2003 Exam I Case 04

Chief Complaint:
This is a 12-year-old male presenting with progressive weakness.

History of Chief Complaint:
The patient recently emigrated from India with his parents about 2 weeks ago. Until this point, he had been an athletic and very active child.  One week prior to his arrival in the United States he had a short viral-like illness including sore throat, nausea, vomiting, and diarrhea lasting about 4 days. One week ago, he began complaining of difficulty in walking and his parents state that he began having difficulty holding various objects in his right hand. The weakness has progressed rapidly over the past week. By the end of the week he could not walk unassisted and was unable to feed himself with his right arm.  He has not received any childhood immunizations.

Physical Exam:
This is an alert and oriented boy carried into the office by his parents. His righ arm hangs limply by his side when seated.  He appears well-hydrated with moist mucosa and good skin turgor. Throat is non-reddened with no tonsillar edema. Lung sounds are clear to auscultation in all fields. No murmurs, rubs, or gallops are heard. Abdomen is soft and non-tender. Obvious muscle atrophy is noted in the right upper extremity, especially the thenar eminence, and in the left lower extremity around the calf muscle.  Parents deny any urinary or fecal incontinence.

Neurological Exam:
Mental Status:
He is awake, alert, and oriented. Knowledge is appropriate for age. Speech is clear and meaningful. Although weak, he can follow 2 step commands without confusion.

Cranial Nerves:
Visual fields are intact to confrontation. Extra-ocular movements are intact bilaterally. Sensation is intact throughout the face. Facial motion is symmetrical. Corneal reflex is present. Uvula is midline with symmetrical elevation of palate. Shoulder shrug is strong and symmetrical. The tongue protrudes in the midline with no fasciculations.

Motor Exam:
Strength in the upper extremity on the right was 2/5 at the shoulder, 1/5 at the elbow and wrist with 2/5 grip strength.  Deep tendon reflexes were trace about the right elbow and wrist.  Strength was normal in the left upper extremity and deep tendon reflexes were 2/4 about the elbow and wrist on the left.  In the left lower extremity, strength was 5/5 at the hip and knee and 1/5 at the ankle.  Deep tendon reflexes were 2/4 about the knee and 1/4 about the ankle.  Strength was normal in the right lower extremity and deep tendon reflexes were 2/4 about the knee and ankle on the left.  Past-pointing was not present in the upper extremity on the left or the lower extremity on the right; it could not be tested in the upper extremity of the right nor in the lower extremity on the left.  Bladder and bowel functions were intact.

Sensory Exam:
Proprioception, vibratory sense, 2–point discrimination, and pin–prick are intact throughout his body.

Follow-Up: Re-examination at 6 months post initial presentation finds him in good health except for a dense weakness and severe atrophy of muscles throughout the right upper extremity and in the calf muscles of the lower extremity on the left.  All reflexes are absent in the effected extremities and a few fasciculations are left, especially in the gastrocs muscle.  He walks assisted with a crutch and is awaiting an appointment for the fitting of a leg brace.

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