Pediatric neurology III

Question 1

what is cerebral palsy?

Answer 1

static motor encephalopathy due to an insult to the developing brain in the first 3 years

Question 2

what are the classical physical findings for cerebral palsy?

Answer 2

axial hypotonia (head lag, truncal hypotonia)

Question 3

what are the classifications of cerebral palsy?

Answer 3

• spastic hemiplegia
• spastic diplegia
• spastic quadriplegia
• dyskinetic

Question 4

what are the features of spastic hemiplegia?

Answer 4

• one side of body is affected and usually the arm more than the leg
• unilateral perinatal stroke is a classic cause

Question 5

what are the features of spastic diplegia?

Answer 5

• both legs only affected

- classically seen in premature infants with periventricular leukomalacia

Question 6

what are the features of spastic quadriplegia?

Answer 6

all extremities affected

Question 7

what are the features of dyskinesic CP?

Answer 7

athetoid, chorioathetoid and dystonic

Question 8

what are the different types of complicated migraines?

Answer 8

• hemiplegic (unilateral)
• basilar (vertigo, diplopia, nystagmus, dysarthria)
• opthalmoplegic (occulomotor palsy)
• confusional

Question 9

what are the commonly used prophylaxis medications for migraines?

Answer 9

• cyproheptadine (antihistamine): for younger children
• topiramate (antiepileptic): obese children and children with epilepsy
• amitriptyline (TCA): children with depression
• propanolol (BB): contraindicated in asthma

Question 10

what are the side effects of:

• cyproheptadine
• topiramate
• amitriptyline

Answer 10

• cyproheptadine: increased appetite and somnolence
• topiramate: weight loss, renal stone, cog impairment
• amitriptyline: sleepiness and arrhythmia (rec EKG)

Question 11

what is the inheritance of neurofibromatosis?

Answer 11

AD

Question 12

what are the diagnostic criteria for NF1?

Answer 12

2/7 of:

• 6 or more cafe au lait spots
• 2 axillary or inguinal freckling
• 2 neurofibroma or 1 complex neurofibroma
• optic glioma
• 2 lisch nodules (iris hamartoma)
• sphenoid dysplasia or long bone abnormalities
• first degree relative with NF1

Question 13

what is the inheritance of tuberous sclerosis?

Answer 13

AD

Question 14

what are the major criteria for tuberous sclerosis diagnosis?

Answer 14

• skin: hypomelanotic macule (ASH LEAF), facial angiofibroma (SHAGREEN PATCH), ungual and periungual fibroma
• CNS: subependymal nodules, cortical tubers, giant cell astrocytoma
• eye: retinal hamartoma
• heart: cardiac rhabdomyoma
• kidney: renal angiomyolipoma
• lungs: lymphangioleiomyomatosis

Question 15

what is sturge weber syndrome?

Answer 15

non inherited disorder characterized by devellopment of angiomas mainly in the brain, skin, and eyes

Question 16

what is the presentation for sturge weber syndrome?

Answer 16

• port wine stain affecting V1 and V2 trigeminal distribution (usually unilateral)
• CNS: leptomeningeal angiomas - seizures, hemiparesis, cognitive delay of various degree, calcified meninges
• eyes: unilateral glaucoma