve automatisms.

Question 41

For which aspect of pain is the dorsal anterior cingulate cortex (dACC) responsible? a. Somatosensory. b. Psychosomatic. c. Affective. d. Nociceptive. e. Neurogenic.

Answer 41

Affective. The dorsal anterior cingulate cortex (dACC) is thought to be responsible for the affective component of pain – the emotional valence attached to the sensation. The pain experience has sensory, affective, and cognitive components. DBS of PAG/PVG and VPL/VPM thalamic nuclei are thought to reduce the sensory component of pain.

Question 42

When determining the initial programming settings for a patient with Parkinson disease who has undergone subthalamic nucleus deep brain stimulation, what is the most useful sign to determine the benefit of stimulation? a. Postural instability. b. Dyskinesia. c. Resting tremor. d. Rigidity. e. Bradykinesia.

Answer 42

Rigidity. Rigidity is the most useful sign for determining DBS stimulation benefits and adjusting settings initially because it doesn't fluctuate, responds quickly to changes, and is easily examined. Bradykinesia changes more slowly and is affected by fatigue. Resting tremor fluctuates, and dyskinesias are affected by medication.

Question 43

You are placing a deep brain stimulator in the GPi for Parkinson's disease. Your microelectrode has passed through the bottom of the GPi. What is the next structure to check for? a. Subthalamic nucleus. b. Substantia nigra reticulata. c. Optic tract. d. VPL thalamus. e. Globus pallidus externa.

Answer 43

Optic tract. The sensorimotor area of the pallidum lies directly over the optic tract. Exiting the GPi is marked by a transition through a lamina with slow tonic border cells, followed by a silent area. Flashes of light may be picked up by the microelectrode near the optic tract, or stimulation can cause phosphenes.

Question 44

When counseling a patient regarding temporal lobectomy for temporal lobe epilepsy, what is the most consistent and important predictor of a very favorable outcome after surgery? a. Duration of epilepsy > 20 years. b. Confirmation of laterality based on invasive monitoring. c. Male gender. d. Greater than 50 years old. e. Absence of generalized seizures preoperatively.

Answer 44

Absence of generalized seizures preoperatively. The absence of generalized seizures preoperatively is the most consistent and important predictor of a very favorable (Engel class I) outcome after temporal lobectomy. Abnormal preoperative imaging is also a predictor on univariate analysis, but only absence of generalized seizures remained significant on multivariate analysis. Duration >20 years is unfavorable.

Question 45

A 70-year old male presents with personality changes, gait instability, bradykinesia, slurring of speech, and difficulty moving the eyes. Which of the following imaging features is most consistent the diagnosis? a. PET scan showing asymmetrical reduced FDG-uptake in the cerebral hemispheres. b. PET scan showing reduced FDG-uptake in the frontal cortices, basal ganglia, and brainstem. c. MRI scan showing marked frontal and temporal cortical atrophy. d. PET scan showing decreased FDG-uptake in the cerebellum and cortex. e. CT scan showing enlarged ventricles.

Answer 45

PET scan showing reduced FDG-uptake in the frontal cortices, basal ganglia, and brainstem. The symptoms are suggestive of Progressive Supranuclear Palsy (PSP). [18F]FDG PET in PSP shows decreased metabolism in the midline frontal regions, basal ganglia, and brainstem. PD shows increased metabolism in basal ganglia/thalamus. MSA shows decreased metabolism in striatum, cerebellum, cortex (advanced). CBD shows asymmetric decreased cerebral metabolism. Frontotemporal atrophy suggests FTD/Pick's.

Question 46

A 36 year old woman with a known history of epilepsy treated with phenytoin presents in convulsive status epilepticus. After administration of lorazepam and supplementation with IV phenytoin, convulsions cease. Two hours later she remains unresponsive despite a normal head CT and normal serum electrolyte levels and blood counts. What is the most appropriate next diagnostic test? a. Angiogram. b. Lumbar puncture. c. MRI of the brain, including diffusion imaging. d. Electroencephalogram. e. Repeat serum sodium.

Answer 46

Electroencephalogram. A significant number of patients can have ongoing electrographic seizures (nonconvulsive status epilepticus) after apparent clinical resolution of convulsive status. Up to 14% of patients with persistent coma after convulsive status epilepticus may have nonconvulsive status epilepticus. Bedside continuous EEG is needed for rapid identification and treatment.

Question 47

A 50-year-old patient has a history of right-sided hemiparesis and recurrent seizures. On examination, he has a port-wine stain in the left V1 and V2 distribution. What is the most likely diagnosis? a. Encephalotrigeminal angiomatosis. b. Tuberculosis with leptomeningeal involvement. c. Meningioangiomatosis. d. Neurocutaneous melanosis. e. Hemorrhagic hereditary telangiectasia (Rendu-Osler-Weber syndrome).

Answer 47

Encephalotrigeminal angiomatosis. This is encephalotrigeminal angiomatosis (Sturge-Weber syndrome). MRI findings are consistent with chronic venous ischemia/occlusion. Neurocutaneous melanosis occurs in infancy, often in the posterior fossa. Meningioangiomatosis is a rare, focal hamartoma. Hereditary hemorrhagic telangiectasia is typically focal with flow voids.

Question 48

A patient is undergoing DBS lead placement in the subthalamic nucleus for Parkinson disease. During intraoperative testing the patient has good relief of symptoms but also notes facial pulling and transient paresthesias. Different monopolar and bipolar options produce the same result. Which of the following is the best option? a. Move the lead medially. b. Advance the lead deeper. c. Abort implantation on this side and move on to DBS implantation on the left side. d. Leave the DBS in its current location. e. Move the lead laterally.

Answer 48

Move the lead medially. Facial pulling indicates current spread to the internal capsule, which is lateral and anterior to the STN. This suggests lateral and/or anterior lead placement. Transient paresthesias suggest the lead is more posterior (not too anterior). Moving the lead medially will likely reduce current spread to the capsule.

Question 49

In childhood epilepsy syndromes, what is the most common EEG finding characteristic for patients with Lennox-Gastaut syndrome? a. Triphasic spike-and-wave. b. Bilateral hypsarrhthmia. c. Three per second spike-and-wave. d. Multifocal slow spike and wave. e. Normal or discontinuous.

Answer 49

Multifocal slow spike and wave. Lennox-Gastaut syndrome (children 1-7 yrs) is characterized by multifocal seizures and a background EEG of slow spike-wave activity. Triphasic spike-and-wave is seen in CJD. Hypsarrhythmia is characteristic of infantile spasms (West Syndrome). Three per second spike-and-wave is consistent with absence epilepsy.

Question 50

Orally administered baclofen may be useful in the treatment of the spasticity of cerebral palsy BECAUSE IT: a. Weakens muscles by inhibiting the release of calcium from sarcoplasmic reticulum. b. Has a specific beneficial effect on dystonia. c. Increases nor-adrenergic inhibition in the spinal cord. d. Has little sedative effect compared with other medications. e. Is a GABA agonist that inhibits presynaptic neurotransmitter release.

Answer 50

Is a GABA agonist that inhibits presynaptic neurotransmitter release. Baclofen is a GABA agonist acting on GABA-B receptors to inhibit presynaptic neurotransmitter release in alpha motor neurons, useful for spastic cerebral palsy. Its usefulness can be limited by sedative effects. Dantrolene weakens spastic muscles by inhibiting calcium release from sarcoplasmic reticulum. Oral baclofen is usually not helpful for dystonia.

Question 51

When screening a patient for DBS, what clinical finding most suggests an atypical parkinsonism rather than idiopathic Parkinson Disease? a. Drooling. b. Orthostatic hypotension. c. Reduced arm-swing and stride length on one side. d. Marked ataxia on finger-nose-finger testing. e. Masked facies.

Answer 51

Marked ataxia on finger-nose-finger testing. Ataxia is not seen in idiopathic PD but is a feature of the cerebellar subtype of multiple system atrophy (MSA-C). Patients with atypical parkinsonism ("Parkinson-plus" syndromes) are not good DBS candidates. Minimal initial levodopa response, rapid progression, and early falls are red flags for atypical parkinsonism. Autonomic dysfunction can occur in idiopathic PD. Drooling and reduced arm swing are signs of PD.

Question 52

Of the following, which symptom, associated with advanced Parkinson disease and/or its long-term medical treatment, is most reliably controlled by the therapeutic lesion depicted in the magnetic resonance image shown in Figure 1? a. Akinesia. b. Levodopa-induced dyskinesia. c. Tremor. d. Rigidity. e. Ataxia.

Answer 52

Levodopa-induced dyskinesia. The MRI shows a radiofrequency lesion in the posteroventral globus pallidus pars internus (PVP). PVP improves rigidity, tremor, and bradykinesia, but levodopa-induced dyskinesia is most reliably controlled by this intervention. Bilateral PVP has increased risk of speech/swallowing issues; DBS is preferred for bilateral symptoms.

Question 53

What is the most common major complication of stereoelectroencephalography (SEEG)? a. Electrode migration. b. Status epilepticus. c. Infection. d. Intracranial hematoma.

Answer 53

Intracranial hematoma. The most common major complication of SEEG is intracranial hemorrhage (1% incidence), usually as an intraparenchymal hematoma at the electrode site, or epidural/subdural hematomas. Encephalitis (0.4%) and status epilepticus (0.2%) are less common.

Question 54

Which of the following structures is BEST described as lateral to the hippocampal complex (hippocampus, subiculum and parahippocampal gyrus)? a. Fusiform gyrus. b. Oculomotor nerve. c. Brain stem. d. Ambient cistern. e. Posterior cerebral artery.

Answer 54

Fusiform gyrus. The fusiform gyrus is lateral to the hippocampal complex. The brain stem, oculomotor nerve, ambient cistern, and posterior cerebral artery are medial to the hippocampal complex.

Question 55

A 63 year old patient with levodopa-responsive Parkinson's disease is referred for deep brain stimulation surgery (DBS). With respect to motor symptoms, what benefit is DBS most likely to provide? a. Raise UPDRS III scores. b. Increase medication "on"-time with troublesome dyskinesias. c. Reduce freezing events. d. Improve gait. e. Decrease medication "off"-time.

Answer 55

Decrease medication "off"-time. DBS can decrease medication "off"-time and increase "on"-time without troublesome dyskinesias. Studies show DBS improves motor symptoms, including lowering UPDRS III scores (off medication), and improves quality of life. DBS is not consistently effective for gait disturbance and freezing events.

Question 56

A 55-year-old man is seen with early Parkinson disease manifested by tremor, rigidity, and bradykinesia. What is the preferred initial medication for treating this patient's Parkinson disease? a. Levodopa. b. Catechol methyltransferase inhibitor. c. Dopamine agonist. d. Combination of levodopa and an anticholinergic. e. Anticholinergic.

Answer 56

Dopamine agonist. For a 55-year-old with early PD, starting with a dopamine agonist may provide sustained relief with fewer motor complications compared to levodopa. Levodopa is highly effective but can lead to motor fluctuations and dyskinesias. Levodopa might be first-line for older patients. Anticholinergics have minor efficacy and cognitive side effects in the elderly. COMT inhibitors are used with levodopa.

Question 57

A 30 year-old undergoes an anterior temporal lobectomy for intractable epilepsy secondary to mesial temporal sclerosis. Postoperatively, the patient has new diplopia that improves over the next 4 months. Tilting his head to the right and tucking his chin improves his symptoms. Damage to what structure accounts for this complication? a. Trochlear nerve. b. Quadrigeminal plate. c. Posterior communicating artery. d. Oculomotor nerve. e. Meyer's loop.

Answer 57

Trochlear nerve. The symptoms (diplopia improving with head tilt away from affected eye and chin tuck) are consistent with a trochlear nerve (CN IV) palsy. CN IV injury is more commonly reported than CN III injury during mesial temporal lobe resection. CN IV palsy presents with hypertropia and extorsion of the affected eye.

Question 58

When performing deep brain stimulation surgery, what is the most likely clinical consequence of transgressing the ventricle? a. Intraventricular hemorrhage. b. Hemiparesis. c. Profound delirium. d. Coma. e. No effect.

Answer 58

No effect. Most ventricular wall transgressions during DBS procedures cause no adverse sequelae. While intraventricular hemorrhage (IVH) can occur, it is unlikely and rarely symptomatic. Transient post-operative confusion can occur with or without IVH. Neurological deterioration is unlikely.

Question 59

A 17 year-old female has chronic severe progressive tremor and dysphagia. She was recently hospitalized for an episode of psychosis. On examination she is dysarthric, drools, and has marked tremor with extension of her arms. Eye examination reveals yellow-brown granular deposits at the limbus of the cornea. What is the most likely diagnosis? a. Lesch-Nyhan Syndrome. b. Tardive dyskinesia. c. Hepatolenticular degeneration. d. Thyrotoxicosis. e. Sydenham Chorea.

Answer 59

Hepatolenticular degeneration. This is hepatolenticular degeneration (Wilson Disease), an autosomal recessive disorder of copper metabolism causing hepatic, neurologic (tremor, dysarthria, dystonia), and psychiatric symptoms. Kayser-Fleischer rings (copper deposits in cornea) are characteristic.

Question 60

A 55 year-old man presents with worsening short-term memory and confusion. On examination he appears depressed, has difficulty with fine motor skills such as finger tapping, and shows saccadic eye movements. His father died at age 60 after a prolonged illness with worsening rigidity and dementia. His MRI shows loss of striatal volume, with somewhat boxy lateral ventricles. He most likely has early symptoms of which disorder?a. Batten Disease (Neuronal Ceroid Lipofuscinosis). b. Mitochondrial encephalomyopathy. c. Spinocerebellar Ataxia. d. Huntington Disease. e. Parkinson Disease.

Answer 60

Huntington Disease. The symptoms (memory loss, confusion, depression, motor difficulties, saccadic eye issues), family history, and MRI findings (striatal atrophy) point to early Huntington's disease. It's an autosomal dominant disorder with chronic neurologic degeneration. Diagnosis is confirmed by genetic testing for expanded trinucleotide repeat.

Question 61

A 37-year-old female with long-standing epilepsy despite anti-epileptic medications and prior epilepsy surgery, reports to her physician that she has been seizure-free for 12 months. What is the most likely psychosocial pressure motivating the patient to report that she has been seizure-free for a 12-month period? a. Full-time employment. b. Holding an active driver's license. c. Financial independence. d. Commuting via public transportation. e. Living independently.

Answer 61

Holding an active driver's license. Driving (holding an active driver's license) is the psychosocial outcome that most significantly correlates with reporting seizure-freedom in the past 12 months. Other factors like full-time employment or living independently did not show a significant correlation in one study.

Question 62

Which MRI sequence and cut is most helpful in evaluating anatomical eligibility for endoscopic third ventriculostomy in patients?
Noncontrast T1-weighted sequence, axial cut. a. T2-weighted sequence, axial plane. b. Constructive interference in steady state (CISS), sagittal plane. c. Flair sequence, coronal cut. d. Noncontrast T1-weighted sequence, coronal cut.

Answer 62

Constructive interference in steady state (CISS), sagittal plane. CISS (or FIESTA) MRI sequences in the sagittal plane are most effective for assessing anatomical eligibility for ETV. These help identify aqueductal occlusive membranes and demonstrate premesencephalic cisternal anatomy to ensure adequate space anterior to the basilar artery.

Question 63

Which of the following is a significant risk factor for intraoperative seizure during awake craniotomy? a. Age > 60 years. b. History of seizures. c. Blood levels of antiepileptic drugs. d. Treatment with only a single antiepileptic drug.

Answer 63

History of seizures. Intraoperative seizures during awake craniotomy are more prevalent in younger patients, those with a history of seizures, patients on multiple AEDs, tumors in frontal lobes, and low-grade gliomas. AED type or blood levels did not correlate with intraoperative seizure incidence.

Question 64

A 10 year old child is referred to your interdisciplinary spasticity clinic. It is determined that he has spasticity as well as significant disabling dystonia in all four extremities, the face, and the neck. His symptoms have been medically refractory. What is the most appropriate treatment? a. Intrathecal baclofen therapy. b. Deep brain stimulation. c. Selective dorsal rhizotomy. d. Stereotactic pallidotomy. e. Botox injections.

Answer 64

Intrathecal baclofen therapy. For medically refractory widespread spasticity and dystonia in a child with cerebral palsy, intrathecal baclofen therapy can be highly effective for both upper and lower extremity dystonia, often at higher doses than for spasticity alone. Lumbar rhizotomy mainly treats lower extremity spasticity and is less effective for dystonia. Botox is unlikely to be effective for severe, widespread symptoms. GPi DBS is investigational for secondary dystonia.

Question 65

During deep brain stimulator implantation targeting the subthalamic nucleus, macrostimulation testing reveals good tremor control with low voltage stimulation but also parasthesias that resolve rapidly and contralateral facial pulling and wrist flexion at low voltages. Similar findings are noted with stimulation at all contacts. What is the most appropriate next step? a. The lead should be moved laterally away from the red nucleus. b. The lead should be moved anteriorly due to the observation of transient parasthesias. c. Implantation should be aborted due to narrow therapeutic window. d. Secure the electrode in the current position due to an adequate therapeutic window. e. The lead should be moved medially away from the internal capsule.

Answer 65

The lead should be moved medially away from the internal capsule. Contralateral facial pulling and wrist flexion at low voltages indicate current spread to the internal capsule, which is lateral and anterior to the STN. This suggests the lead is too lateral and/or anterior. Transient paresthesias are common and not an indication for lead movement if they resolve rapidly. Moving the lead medially (or posteriorly) should reduce capsular recruitment.

Question 66

Which seizure type responds the BEST to Vagus Nerve Stimulation (VNS) for medically intractable epilepsy? a. Secondary Generalized Seizure. b. Myoclonic Seizure. c. Atonic Seizure. d. Epilepsia Partialis Continua. e. Gelastic Seizure.

Answer 66

Atonic Seizure. Studies suggest higher response rates to VNS (approaching 80%) for atonic, absence, partial, and post-traumatic seizures. Younger patient age is also associated with better response. Response in complex partial and generalized seizures is lower (~50%).

Question 67

What visual field deficit is most common after right anterior temporal lobectomy for epilepsy? a. Left inferior quadrantanopsia. b. Bitemporal hemianopsia. c. Scotoma. d. Left superior quadrantanopsia. e. Left homonymous hemianopsia.

Answer 67

Left superior quadrantanopsia. A partial contralateral superior quadrantanopia ("pie in the sky") is the most common visual field deficit after anterior temporal lobectomy. This is due to damage to Meyer's loop fibers in the temporal lobe, which carry information from the contralateral superior visual field.

Question 68

In preparation for a frame-based needle biopsy, a stereotactic frame is applied to a patient's head and an MRI is obtained. After imaging, the frame is noted to have shifted due to poor fixation. What is the most appropriate next step in the management of this patient? a. Re-fix the frame and use a software correction algorithm before proceeding with surgery and stereotactic guidance; the software adjustment will correct for the change in frame position. b. Firmly re-affix the frame to the patient's skull in the same place, and proceed with surgery using stereotactic guidance; since the frame is in roughly the same position, it should still be accurate. c. Proceed with surgery without using stereotactic guidance; anatomical landmarks should be enough of a guide. d. Proceed with surgery with the loosened frame using the stereotactic guidance; the guidance will adjust accordingly. e. Re-fix the frame, re-image the patient, and then proceed with surgery using the updated stereotactic guidance.

Answer 68

Re-fix the frame, re-image the patient, and then proceed with surgery using the updated stereotactic guidance. If a stereotactic frame shifts after imaging, the frame must be re-affixed, and the patient must be re-imaged to ensure accuracy. Proceeding with a shifted or loosened frame, or relying on software correction or anatomical landmarks alone, is unsafe and inaccurate.

Question 69

A 50-year-old woman diagnosed with Parkinson disease 10 years ago presents with increasingly frequent periods of involuntary writhing movements of her hands and arms. What is the most likely cause of her abnormal movements? a. Parkinson-plus syndrome. b. Co-morbid Huntington disease. c. Tardive dyskinesia from taking antipsychotic medications. d. Non-compliance with Parkinson medications. e. Levodopa-induced dyskinesias.

Answer 69

Levodopa-induced dyskinesias
The involuntary, hyperkinetic, chorea-like movements are likely levodopa-induced dyskinesias (LID), a common complication in patients treated with levodopa long-term. These are temporally related to levodopa dosing (e.g., peak-dose dyskinesia). Increasing LID burden is an indication for DBS.

Question 70

A 19-year-old woman presents with intermittent episodes involving a motionless stare, lip smacking, dystonic posturing of the left arm, and unresponsiveness. What type of seizure is this patient experiencing? a. Atonic. b. Complex partial. c. Secondarily generalized. d. Simple partial. e. Aura.

Answer 70

Complex partial. The patient is experiencing a complex-partial seizure, with semiology consistent with mesial temporal lobe origin. Complex-partial seizures involve impaired consciousness and responsiveness. Simple-partial seizures have preserved consciousness. ILAE now terms these "focal seizures with or without impaired awareness."

Question 71

What imaging modality complements video-EEG, MRI and neuropsychological testing, in the standard pre-surgical evaluation of patients with medically intractable focal neocortical epilepsy? a. DAT scan. b. Resting state fMRI. c. Interictal PET imaging. d. Interictal SPECT imaging. e. Simultaneous EEG-fMRI.

Answer 71

Interictal PET imaging. Interictal PET imaging is a standard component of the pre-surgical workup for medically intractable focal neocortical epilepsy. Ictal onset zones are typically hypometabolic interictally. SPECT is usually acquired ictally. EEG-fMRI and resting state fMRI are investigational. DAT scans diagnose Parkinson's.

Question 72

In addition to higher pre-operative seizure frequency and the presence of generalized seizures, what factor best predicts less favorable seizure outcome in the surgical management of patients with focal neocortical epilepsy? a. Socioeconomic status. b. Patient age. c. Normal MRI. d. Laterality of seizure origin. e. Gender.

Answer 72

Normal MRI. A normal MRI (non-lesional cases) predicts poorer seizure outcomes in surgical management of focal neocortical epilepsy, as it makes identifying and completely removing the ictal substrate more difficult. Laterality, age, socioeconomic status, and gender have not been shown to influence seizure freedom rates.

Question 73

A craniotomy using frameless stereotactic guidance is planned. After the surgeon registers the patient and image, the fiducial reference frame moves in relation to the patient. Which of the following is the best option before proceeding with the operation?a. Ignore the shift if minor. b. Return the reference frame to its previous position. c. Re-register. d. Touch a skull reference point. e. Use a software correction algorithm.

Answer 73

Re-register. If the fiducial reference frame moves in relation to the patient after registration in frameless stereotaxy, repeat registration is necessary. Any change renders the registration inaccurate, and no software or shortcuts can correct this. Relying on inaccurate navigation can lead to surgical catastrophes.

Question 74

A lateral view of the cortical surface bordering the right sylvian fissure that is commonly exposed in a frontotemporal craniotomy is shown in Figure 1. The lower ends of the precentral (1) and postcentral (2) gyri are in the exposure. The supramarginal gyrus is indicated by what number? (Options are numbers on an image)

Answer 74

7. The supramarginal gyrus (7) wraps around the upturned posterior end of the sylvian fissure. The pars orbitalis (6), pars triangularis (5), and pars opercularis (4) form the inferior frontal gyrus. The precentral gyrus (1) is behind pars opercularis. The postcentral gyrus (2) is anterior to the supramarginal gyrus.

Question 75

Which of the following is a characteristic of primary generalized dystonia? a. The involvement of a single body part. b. The presence of a distinct underlying neuropathology. c. The presence of familial predisposition. d. The presence of bradykinesia. e. The presence of developmental delay.

Answer 75

The presence of familial predisposition. Primary generalized dystonia (PGD) involves more than one body part (e.g., trunk and at least 2 other limbs), typically lacks other neurological symptoms, has no distinct neuropathology, and often has a familial predisposition.

Question 76

When treating status epilepticus in adults, which intravenous medication should be co-administered with benzodiazepines? a. Propofol. b. Nitroprusside. c. Phenobarbital. d. Succinylcholine. e. Phenytoin.

Answer 76

Phenytoin. Intravenous phenytoin (18 mg/kg) should be co-administered with IV benzodiazepines (first-line, e.g., lorazepam) to provide longer-term seizure control in status epilepticus. Phenobarbital or propofol are used for refractory cases. Succinylcholine is for intubation and should not mask seizures. Nitroprusside is a hypotensive agent and can raise ICP.

Question 77

You are consulted about a patient who suffered a cardiac arrest secondary to an asthma attack. Hypothermia to 33° C has been initiated. CT reveals diffuse edema with slit ventricles, sulcal effacement, and obliteration of the basal cisterns. You suggest assessing median nerve somatosensory evoked potentials for aid in prognostication. Which SSEP component is most commonly measured to identify patients with a probable poor neurological outcome? a. Wave V. b. N13 (C5s). c. EP (Erb's point). d. Wave II. e. N20

Answer 77

N20. The N20 component, a negative deflection recorded from scalp electrodes ~20ms after median nerve stimulation, represents cortical post-synaptic activity. Its absence is relatively specific for poor outcome after cardiac arrest. N13 is a spinal potential; EP is brachial plexus activity. Waves II and V are part of BAER.

Question 78

A 42 year old male presents with a 5 year history of Parkinson disease (PD) with severe motor fluctuations. What would be his expected benefit from deep brain stimulation (DBS) surgery? a. DBS will result in reduction in progression of the natural history of Parkinson disease. b. DBS will provide improvement in motor symptoms comparable to the effects of Levadopa medication, with less motor fluctuations and without the medication-induced dyskinesia c. DBS will provide improvement in freezing of gait, a motor symptom frequently associated with Parkinson disease. d. DBS will eliminate the need for Parkinson medications. e. DBS will provide no benefit, because he has not had the disease long enough.

Answer 78

DBS will provide improvement in motor symptoms comparable to the effects of Levadopa medication, with less motor fluctuations and without the medication-induced dyskinesia. DBS provides motor symptom benefit comparable to levodopa "ON-time" effects but without dyskinesias and with fewer motor fluctuations. Medications can be reduced (20-40%) but usually not eliminated. DBS improves levodopa-responsive symptoms (bradykinesia, rigidity, tremor) but not typically freezing of gait or non-motor symptoms. DBS does not alter PD's natural history.

Question 79

The patient whose magnetic resonance image is shown in Figure 1 most likely underwent surgery to control which of the following symptoms associated with which disease: a. Tremor associated with Essential Tremor. b. Levodopa-induced dyskinesia associated with Parkinson disease. c. Torticollis associated with cervical dystonia. d. Akinesia associated with Parkinson disease. e. Rigidity associated with Parkinson disease.

Answer 79

Tremor associated with Essential Tremor. The MRI shows a DBS lead in the ventral intermediate (VIM) nucleus of the thalamus. This target is used for treating medically refractory Essential Tremor (ET). While it can treat PD tremor, it has little effect on other PD motor symptoms like akinesia or rigidity, for which STN or GPi are preferred. GPi DBS treats torticollis in cervical dystonia.

Question 80

A 65-year-old presents with difficulty initiating movements, rigidity, and a mild resting tremor. Which of the following neurotransmitters is likely deficient in this patient? a. Norepinephrine. b. Acetylcholine. c. Serotonin. d. GABA. e. Dopamine.

Answer 80

Dopamine. Bradykinesia, rigidity, and tremor are cardinal features of Parkinson's disease, which involves dopamine depletion due to loss of dopaminergic neurons in the substantia nigra. Acetylcholine deficiency is associated with Alzheimer's. Serotonin and norepinephrine deficiency are linked to depression.

Question 81

What is the FDA Human Device Exemption (HDE) approved target for deep brain stimulation for medically refractory obsessive compulsive disorder? a. Subgenual cingulate gyrus. b. Ventral capsule/ventral striatum. c. Anterior cingulate gyrus. d. Medial forebrain bundle. e. Subthalamic nucleus.

Answer 81

Ventral capsule/ventral striatum. DBS is FDA HDE approved for obsessive compulsive disorder (OCD) targeting the ventral anterior internal capsule and ventral striatum. Subgenual cingulate gyrus (Area 25) is a target for depression. Anterior cingulate gyrus is an ablative target for pain/OCD. STN is FDA approved for PD. Medial forebrain bundle is investigational.

Question 82

Parkinsonian tremor can be diminished by high frequency stimulation of: a. Cingulate bundle. b. Subcaudate white matter. c. Dorsomedial thalamus. d. Globus pallidus externa. e. ViM thalamus.

Answer 82

ViM thalamus. Tremor cells are found in the VIM thalamus, GPi (not externa), and STN. Stimulation of these reduces tremor. GPi and STN also improve other PD symptoms, so are often chosen for PD tremor. Cingulum bundle and subcaudate white matter are psychosurgery targets. Dorsomedial thalamus is part of the limbic thalamus.

Question 83

An eight-year old male of Ashkenazi Jewish descent presents with generalized dystonia, refractory to all medications. A similar condition has been noted in a number of his first-degree relatives. Which bilateral procedure is the BEST option for surgical treatment of this condition? a. Thalamic deep brain stimulation. b. Pallidal deep brain stimulation. c. Subthalamic deep brain stimulation. d. Thalamotomy. e. Pallidotomy.

Answer 83

Pallidal deep brain stimulation. The patient likely has inherited idiopathic generalized dystonia (often DYT1 mutation). Deep brain stimulation of the globus pallidus internus (GPi) has shown dramatic clinical efficacy, especially in children, with efficacy improving over time. Bilateral pallidal lesions have serious side effects. STN DBS is for PD; thalamic (Vim) DBS is for essential tremor.

Question 84

A 24 year-old man has refractory complex partial seizures localized to the left temporal lobe with EEG. MRI is consistent with left mesial temporal sclerosis. What is the most appropriate next step in his management? a. Vagal nerve stimulation. b. Corpus callosotomy. c. Intracarotid amytal test. d. Temporal lobectomy. e. Invasive EEG monitoring.

Answer 84

Intracarotid amytal test. With concordant EEG and MRI suggesting a left temporal focus (mesial temporal sclerosis), an intracarotid amytal test (Wada test) is needed before temporal lobectomy to evaluate language and memory dominance. This helps determine if the contralateral hippocampus can support memory post-surgery. Invasive EEG isn't needed with concordant findings. VNS is less desirable if a surgical candidate. Corpus callosotomy is for atonic seizures.

Question 85

Which syndrome is most likely to benefit from hemispherectomy? a. Lennox-Gastaut Syndrome. b. Nonlesional Extratemporal Epilepsy. c. Mesial temporal Sclerosis. d. Rasmussen's Encephalitis. e. Drop attacks.

Answer 85

Rasmussen's Encephalitis. Rasmussen's Encephalitis, characterized by intractable epilepsy and progressive hemiparesis, is classically treated with hemispherectomy. Mesial temporal sclerosis responds to mesial temporal resection. Nonlesional extratemporal epilepsy has a discrete focus. Lennox-Gastaut is a childhood epilepsy with characteristic EEG. Medically refractory drop attacks are treated by corpus callosotomy.

Question 86

You are evaluating a cerebral palsy patient with spastic paraplegia for intrathecal baclofen pump placement. What is the mechanism of action of intrathecal baclofen? a. It facilitates presynaptic inhibition from Ia sensory afferents to alpha motor neurons, reducing excitatory output. b. It reduces presynaptic inhibition from Ia sensory afferents to alpha motor neurons, enhancing excitatory output. c. It reduces postsynaptic excitation of alpha motor neurons, reducing excitatory output. d. It reduces gamma motor neuron activity. e. It facilitates presynaptic excitation from Ia sensory afferents to alpha motor neurons, enhancing excitatory output.

Answer 86

It facilitates presynaptic inhibition from Ia sensory afferents to alpha motor neurons, reducing excitatory output. Intrathecal baclofen (a GABA-B agonist) facilitates presynaptic inhibition of the monosynaptic reflex pathway from Ia sensory afferent fibers to alpha motor neurons, thereby reducing alpha motor neuron output and spasticity.

Question 87

A 63 year old woman with Parkinson disease undergoes unilateral deep brain stimulator (DBS) electrode implantation. 3 months later, she returns to the office with redness, pain, swelling and purulence from the cranial incision. Culture grows methicillin-sensitive S. aureus. What is the most appropriate management? a. Removal of the implantable pulse generator (IPG) only. b. Removal of the intracranial lead only. c. Removal of the entire hardware system with antibiotics. d. Removal of the implantable pulse generator (IPG) and extension with antibiotics. e. Antibiotics only.

Answer 87

Removal of the entire hardware system with antibiotics. With evidence of a deep surgical site infection (purulence from subcutaneous layer) involving implanted DBS hardware, the most appropriate management is removal of the entire hardware system along with targeted antibiotic treatment. Superficial SSIs might be treated with antibiotics to try and salvage hardware, but deep SSIs almost always represent hardware infection requiring complete explant.

Question 88

Anatomic hemispherectomy historically is associated with what complication? a. Syndrome of the trephined. b. Hemibalismus. c. Superficial cerebral hemosiderosis. d. Callosal syndrome. e. Gerstmann syndrome.

Answer 88

Superficial cerebral hemosiderosis. Anatomic hemispherectomy is historically associated with superficial cerebral hemosiderosis, a delayed symptom complex (hearing loss, ataxia, dementia, pyramidal signs) believed to result from repeated hemorrhages into the resection cavity. Functional hemispherectomies were developed to avoid this.

Question 89

A patient with a baclofen pump presents with a temperature of 104 degrees F and hyperreflexia. What is the next appropriate treatment? a. Cooling blanket. b. Intrathecal baclofen administration. c. Intravenous dantrolene. d. Broad spectrum antibiotics. e. Intravenous acetaminophen.

Answer 89

Intrathecal baclofen administration. Hyperthermia and hyperreflexia in a patient with a baclofen pump suggest baclofen withdrawal. Acute withdrawal can cause symptoms similar to neuroleptic malignant syndrome. Treatment involves replacement of baclofen (intrathecal is more bioavailable than oral) or administration of benzodiazepines.

Question 90

A five-year-old right-handed boy has had medically intractable epilepsia partials continua for 4 years. Evaluation demonstrates developmental delay, worsening of verbal IQ and right-sided hemiparesis. EEG demonstrate several epileptogenic foci over the left parietal lobe. MRI imaging is shown. Aside from invasive monitoring, which of the following is the most appropriate surgical treatment? a. Selective amygdalohippocampectomy. b. Vagus nerve stimulation. c. Parietal lobectomy. d. Functional hemispherectomy. e. Corpus callosotomy.

Answer 90

Functional hemispherectomy. The presentation (intractable epilepsia partialis continua, developmental delay, worsening VIQ, hemiparesis, EEG findings, and hemispheric atrophy on MRI) is consistent with Rasmussen's encephalitis. Functional hemispherectomy (disconnection surgery) is indicated to control seizures and preserve language/cognitive development, with 70-80% seizure freedom success.

Question 91

The current model of basal ganglia physiology suggests that the 'negative symptoms' of Parkinson disease (i.e., rigidity and bradykinesia) are attributable to which of the following physiological events: a. Inhibitory input to the globus pallidus pars internus (GPi) via the direct pathway from the striatum is decreased. b. Glutamatergic input from the VL thalamus to the supplementary motor area is decreased. c. The hyperactive GPi hyperinhibits the ventrolateral (VL) nucleus of the thalamus. d. Excessive glutamatergic input from the disinhibited subthalamic nucleus results in GPi hyperactivity. e. All of the above.

Answer 91

All of the above. In PD, decreased dopaminergic input to the striatum leads to an imbalance. The net result is hyperactivity of both STN and GPi. Consequently, GPi hyperinhibits the VL thalamus, reducing its glutamatergic output to the SMA, accounting for rigidity and bradykinesia. All listed physiological events contribute to these negative symptoms according to the model.

Question 92

What is the most common side effect of vagus nerve stimulation? a. Dyspnea. b. Dyspepsia. c. Wound infection. d. Voice hoarseness. e. Cardiac arrhythmia.

Answer 92

Voice hoarseness. Hoarseness or tremulousness of the voice is the most common side effect of VNS, typically occurring during stimulation to some degree in every patient and reported as an adverse event in 37-66% of patients. It is usually well-tolerated. Dyspnea was reported in 5.6-23% of patients. Infection around the VNS device is less common (e.g., 3 of 198 patients).

Question 93

What deep brain structure is the most appropriate deep brain stimulation target for chronic nociceptive pain? a. Periaqueductal grey. b. Globus pallidus internus. c. Subthalamic nucleus. d. Anterior limb of the internal capsule. e. Ventral posterolateral nucleus of the thalamus.

Answer 93

Periaqueductal grey. For pharmacoresistant nociceptive pain, the periventricular/periaqueductal gray area (PAG) is traditionally the optimal DBS target. Ventral posteromedial (VPM) and posterolateral (VPL) thalamic nuclei are preferred for neuropathic pain. PAG DBS may exert effects via opioidergic or autonomic mechanisms.

Question 94

Which ONE of the following statements concerning essential tremor is TRUE? a. A patient with tremor that begins after age 40 years should be tested for Wilson disease. b. The tremor of hyperthyroidism can be distinguished from essential tremor by recording devices that measure tremor frequency. c. The presence of head tremor in addition to tremor of the extremities should make one question the diagnosis of essential tremor. d. Thalamic stimulation of the ventralis intermedius nucleus for unilateral tremor is preferred over thalamotomy because the effects of the latter last for only several years. e. Primidone is probably somewhat superior to propanolol in treating essential tremor, but it causes more side effects in some patients and a significant number of patients do not respond to either treatment.

Answer 94

Primidone is probably somewhat superior to propanolol in treating essential tremor, but it causes more side effects in some patients and a significant number of patients do not respond to either treatment. Essential tremor is an action tremor (4-12 Hz). Head, trunk, voice, and legs can be involved. Wilson disease should be considered in young patients with tremor. Primidone and propranolol are first-line; primidone may have more side effects initially but slightly more long-term efficacy. 25-55% don't respond to these drugs. Thalamic stimulation (VIM) is preferred for medically unresponsive tremor; thalamotomy is as effective but has more side effects.

Question 95

Each of the following physiological techniques has been used reliably to confirm correct anatomical targeting during surgery within the basal ganglia except: a. Impedance monitoring. b. Macroelectrode stimulation. c. Single-cell microelectrode recording. d. Motor evoked potentials. e. Bipolar semi-microelectrode recording.

Answer 95

Motor evoked potentials. Motor evoked potentials (MEPs) are primarily used to monitor corticospinal tract function during brainstem or spinal cord surgery, not as a localizing tool during basal ganglia surgery. Impedance monitoring, macroelectrode stimulation, single-cell microelectrode recording, and bipolar semi-microelectrode recording have all been used to confirm anatomical targeting in the basal ganglia.

Question 96

Approximately what percentage of patients experience favorable outcomes (Engel Class I) 3-5 years after temporal lobectomy for temporal lobe epilepsy? a. 30%. b. 70%. c. 90%. d. 10%. e. 50%.

Answer 96

0.7. Approximately 70% of patients report favorable outcomes (Engel Class I) 3-5 years after temporal lobectomy for temporal lobe epilepsy. This rate may decrease to about 60% after 10 years.

Question 97

What is a known possible side effect of corpus callosotomy for epilepsy? a. Hemiplegia. b. Gerstmann syndrome. c. Superficial cerebral hemosiderosis. d. Hemibalismus. e. Disconnection syndrome.

Answer 97

Disconnection syndrome. Disconnection syndrome (also known as callosal or split-brain syndrome) is a known possible side effect of corpus callosotomy. It involves the inability to name objects recognized by one hemisphere when information cannot cross to the language-dominant hemisphere. Superficial cerebral hemosiderosis is associated with anatomic hemispherectomies. Gerstmann syndrome from dominant parietal lesions. Hemiballismus from STN injury.

Question 98

A 54 year-old male had a good result from left-sided thalamotomy for essential tremor 10 years ago. He now wishes to have his other side treated. What is the MOST appropriate surgical procedure for his contralateral side? a. Subthalamic DBS. b. Thalamic DBS. c. Pallidotomy. d. Pallidal DBS. e. Thalamotomy.

Answer 98

Thalamic DBS. While both thalamotomy and thalamic DBS are effective for essential tremor, bilateral thalamic lesions (thalamotomies) are associated with a higher incidence of permanent speech deficits. Therefore, a contralateral thalamic DBS is recommended if the other side needs treatment after a prior thalamotomy. Pallidal and subthalamic targets are for PD and dystonia.

Question 99

What is the most important factor in determining whether a patient with a history of seizures is allowed to drive? a. Discretion of the treating physician. b. History of seizure surgery. c. Seizure-free off medications. d. Seizure-free interval. e. Number of anti-epileptics.

Answer 99

Seizure-free interval. The most important factor is the seizure-free interval, which varies by state (typically 3 months to 2 years). Physicians generally counsel patients on risks but do not grant/restrict driving privileges. A significant percentage of patients with refractory epilepsy have experienced seizure-related motor vehicle accidents.