2001 Exam I Case 03

CHIEF COMPLAINT:

This is a 57-year-old, left-handed financial planner who was referred for complaints of progressive weakness and atrophy of his upper extremities.

HISTORY OF CHIEF COMPLAINT:

He had enjoyed reasonably good health until about 7 months ago when he began to notice an abnormal weakness in his arms and hands. Since that time the weakness has progressed throughout his arms and even into his legs. The patient noticed 3 months ago that he is more frequently experiencing cramping in his legs. About one month ago, his wife noted a change in his speech pattern, noting that there was slurring which at first was sporadic, but then progressed to being present with all speech. Two weeks ago he began experiencing a difficulty swallowing.

MEDICAL HISTORY:

Hypertension controlled with a á-blocker, anterior wall myocardial infarction 12 years ago, and surgical repair of external hemorrhoids at age 52.

GENERAL PHYSICAL EXAM:

He is an awake, oriented, well-nourished man who appears older than his stated age. There is significant loss of muscle mass noted in shoulders and arms bilaterally, with the most prominent area of muscle loss being the thenar eminences bilaterally. Heart rate is 76 and regular, blood pressure is 148/96 and respiratory rate is 19. Peripheral pulses are intact bilaterally. Rectal sphincter tone is normal.

NEUROLOGIC EXAM:

MENTAL STATUS: He is awake and oriented to person, place and time, with intact fund of knowledge and memory. His speech is dysarthric with meaningful speech patterns and content.

CRANIAL NERVES: His visual fields are full and extraocular movements intact. Pupils are equal, round and reactive to light and accommodation. Facial expressions are intact and symmetrical. Corneal, jaw-jerk and gag reflexes are sluggish. The uvula elevated on the midline. The tongue protruded on the midline but shows weakness and fasciculations. Pinprick and light touch are intact throughout the face.

MOTOR EXAM: Strength is 4/5 at the shoulder, 3/5 at the elbow, 2/5 at the wrist on the right side and 3/5 at the shoulder, 2/5 at the elbow and 2/5 at the wrist on the left side. Grip strength is 1/5 bilaterally. Strength is 3/5 at the hips and knees bilaterally, and 2/5 at the ankles bilaterally. Deep tendon reflexes are depressed at the elbows and wrists bilaterally (1/4) and elevated at 3/4 at the knees and 4/4 at the ankles bilaterally. Significant atrophy is present bilaterally in the arms and forearms and is most prominent in the thenar eminences bilaterally. Widespread fasciculations are present in all four extremities. The patient can rise from a chair and walk a short distance unassisted. A fine motor positional tremor is present in the upper extremities when they are held in an extended and pronated position. The tremor disappears when his arms are at rest. He notes that the tremor is exacerbated when he is stressed. Finger-to-nose and heel-to-shin testing is normal bilaterally. No pronator drift is observed. Bowel and bladder functions are intact.

SENSORY EXAM: Discriminative touch, vibratory sense, proprioception, and pain and temperature sensation are intact throughout his body.

FOLLOW-UP:

Re-exam at three-months finds further decrease in strength, 3/5 in the shoulders bilaterally, 2/5 in the wrists and 1/5 for grip strength bilaterally. Strength at the hips, knees and ankles bilaterally are 2/5. DTR's remain diminished in the upper extremity (1/4) and are 2/4 at the knees and 1/4 at the ankles bilaterally. The patient complains of frequent choking spells when swallowing, and he lacks any gag reflexes.