Neurology Board Review - Pediatrics

Front 1

EEG of prematurity

Back 1

Delta brushes (asynchronous)
tracé discontinu

Front 2

Infantile seizures (vitamin deficiency)

Back 2

Vitamin B6

Front 3

Likelihood an autistic child will have seizures

Back 3

20-25%

Front 4

Aicardi's syndrome

Back 4

- Agenesis of the corpus callosum
- Retinal lacunes
- Vertebral abnormalities
- In girls
- Seizures (infantile spasms)

Front 5

Valproate in children, adverse reactions

Back 5

- Hepatic failure with encephalopathy (AST may be normal, may treat with L-carnitine)
- Thrombocytopenia

Front 6

Neonatal myasthenia

Back 6

- 10 % of infants born to mothers with myasthenia
- Hypotonia and poor suckling, weak cry, rare respiratory distress
- Responds well to acetylcholinesterase inhibitors

Front 7

Ataxia-telangectasia

Back 7

- AR
- Ataxia
- Variable immunodeficiency syndromes with pulmonary infections
- Ocular and cutaneous telangectasia
- malignancies (ALL or lymphoma)

Front 8

Congenital myotonic dystrophy

Back 8

- Children of mothers with myotonic dystrophy
- Hypotonia
- Orthopedic deformities
- Facial diplegia and fish mouth

Front 9

Infant botulism

Back 9

- EMG shows incremental response
- Treat with ventilatory support, BIG-IV

Front 10

Benign neonatal sleep myoclonus

Back 10

- Affects infants 1-6 months of life
- Myoclonic jerks occurring only during sleep
- Abrupt cessation upon awakening

Front 11

Landau-Kleffner syndrome

Back 11

- Infantile acquired aphasia
- Onset between ages 3 and 7
- Loss of previously acquired language milestones
- May have clinical seizures or elictrographic status epilpepticus of sleep
- Onset after 6 has a better prognosis

Front 12

Meningocele of spinal cord

Back 12

- Often has lipoma associated

Front 13

Neonatal seizures

Back 13

- First 24 hours: HIE
- 24-72 hours: IVH for premies, SAH and contusion for term babies, spesis for both
- 72 hours: inborn errors of metabolism

Front 14

Vein of Galen malformation

Back 14

- Cardiac failure due to high output

Front 15

Most common cause of congenital hemiparesis

Back 15

- Stroke
- Maternal factors include hypercoagulable disorders, cocaine abuse, and placental complications
- Neonatal conditions include congenital heart disease, hypercoagulable disorders, meningitis, and systemic infection

Front 16

Benign paroxysmal torticollis

Back 16

- Recurrent episodes of head tilt, accompanied by vomiting, ataxia, and pallor.
- 12 months to 5 years old
- A type of migraine, risk for basilar migraine is increased

Front 17

Hydrancephaly

Back 17

- Near absence of the cortex and basal ganglia
- Loss of anterior circulation, with possibly some preservation of the posterior circ structures
- Fatal.

Front 18

Dopa- responsive dystonia

Back 18

- Worsening symptoms at end of day, better with sleep
- Complete relief with small amounts of L-dopa
- Enzyme deficiency is GTP cyclohydrolase
- Chromosome 14
- Enzyme is needed to make cofactor in tyrosine hydroxylase
- BH4 levels in CSF reduced

Front 19

Infant intractable seizures and low CSF glucose

Back 19

- Glut-1 DS (glucose transporter type 1)
- Progresses to atxia, small head, developmental delay, spasticity

Front 20

Infarction of basal ganglio

Back 20

- Seen most commonly with varicella associated strokes
- Varicella angiopathy
- More subject to recurrent TIAs

Front 21

Breath holding spells

Back 21

- Pallid: response to painful stim, little crying
- Cyanotic: Response to anger or frustration, crying with cyanosis.

Front 22

Infant botulism

Back 22

- Children under 12 months
- Constipation, weak sucking, hypotonia, weak cry
- EMG shows incremental response (looks like adult Lambert-Eaton)
- Soil or dust inhalation most common cause

Front 23

Newborns spend what % of time in REM sleep

Back 23

- 50%
- Higher in premature babies
- Declines to 25% by age 2

Front 24

Treatment for tourette's

Back 24

- Haldol, pimozide (neuroleptics)