Neuro

Question 1

What is Erb-Duchenne palsy?

Answer 1

Can be due to a difficult delivery where traction is applied. It affects the fifth and sixth cervical nerves; the affected arm cannot be abducted or externally rotated at the shoulder, and the forearm cannot be
supinated

Question 2

What is Klumpke paralysis?

Answer 2

Can be due to a difficult delivery where traction is applied. Injury to the seventh and eighth cervical and first thoracic nerves results in palsy of the hand and also can produce Horner syndrome.

Question 3

What should you think about in a child with a subacute disorder of the CNS that produces cranial nerve abnormalities (especially of cranial nerve VII and the lower bulbar nerves), long-tract signs, unsteady gait secondary to spasticity, and some behavioral changes?

Answer 3

Pontine glioma

Question 4

What is the presentation of metachromatic leukodystrophy?

Answer 4

Deficiency of galactosyl-3-sulfateceramide sulfatase. It has its onset between 1 and 2 years of age and is notable for progressive ataxia, weakness, and peripheral neuropathy. In this disorder, gray macular lesions can be seen that look somewhat similar to cherry-red spots.

Question 5

What is the presentation of Fabry’s disease?

Answer 5

Reduced activity of a-galactosidase, presents in older children as acroparesthesia (numbness or tingling in one or more extremities), intermittent painful crises of the extremities or the abdomen, frequently low-grade fevers, and sometimes cataracts.

Question 6

What enzyme is deficient in Niemann-Pick disease?

Answer 6

Sphingomyelinase

Question 7

How are concussions classified?

Answer 7

Grade 1 (mild) concussions have no loss of consciousness and a return to baseline in less than 15 minutes. Grade 2 (moderate) concussions also have no loss of consciousness, but include confusion lasting longer than 15 minutes. Grade 3 (severe) concussions include any loss of consciousness.

Question 8

How are reflexes affected in Tay-Sachs vs Niemann-Pick disease?

Answer 8

Infants have hyperreflexia in Tay-Sachs and arefelxia in Niemann-Pick

Question 9

What comorbidities are patients with absence seizures at risk for?

Answer 9

ADHD and anxiety

Question 10

What should you be concerned for in a child with trauma to the pharynx (e.g. fall with object like toothbrush or pencil in mouth) and a gradual onset of hemiplegia?

Answer 10

Carotid artery dissection (internal)

Question 11

What is the treatment for Guillain-Barre syndrome in children?

Answer 11

IVIG.

Plasmapheresis has not been sufficiently tested in children and should only be used in rapidly progressing disease.