PreTest Pediatrics: "The Neuromuscular System"

Question 1

Describe ADEM.

Answer 1

• Acute demyelinating encephalomyelitis
• Presents with multiple-sclerosis-like symptoms
• Usually preceded by a viral infection
• Treatable with corticosteroids
• Mortality high! (10% to 30%)

Question 2

A child with poor growth and early thromboembolic events likely has a deficiency in which enzyme?

Answer 2

Cystathionine beta-synthase

This describes homocystinuria.

Question 3

A child with a long, narrow skull with an absent fontanelle likely has _______________.

Answer 3

craniosynostosis

Question 4

A child with ptosis, a flat facial expression, and dysphagia may have _______________.

Answer 4

myasthenia gravis

Although more common in older people, there is a juvenile form.

Question 5

Hemiparalysis with ocular deviation to the opposite side is suggestive of ______________.

Answer 5

acute infantile hemiplegia (a form of childhood stroke)

Question 6

How can you differentiate between myotonic dystrophy and muscular dystrophy based on the child’s movement?

Answer 6

Myotonic dystrophy presents with slow/sluggish movements because the child cannot relax fast enough.

Question 7

True or false: SSPE does not present with motor symptoms.

Answer 7

True

It is primarily characterized by dementia and behavioral changes.

Question 8

Brisk reflexes, hypertonicity, and bulbar nerve anomalies are potential signs of ______________.

Answer 8

pontine gliomas

Question 9

True or false: nystagmus is not a contraindication to LP.

Answer 9

False

Question 10

Those with ____________ are more likely to get brain abscesses.

Answer 10

heart defects

Question 11

A cherry-red spot in the eye with hepatomegaly can represent a defect in sphingomyelinase or _______________.

Answer 11

beta-galactosidase

Question 12

A child with a poor suck reflex and tongue fasciculations may have ______________.

Answer 12

spinal muscular atrophy

Question 13

A headache that worsens during the day and with cognitive strain is likely a _____________.

Answer 13

tension headache

Question 14

Explain the exam finding of “milkmaid’s grip”.

Answer 14

Irregular contractions of the hand muscles when gripping the examiners fingers

A sign of Sydenham chorea

Question 15

What is PHACE syndrome?

Answer 15

• Posterior fossa malformations
• Hemangiomas
• Arterial anomalies
• Coarctation of the aorta
• Eye anomalies