Pediatric neurology II

Question 1

what are the characteristics of lennox-gastaut syndrome?

Answer 1

• different types of seizures including tonic seizures, atypical absence, complex partial and myoclonic seizures
• mental retardation
• characteristic EEG: less than 2.5 spike and slow wave discharges

Question 2

what is the characteristic EEG finding with lennox-gastaut syndrome?

Answer 2

less than 2.5 spike and slow wave discharges

Question 3

when do neonatal seizures occur?

Answer 3

within first month of life

Question 4

why do neonates NOT have generalized seizures?

Answer 4

incomplete myelination

Question 5

neonatal seizures are typically of what types?

Answer 5

• focal tonic
• focal clonic
• myoclonic

Question 6

what are the 4 most common etiologies of neonatal seizures?

Answer 6

• hypoxic ischemic encephalopathy
• CNS infection
• intracranial bleeding
• brain malformation

Question 7

what is the treatment for neonatal seizure?

Answer 7

• phenobarbital (first line)

- fosphenytoin (second line)

Question 8

what is the workup for neonatal seizures?

Answer 8

• glucose, Ca, Mg, electrolytes
• CSF analysis
• imagine: MRI (ischemia, stroke, bleeds)

Question 9

how do you differentiate jitteriness vs seizure?

Answer 9

jitteriness:
- tremor-like
- induced by stimulation
- stopped by holding extremity

Question 10

what is guillain-barre syndrome?

Answer 10

• immune-mediate acute inflammatory demyelinating polyneuropathy
• sudden onset

Question 11

which virus is commonly associated with guillain-barre syndrome?

Answer 11

c. jejuni

Question 12

what characterizes the paralysis in guillain-barre syndrome?

Answer 12

ascending

Question 13

what does CNS analysis show for guillain-barre syndrome?

Answer 13

increased protein with normal WBC count

Question 14

what is the most specific and sensitive test which shows signs of demyelination in guillain-barre syndrome?

Answer 14

nerve conduction study

Question 15

what is seen on MRI in guillain-barre syndrome?

Answer 15

nerve root enhancement

Question 16

what is the treatment for guillain-barre syndrome?

Answer 16

IVIG or plasmaphoresis

Question 17

are steroids helpful in guillain-barre syndrome?

Answer 17

no

Question 18

what is the prognosis for guillain-barre syndrome in children?

Answer 18

generally excellent with complete recovery within months

Question 19

what is the inheritance of duchenne muscular dystrophy? which gene is mutated?

Answer 19

• X linked recessive

- dystrophin

Question 20

how does duchenne muscular dystrophy usually present?

Answer 20

• by age 3
• proximal muscle weakness affecting legs before upper extremities
• wheelchair bound by age 12
• dilated cardiomyopathy and conductino abnormalities

Question 21

calf hypertrophy and gower’s sign are classic findings for what condition?

Answer 21

duchenne muscular dystrophy

Question 22

what is the most common cause of death in duchenne muscular dystrophy? when does death usually occur?

Answer 22

respiratory failure in early 20s

Question 23

what is the treatment for duchenne muscular dystrophy?

Answer 23

• daily small dose of prednisolone or deflazacort

- improve muscle strength and respiratory function

Question 24

what lab test is highly informative for duchenne muscular dystrophy diagnosis? what is the level?

Answer 24

• CPK

- high

Question 25

what is acute ataxia?

Answer 25

unsteadiness of walking or fine movements of less than 72 hours duration

Question 26

what are the 3 most common causes of acute ataxia in children? which is most common?

Answer 26

- post infectious cerebellar ataxia (most common) - intoxication - guillain barre syndrome