Pediatric Examination and Board Review (229 page)

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FIGURE 133-2.
Bartonellosis: cat-scarch disease with a primary lesion. Erythematous nodule of the check of a 9-year-old girl at the site of the cat scratch. The diagnosis was made on the histologic findings of the excised specimen. (Reproduced, with permission, from Wolff K, Johnson RA. Fitzpatrick’s Color Atlas & Synopsis of Clinical Dermatology, 6th ed. New York: McGrawHill; 2009: Fig. 24-55.)

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Red Book: 2009 Report of the Committee on Infectious Diseases.
28th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2009.

Bale JF. Viral infections of the nervous system. In: Swaiman KF, Ashwal S, Ferriero DM, eds.
Pediatric Neurology: Principles & Practice.
4th ed. Philadelphia, PA: Mosby; 2006: 1595.

Bonthius DJ, Karacay B. Meningitis and encephalitis in children—an update.
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2002;20:1013-1038.

Cochrane DD. Brain abscess.
Pediatr Rev.
1999;20:209-215.

Glaser CA, Lewis PF, Schuster FL. Fungal, rickettsial, and parasitic diseases of the nervous system. In: Swaiman KF, Ashwal S, Ferriero DM, eds.
Pediatric Neurology: Principles & Practice.
4th ed. Philadelphia, PA: Mosby; 2006:1631.

Täuber MG, Schaad UB. Bacterial infections of the nervous system. In: Swaiman KF, Ashwal S, Ferriero DM, eds.
Pediatric Neurology: Principles & Practice.
4th ed. Philadelphia, PA: Mosby; 2006:1571.

Volpe JJ. Viral, protozoan, and related intracranial infections. In: Volpe JJ, ed.
Neurology of the Newborn.
4th ed. Philadelphia, PA: WB Saunders; 2001:717.

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CASE 134: A 17-YEAR-OLD ADOLESCENT GIRL WITH MUSCLE WEAKNESS

A 17-year-old athletic, right-handed adolescent girl comes into your clinic with the chief complaint of lower extremity muscle weakness. She states that she first noted the weakness 1 month ago while playing soccer. At that time, she noted she was unable to keep up with her teammates. She states that she feels fine for the first 10-15 minutes but after that seems to fatigue. In addition, during routine practices, she falls approximately 10-12 times per session. She states that although she can walk well, she has difficulty climbing stairs. She has to rest after 2 flights and cannot climb without holding on to the railing. In addition to soccer, the patient runs competitively, stating she used to be able to run 400 meters in 65 seconds. She can no longer compete. These symptoms were not present before a month ago. She denies any history of muscle pain, myoglobinuria, or previous infectious illness. She denies any history of rash or joint swelling.

On review of her past medical history, she was born at full term without complications. She has a history of bifrontal headaches associated with photophobia and phonophobia. A head CT was reportedly normal. On review of symptoms she has experienced occasional diplopia. She states that her vision is worse in the evening and better in the morning. There is no family history of rheumatologic or neuromuscular disorders. She has a maternal aunt with “thyroid problems.” On physical examination, she is alert and in no apparent distress. Her general examination is unremarkable. Her cranial nerves are intact. However, on prolonged upward gaze, she developed moderate bilateral ptosis after 20 seconds. When asked to abduct her left arm repetitively, she developed left deltoid weakness after 45 attempts. Her strength returned to normal following a 4-minute rest period. The remainder of her neurologic examination was normal.

SELECT THE ONE BEST ANSWER

1.
Which of the following tests is most likely to provide you with a diagnosis?

(A) MRI of the brain with and without contrast

(B) lumbar puncture

(C) EEG

(D) MRI of the spine

(E) antibody testing

2.
You send blood for a creatine kinase (CK) level and antibodies to the acetylcholine receptor. The CK comes back at 89 (45-230) U/L. The acetylcholine binding, blocking, and modulating antibody titers are 69 (0-0.4) mmol/L, 55% (0-15), and 46% (0-20), respectively. The most likely diagnosis is which of the following?

(A) botulism

(B) congenital myasthenia

(C) Lambert-Eaton myasthenic syndrome

(D) juvenile myasthenia gravis

(E) neonatal myasthenia gravis

3.
Which of the following statements regarding juvenile myasthenia gravis is false?

(A) it is more common in boys than in girls

(B) ptosis can be unilateral

(C) the disease is frequently associated with other autoimmune disorders

(D) anti-AchR antibodies can cross the placenta

(E) ptosis is the most common clinical finding

4.
An important study to obtain in the management of this patient is which of the following?

(A) muscle biopsy

(B) pulmonary function tests

(C) chest CT

(D) oropharyngeal motility testing

(E) thyroid function tests

5.
Treatment of this patient’s condition includes which of the following?

(A) pyridostigmine

(B) corticosteroids

(C) azathioprine

(D) plasmapheresis

(E) all of the above

6.
A patient with myasthenia presents to the ED with the chief complaint of dysphagia. The patient is given a dose of pyridostigmine. Shortly after, the patient complains of nausea and abdominal cramps. The patient has a hard time handling her secretions. The patient soon develops respiratory distress. The most likely cause of this patient’s exacerbation is which of the following?

(A) myasthenic crisis

(B) cholinergic crisis

(C) pneumonia

(D) Guillain-Barré syndrome

(E) none of the above

7.
In counseling this patient, you warn her that certain common medications may make her condition worse. From the following list of medications, which will potentially exacerbate her condition?

(A) erythromycin

(B) penicillin

(C) lidocaine

(D) phenytoin

(E) all of the above

8.
A 4-year-old boy comes to your clinic with the chief complaint of muscle weakness. During the examination, you notice a waddling gait and hypertrophic calves. He has a high serum CK enzyme level. A muscle biopsy demostrates a lack of dystrophin staining. The most likely diagnosis would be which of the following?