Neuro Flashcards
An infant has a sacral dimple. What would make you more concerned about spinal dysraphism? Slate-gray nevus over dimple Located 2 cm from anal verge 3 mm in diameter Located above the gluteal cleft
Located above the gluteal cleft
ess than 0.5 cm in diameter, are typically benign with little or no clinical significance. In contrast, sacral dimples that are deep and large (greater than 0.5 cm), fall within the superior portion or above the gluteal crease (>2.5 cm from the anal verge), or are associated with other cutaneous markers for NTDs (eg, hypertrichosis, and discoloration
18 month old boy with first episode febrile seizure. What factor influences likelihood of repeat febrile seizure?
a. MRI result
b. EEG result
c. Family history of febrile seizures
d. Etiology of fever
family hx
Febrile seizures recur in 30% of those experiencing a first episode, 50% after 2 or more episodes, and 50% if <1 year old at onset. Several factors affect recurrence rate:
MAJOR factors
Age < 1yo
Duration of fever <24 hours (short onset between fever and seizure)
Fever 38-89 degrees Celsius (seizure occurred at lower temperature)
MINOR factors
Family history of febrile seizures
Family history of epilepsy
Complex febrile seizure
Male gender
Lower sodium at time of presentation
11 month old baby boy is admitted because yesterday morning before breakfast he had two episodes where he “flopped back” and seemed hypotonic. This resolved. This morning after breakfast, he had another episode where he flopped back, became hypotonic, and had irregular breathing. His blood glucose is normal, as was a CBC and electrolytes. Which of the following would be next step?
a. EEG (as long as child is well)
b. Lumbar puncture
c. Pyruvate, lactate, fasting blood glucose
EEG
Tonic and atonic seizures are more common than, but not necessarily always associated with, Lennox-Gastaut syndrome. Atonic seizures (usually called drop attacks) consist of a sudden loss of postural tone. In some patients, the drop attacks are preceded by one or more clonic jerks. In mild forms, the child may have a brief head drop (forward flexion of head and neck)
5 month old boy with episodes of head flexion and limb extension lasting a few seconds. His EEG shows a disorganized background with multifocal spikes. What is the most likely diagnosis?
a. Benign familial neonatal convulsions
b. Infantile spasms
c. GERD
d. Benign myoclonus
IS
The clinical presentation of infants ages 3 to 9 months includes spasm like seizures that involve flexion, extension, or mixed flexion-extension of the arms, legs, and trunk. The background or interictal EEG is chaotic, with a characteristic pattern called hypsarrhythmia. The classic pattern of hypsarrhythmia consists of very high voltage, random, slow waves and spikes in all cortical areas. Other interictal patterns in patients with IS include focal or multifocal spikes and sharp waves,
BFN - benign - normal EEG
A child has symptoms suggestive of Guillain Barre syndrome and has a lumbar puncture. What are you most likely to find on the CSF?
a. High WBCs, low protein
b. High WBC, high protein
c. Low WBC, low protein
d. Low WBC, high protein
low WBC, high protien
Previously healthy 2 yo presents with 2 weeks proximal muscle weakness. Most likely disorder: congenital myopathy neuromuscular junction peripheral neuropathy Spinal cord injury
congenital myopahty
Newborn found to have cardiac rhabdomyoma. What syndrome is this associated with?
Tuberous Sclerosis
NF-
TS need 2 major and 1 minor. Major Features of TS Cortical tuber Subependymal nodule Subependymal giant cell astrocytoma Facial angiofibroma/forehead plaque Ungual/periungual fibroma (non-traumatic) Hypomelanotic macules (>3) Shagreen patch Multiple retinal hamartomas Cardiac rhabdomyoma Renal angiomyolipoma Pulmonary lymphangioleiomyomatosis
MINOR Cerebral white matter migration lines Multiple dental pits Gingival fibromas Bone cysts Retinal achromic patch Confetti skin lesions Nonrenal hamartomas Multiple renal cysts Hamartomous rectal polyps
2. A child with epilepsy presents unwell with findings of hepatotoxicity and pancreatitis and thrombocytopenia. Which medication is this likely related to? Valproic acid Carbamazepine Topiramate Keppra
vpa
Nausea, vomiting, alopecia
Weight gain, metabolic syndrome
Thrombocytopenia
Hepatotoxicity, pancreatic toxicity
Kid 4 yo comes in with torticollis and rigidity. Mom says she has been giving enema for illness for past several days but doesn’t know what it is. Which medication will you give immediately?
Diphenhydramine
Diazepam
Dystonic Acid-
treatment of acute dystonia with antihistamine or anticholinergic medications is usually rapidly effective [25,26]. Parenteral diphenhydramine (1 to 2 mg/kg per dose, maximum dose 50 mg) is used most frequently and typically results in resolution of an acute dystonic reaction
7 year old migraine, 3-4 x/month. Management: A. Ibuprofen as needed B. Propranalol prophylaxis C. Referral to neurologist D. Brain MRI
ibuprofen
then tryptan
(more than 1/week), or there is more than 1 debilitating HA per month (missing school, home or social activities), preventative prophylactic therapy is warranted. - amytryptilline
Child with concussion. When can she return to play?
Back at school full time with no symptoms and no accommodations
After symptom free for 7 days
ssx free 7-10d
Child with in-toeing and difficulty walking. What would make you suspicious that this is spastic diplegic CP?
Prematurity, global developmental delay, normal reflexes
Prematurity, delayed motor milestones, increased reflexes
Birth asphyxia, global developmental delay, normal reflexes
Birth asphyxia, delayed motor milestones, increased reflexes
Prematurity, delayed motor milestones, increased reflexes
Spastic diplegia is most commonly associated with premature infants with PVL. Infants with CP caused by an acute intrapartum hypoxic-ischemic event are more likely to have spastic quadriparesis or dyskinetic CP than other subtypes of CP.
Kid presents with 3rd febrile seizure in the past month. Otherwise well, no post-ictal. What do you do? Reassure EEG Admit MRI brain
reassure
as long as simple
In children with complex febrile seizures, the need for an EEG depends on several factors and clinical judgement. A short, generalized seizure repeated twice in 24 hours is, by definition, complex but would not necessitate an EEG
Young child with hypotonia, only some facial movements, no deep tendon reflexes. +Fascicultations on tongue. Parents are asking about chance of recurrence if they have more children. What do you tell them?
a) 25%
b) 50%
c) 50% if the child is male
d) Sporadic
25%
The cause of SMA is genetic as an autosomal recessive mendelian trait: SMN1 gene on chromosome 5q13.2
14 year old girl with asthma has throbbing headaches associated with nausea, photophobia. Her mother has a history of migraines. What treatment do you offer for prophylaxis? Amitriptyline Propranolol Phenytoin Sumatriptan - acute management Ergotamine - acute management
Amitriptyline
The most commonly used preventative therapy for headache and migraine is amitriptyline.
Propranolol is contraindicated for use in children with asthma or allergic disorders (blocks action of beta agonists). It is also contraindicated in children with diabetes (can facilitate hypoglycemia). Caution should be used in adolescents as their is an increased incidence of depression.
Anticonvulsants that are used include valproic acid, topiramate, keppra, gabapentin. (NOT phenytoin)
A 2 month old baby is floppy. He smiles and looks around, but he does not make many voluntary movements. On exam, he has no deep tendon reflexes. What is the most likely diagnosis?
a. Congenital muscular dystrophy
b. Congenital myotonic dystrophy
c. SMA
SMA
SMA 1 - Affected infants may appear normal before the onset of symptoms, but soon develop a severe, symmetric flaccid paralysis with absent DTRs, and never achieve the ability to sit unsupported. Because the upper cranial nerves are mostly spared, patients usually have an alert expression, furrowed brow, and normal eye movements. Weakness of the bulbar muscles results in a weak cry, poor suck and swallow reflexes, pooling of secretions, tongue fasciculations,
A 7 yo boy has become ataxic over the last few months, gradually worsening. What is the probably diagnosis?
a. Adrenoleukodystrophy
b. Encephalitis
ALD - restrict VLCD
ALD is an X-linked disorder. It presents between four and eight years of age (peak seven years). Affected males have one of three main phenotypes and can present from childhood through adulthood: childhood cerebral, adrenomyeloneuropathy (20-40 yrs old), Addison only disease. Boys typically present with learning disabilities and behavior problems that are often diagnosed as attention deficit hyperactivity disorder
A 2 month old baby has an abnormally shaped head. What would make you pursue further investigations?
a. Posterior displacement of the ipsilateral ear
b. Frontal bossing of the ipsilateral forehead
posterior displacement of ipsilateral
Lambdoidal craniosynostosis: Premature closure of unilateral lambdoid suture, posterior displacement and low set ipsilateral ear, contralateral frontal bossing
18-month-old boy presents with fever, AOM, and febrile seizure. This is his third recurrent febrile seizure in the past few months. What is your next step?
a) EEG
b) CT head
c) Neuro consult
d) Reassure parents
reassure parents
Teenage girl with asthma has frontal headaches associated with nausea, photophobia. Her mom has a history of migraines. What treatment do you offer for prophylaxis?
a. sumatriptan
b. propranolol
c. amitriptyline
d. Phenytoin
amitriptyline
. Girl walked and tripped on a curb now has cold extremity painful to touch
a) CRPS
b) osteomyelitis
CRPS
Myotonic infant with fasiculations, low reflexes. Parents wonder chance of recurrence?
a) 25% recur
b) 50%
c) 50% males
d) Sporadic
25% recur
Child previously well has ascending hyporeflexia, paralysis. Cause?
a) Campylobacter
Guillain-Barre Syndrome (UTD)
Antecedent infections are common with GBS, and are thought to trigger the immune response that leads to acute polyneuropathy. Usually respiratory tract or gastrointestinal infections. Campylobacter infection is the most commonly identified precipitant (as many as 30 percent of cases). Others include CMV, EBV, Mycoplasma pneumoniae, and influenza-like illnesses. Typically develops 2-4 weeks after the infection.
Brachial plexus injury-extended hand, pronated, but can grip. Which nerve roots
a) C3-C4
b) C5-C6
c) C7-C8
d) C8-T1
c5-6
Phrenic nerve palsy (C3-5): diaphragmatic paralysis, respiratory distress
Erb’s Palsy (C5-C6/7): Adduction and internal rotation of shoulder, elbow extended, forarm pronated, wrist and fingers flexed (preserved grasp)
Klumpke palsy (C8-T1): claw-like deformity, paralyzed hand, absent grasp, biceps reflex intact (may have Horner syndrome because of T1 involvemet)
Total brachial plexus injury (C5-T1): total paralysis of arm/hand and diaphragm, associated with Horner syndrome
3mo girl with hypotonia, hypotonic facies, 1+ symmetric DTR. What is the most likely diagnosis?
a. Congenital muscular dystrophy - typically have contractures at birth
b. Myotonic dystrophy - reflexes preserved
c. SMA1 - no facial nerve involvement
d. Nemaline rod
Nemaline rod myopathy (UTD): The clinical expression of nemaline myopathy is variable. The presentation in affected newborns can be severe or relatively mild. In the former, profound generalized weakness and hypotonia involving the face, bulbar, and respiratory muscles is seen; the eye muscles are spared. The milder form, with relatively less facial weakness and diaphragm impairment, also can present in children or adults.
14y girl presents with blurry vision, morning headaches and vomiting for 2 weeks. She has bilateral papilledema. No mention of meds. What’s the most likely dx? with normal imaging
a. Posterior fossa tumor
b. Pseudotumour cerebri
Pseudotumour cerebri: idiopathic intracranial hypertension (> 280 mmHg in an obese child or > 250 mmHg in a non obese child) with a normal CSF cell count, protein content and normal MRI (normal to slightly decreased ventricular size, normal ventricular anatomy). Papilledema should be universally present in children with a closed fontanelle.
cause: by hypervitaminosis A, prolonged steroid
Treatment: treat the underlying cause (discontinue the medication, weight loss in obese children), acetazolamide 10-30mg/kg/day
Complication: optic nerve atrophy/blindness.
9mo girl who preferentially uses left hand. Right hand is fisted. When try to sit her up, she keels over to the right. What is the explanation for the findings?
a. Erb’s palsy
b. Trauma to right arm
c. Hemiplegia
d. Spastic diplegia
hemiplegia
infant w hypopigmented macules and repeated flexion movements of the entire body. EEG shows a disorganized pattern. What is the diagnosis?
a. Infantile spasms
b. Benign myoclonus of infancy
c. Benign sleep myoclonus
INFANTILE SPASM
- ACTH first line
- but if u have tuberous scleoriss or downsyndrome- ALSO use vigabitran
9y boy w episodic r side face twitching and drooling at night with preserved consciousness. What is the most likely diagnosis?
a. Rolandic epilepsy
b. Juvenile myoclonic epilepsy
c. Tourette syndrome
Rolandic Epilepsy
Also called benign childhood epilepsy with centrotemporal spokes
Starts during childhood, age 3-10 years and outgrown in adolescence
Clinical manifestation
Child wakes from sleep due to a simple partial/focal seizure
Tingling of throat/cheek
Tonic or clonic contractions of 1 side of the face with drooling and inability to speak but preserved consciousness and comprehension
EEG: broad based centrotemporal spikes that are markedly increased in frequency during drowsiness and sleep
Normal MRI
Good response to AEDs including carbamazepine.
Juvenile Myoclonic Epilepsy (Janz syndrome)
AGE
BODY PART INVOLVED
EEG changes?
Juvenile myoclonic epilepsy (Janz syndrome) is the most common generalized epilepsy in young adults, accounting for 5% of all epilepsies. Typically, it starts in early adolescence with 1 or more of the following manifestations: myoclonic jerks in the morning, often causing the patient to drop things; generalized tonic–clonic or clonic–tonic–clonic seizures upon awakening; and juvenile absences. Sleep deprivation, alcohol (in older patients), and photic stimulation, or, rarely, certain cognitive activities can act as precipitants. The EEG usually shows generalized 4-5 Hz polyspike–and–slow-wave discharges
10 year old boy previously healthy has been waking up the parents at night with symptoms of mouth twitching, and drooling. He has a normal neurological exam and a normal MRI. Which of the following is most likely on his EEG?
a. Centrotemporal Spikes
b. 3 Hz spike and wave
c. high amplitude waves and a background of irregular spikes
centrotemporal spikes
Prophylactic treatment for migraines in child with asthma
a. Propranolol
b. Amitriptyline
c. Sumatriptan
amitripytline
can cause QTc
14 year old boy with Duchenne’s, who is in a wheelchair, has recently seen his FVC fall from 30% to 21% predicted. What symptom will he most likely complain of?
a. Headache early in morning
b. Headaches in the afternoon
c. Tingling of his fingers
d. Dyspnea with exertion
AM headache
15 yr old girl has a headache, then syncope at school for several minutes. She is brought to hospital. Can’t walk because of numbness in her legs. Exam is normal, plantar reflexes normal, DTR normal. Initial loss of sensation to L4, the next day she has sensation to her ankles. Able to walk without ataxia leaning heavily on your hands, feet spaced 8 cm apart. What is your next step in management?
a. EEG
b. MRI head and spine
c. Confrontation and explanation that her symptoms are not organic
d. Refer to PT
PT refer
Boy with recurrent pharyngitis. Episodic jerking of shoulders and head. Most likely diagnosis?
a. Rheumatic fever
b. Transient tic disorder
c. Tourettes
d. ?? Some sort of seizure disorder
transient tic
10 yr old boy with 6 wk history of repetitive movements of head and shoulders. Has history of recurrent strep pharyngitis. What is the most likely diagnosis?
a. Tourettes
b. Transient tic disorder
c. partial seizures
d. acute rheumatic fever
transient tic d/o
Tourette disorder (TD), persistent (chronic) motor or vocal tic(PTD), and provisional tic disorders (Table 24-1) are characterized by involuntary, rapid, repetitive, single or multiple motor and/or vocal/phonic tics that wax and wane in frequency but have persisted for more than 1 year since first tic onset (<1 year for provisional tic disorder)
Symptoms must be present for a minimum of 1 year but can involve motor or vocal tics
5 month old baby who has intermittent flexion of arms/legs for several seconds and in between that, has normal behavior. EEG – abnormal background with polyspikes. What is diagnosis?
a. benign neonatal myoclonus
b. infantile spasms
c. benign sleep myoclonus
IS
with hypsarythmia
Infantile spasms have over 200 known causes including HIE, Tuberous sclerosis, TORCH Infections, encephalitis with HSV, Down Syndrome
3-9 months of age with spasm like seizures, involve flexion, extension of the arms, legs and trunk
The background EEG is chaotic with characteristic pattern called hypsarrhythmia
High dose ACTH is the gold standard for termination of spasms and resolution of hypsarrhythmia on EEG. Vigabitran is the treatment of choice for children who have TS or Down Syndrome x 6 months.
Writing difficulties and moody after a URTI
a. Tourettes
b. Sydenham’s chorea
c. Lupus
d. Huntingtons
Sydenham’s chorea
Chorea: rapid, chaotic movements that seem to flow from 1 body part to another. Affected individuals exhibit motor impersistence, with difficulty keeping the tongue protruded (“darting tongue”) or maintaining grip (“milkmaid grip”). Chorea tends to occur both at rest and with action. Patients often attempt to incorporate the involuntary movements into more purposeful movements, making them appear fidgety. Chorea increases with stress and disappears in sleep
5 yo child wakes up to tell mom that he has been drooling, and facial movements in the middle of the night. Aware of events. What likely finding on EEG?
a. Centrotemporal spikes
b 3 hz
rolandic epilepsy
3hz is absence seziure
Child with proximal weakness, calf hypertrophy. Suspect Duchenne’s. What is the best test?
a. molecular studies
b. serum dystrophin
c. biopsy
d. EMG
biopsy
biopsy is the best, genetics is the first
Polymerase chain reaction (PCR) for the dystrophin gene mutation is the primary test, if the clinical features and serum CK are consistent with the diagnosis. If the blood PCR is diagnostic, muscle biopsy may be deferred, but if it is normal and clinical suspicion is high, the more specific dystrophin immunocytochemistry performed on muscle biopsy sections detects the 30% of cases that do not show a PCR abnormality.
cant detect DUPLICATIONS then need biopsy
6 month old baby with decreased reflexes, fasciculation’s, weak muscles. Best diagnosis?
a. MRI
b. Thyroid test
c. Genetic testing
d. Metabolic testing
genetcs
9 year old child with spastic quadriplegia cerebral palsy presents with a three day history of not swallowing her secretions. On baclofen, fluticasone for asthma. Looks well. Has G-tube and has been fed with thickened feeds orally. Last week received botox for lower leg contractures. Continues to tolerate po feeds and abdo exam is normal. On assessment today he is afebrile, with drooling and clear chest, no stridor. Remaining vital signs are normal. Improved movement of lower legs. Your next step is:
a. pH probe
b. Admit for observation
c. Outpatient follow up regarding dysphagia
d. Cine video swallowing fluoroscopic study
admit obs
Kid with CP presents with difficulties swallowing. She is tolerating her GT feeds but has difficulty managing her oral secretions. Of note, she got Botox injections to her legs last week. On exam, she has oral secretions and her legs feel less hypertonic. What do you do:
a. pH probe
b. do a swallow study
c. observe in hospital
swallow study
Infant with hypopigmented lesions over body and brief flexion/extension of upper and lower body periodically throughout the day. Baby is mildly developmentally delayed. How would you confirm the underlying diagnosis:
a. EEG
b. Urine OA
c. Head MRI
head MRI
Child with suspicion of Duchenne’s muscular dystrophy. Best test to confirm diagnosis: [repeat]
a. Molecular studies
b. Muscle biopsy
c. Muscle Enzyme studies
d. Serum dystrophin assay
biopsy (BEST
First is genetic)
A 1 month old has a droopy lower left lip. The forehead moves normally. What is the problem:
a. centrally mediated facial nerve palsy
b. peripherally mediated facial nerve palsy
c. congential absence of the mouth angle depressor muscle
d. Mobius syndrome
congential absence of the mouth angle depressor muscle
Congenital absence of the depressor angularis oris muscle
often associated with other anatomical defects particularly of the heart. Not a facial nerve lesion but a cosmetic defect that does not interfere with feeding.
IN FACIAL NERVE CENTRAL - ALSO u have forehead sparing, but UPPER and lower lip is involved and eyes cant close
Drooping mouth but eyes were able to close, normal nasolabial folds. Diagnosis?
a. Mobius syndrome
b. congenital absence of the depressor anguli oris muscle
c. CN VII lesion
d. upper lobe lesion
congenital absence of the depressor anguli oris muscle
A kid is having involuntary facial tics. What would NOT strengthen a diagnosis of Tourette’s?
a. Positive family history of OCD -
b. Hyperactivity (ADHD) -
c. Tics lasting for 6 months -
d. Male -
C tic for 6 monnth
A kid presents with facial tics. What would support a diagnosis of Tourette’s?
a. tics are present for 6 months
b. family history of tics
c. attention-deficit, hyperactivity disorde
50% with tourete have ADHD
A kid is having involuntary facial tics. What would MOST strengthen a diagnosis of Tourette’s?
a. Positive family history of OCD -
b. Hyperactivity (ADHD) -
c. Tics lasting for 6 months -
d. Male -
ADHD
A 7 year old girl has had episodes over the past couple months where she awakes from sleep, has twitching of her right lip and is unable to verbalize. The episodes last 1-2 minutes. You suspect seizure activity and order and EEG. What is it likely to show?
a. spikes in the centrotemporal (rolandic) region
b. Generalized slow waves
c. 3 Hz generalized spikes over a normal background
d. normal
centrotemporal
A 14 year old girl presents to the ER with abdominal pain rated 8/10. Soft abdomen on exam, no rebound, tender but not worse with palpation. She is also complaining of a headache. She states she’s had 4 similar episodes in the past which resolved over hours. Likely diagnosis?
a. abdominal migraine
b. appendicitis
c. PID
abdo migraine
paroxysmal
vomitting
matenral hx of ha
