QUESTION 01:
The port wine stain characteristic of Sturge-Weber syndrome is most commonly found:
A. In an upper motor neuron seventh nerve pattern
B. In a lower motor neuron seventh nerve pattern
C. In the first division of the trigeminal nerve, bilaterally
D. In the first division of the trigeminal nerve, unilaterally
E. In the distribution of the greater occipital nerve
QUESTION 02:
2-day-old, former full-term neonate has seizures that are difficult to control. Her mother reports that in utero the baby had significantly more “hiccups” than her other children did. On examination, the infant is encephalopathic and hypotonic. Labor and delivery were uneventful and the infant had Apgar scores of 8 and 9.
What test or intervention would be most useful in identifying the etiology for her seizures?
A. Brain MRI
B. EEG
C. Lumbar puncture
D. EMG
E. Response to therapeutic hypothermia
QUESTION 03:
Which cranial nerve is most likely to be affected by Lyme disease?
A. Optic nerve
B. Oculomotor nerve
C. Trigeminal nerve
D. Facial nerve
E. Hypoglossal nerve
QUESTION 04:
A neonate is being loaded on phenobarbital for seizures. What is the most likely side effect the neonatology team should be prepared for?
A. Hyponatremia
B. Respiratory suppression
C. Anaphylaxis
D. Hypertension
E. Dystonic reaction
QUESTION 05:
What percentage of children with new-onset epilepsy will eventually be able to discontinue medication and remain seizure-free?
A. 10%
B. 30%
C. 50%
D. 70%
E. 90%
ANSWER: MONDAY 10 AM.
QUESTION 01: Answer D
ReplyDeleteSturge-Weber syndrome is a sporadic disorder characterized by seizures and a port wine stain skin lesion. The port wine stain is unilateral and most often involves the first division of the trigeminal nerve, although lower divisions can also be involved. The seventh cranial nerve, the facial nerve, innervates the muscles of facial expression. The forehead receives bilateral innervation and so an upper motor neuron lesion, such as a stroke, will result in unilateral facial weakness that spares the forehead. Lower motor neuron facial weakness, such as that from a Bell’s palsy, involves the forehead. The greater occipital nerves provide sensation over the back of the head, with innervation from the upper cervical cord.
QUESTION 02: Answer C
Glycine encephalopathy classically presents in the neonatal period with difficult to control seizures in an encephalopathic infant. Some infants will have experienced seizures in utero, which the mother may report as frequent “hiccups.” A high CSF to plasma glycine ratio, and therefore lumbar puncture with measurement of serum and CSF glycine, is essential in making the diagnosis.
A glycine peak may be seen on magnetic resonance spectroscopy, but brain imaging is the not the typical way of making the diagnosis. EEG will show frequent seizures, but will not be specific to etiology. EMG is used to evaluate for peripheral nerve, neuromuscular junction, or muscle pathology. Neonates who have experienced hypoxic-ischemic brain injury at birth may suffer seizures and benefit from therapeutic hypothermia; however, this infant had an uncomplicated delivery with good Apgar scores and no evidence of birth asphyxia.
QUESTION 03: Answer D
Neurologic complications of Lyme disease include cranial nerve palsies and meningitis. The facial nerve is most likely to be affected, and Lyme disease is an important consideration in evaluating a child with a Bell’s palsy. Early treatment (within 72 hours of onset) with corticosteroids improves recovery.
Some patients with Bell’s palsy experience hyperacusis due to weakness of the stapedius muscle, an inner ear muscle that is innervated by the seventh nerve and responsible for dampening sound intensity. The seventh nerve also provides taste sensation to the anterior two thirds of the tongue; hence, many patients with Bell’s palsy report abnormal taste on the affected side.
QUESTION 04: Answer B
Multiple loading doses of phenobarbital can sometimes be required to control neonatal seizures. Respiratory suppression is not uncommon and infants may need respiratory support, particularly with escalating doses.
Phenobarbital-induced electrolyte disturbances are not expected. Allergic reactions are rare. Dystonic reactions are associated with dopamine receptor antagonists, not barbiturates.
QUESTION 02: Answer C
About 50% of all children with new-onset epilepsy will ultimately outgrow their epilepsy and no longer require medication. Response to medication is a positive predictor, with about 65% of children who are seizure-free for 2 years on medication remaining seizure-free after weaning medication.