Neuro

Question 1

Port wine stain with ipsilateral ventral dilation, calcifications and cerebral atrophy

Answer 1

Sturge-Weber

Question 2

Most common association with myelomeningocele

Answer 2

Chiari II formation

Question 3

Best diagnostic imaging of strokes in infants

Answer 3

MRI with MRV

Question 4

What diseases can moyamoya be associated with?

Answer 4

Trisomy 21, sickle cell disease, collagen vascular disease, and neurofibromatosis type 1

Question 5

Truncal ataxia, horizontal nystagmus, difficulty wiht fine motor control, tremors, dysarthria - often after a viral infection (varicella, echovirus and coxsackie). Diagnosis of exclusion

Answer 5

Acute cerebellar ataxia

Question 6

Chaotic random eye movements and ataxia with myoclonic jerks concerning for neuroblastoma

Answer 6

Opsoclonus-myoclonus-ataxia syndrome

Question 7

Progressive distal weakness, mild to moderate sensory loss, depressed or no tendon reflexes and high-arched feet. Presents in 1st or 2nd decade

Answer 7

Charcot-Marie-Tooth

Question 8

Biconvex shaped presenting with initial decreased LOC followed by lucid interval and then rapid deterioration

Answer 8

Epidural hematoma

Question 9

Crescent shaped with gradually increasing confusion and headaches

Answer 9

Subdural hematoma

Question 10

Cluster headache treatment

Answer 10

100% oxygen as abortive therapy. Triptans can also be effective. Verapamil for preventative

Question 11

3Hz spike and wave pattern on EEG

Answer 11

Absence epilepsy

Question 12

4-6Hz polyspike and wave pattern

Answer 12

Juvenile myoclonic epilepsy

Question 13

Anti-epilpetics that do NOT cause enzyme induction of OCPs

Answer 13

Levetiracetam, ethosuximdie, gabapentin, zonisamide or valproic acid

Question 14

Descent of cerebellar tonsils (and rarely medulla) into the foramen magnum

Answer 14

Chairi I malformation

Question 15

Descent of cerebellar vermis, 4th ventricle and medulla into foramen magnum

Answer 15

Chiari II malformation

Question 16

Sudden onset progressive weakness and multimodality sensory distubrances in lower extremities

Answer 16

Transverse myelitis

Question 17

MRI with spinal cord swelling and hyperintensity on T2 weighted imaging with mild lymphocyte pleocytosis and slightly elevated protein in CSF

Answer 17

Transverse myelitis

Question 18

Next steps with lumbosacral dimple with overlying hair tuft

Answer 18

Needs further imaging and work up

Question 19

Flexor spasms of arms, necks, legs followed by sudden extension of neck, arms, trunk and legs

Answer 19

Infantile spasms

Question 20

high voltage, irregular, slow waves on EEG that appear out of sync and over all head regions

Answer 20

EEG in infantile spasms

Question 21

medication for infantile spasms

Answer 21

ACTH and/or oral vigabatrin

Question 22

Bilateral stenosis/occlusion of carotid arteries with prominent arterial collaterals “puff of smoke”

Answer 22

Moyamoya

Question 23

What is moyamoysa associated with?

Answer 23

NF1, trisomy 21, sickle cell disease and cranial irradiation

Question 24

Seizures in a 4-6 day old that are brief and infant returns to baseline in between with normal exam, CSF and lab imaging

Answer 24

Benign neonatal seizures. Completely resolve within 24-48 hours of onset without any long term sequelae

Question 25

Nighttime episosdes of facial twitching with difficulty speaking that sometimes evolve into generalized shaking

Answer 25

Benign rolandic epilepsy

Question 26

Centrotremporal spikes on EEG

Answer 26

Benign rolandic epilepsy

Question 27

Ataxia, lower extremity weakness, cardiomyopathy, hypoactive to absent DTRs

Answer 27

Friedrich ataxia

Question 28

Seizure disorder which presents in adolscence, can initially be misdiagnosed as tics. Jerks noted typically in the early morning most frequently. Can later develop generalized clonic episodes

Answer 28

Juvenile myoclonic epilepsy

Question 29

What is the most common presentation of neonatal stroke?

Answer 29

Seizures