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A 37-year-old ski instructor came to the Emergency Room complaining of weakness of the legs of one days' duration. He had been well until two and a half weeks before admission when he developed a flu-like syndrome consisting of malaise, vomiting and diarrhea. These symptoms subsided in three or four days. He was then well until the day before admission. He first noted mild tingling dysesthesias in his feet and in the fingers. Later the same day, a sensation of rubberiness of his legs appeared, so that he stayed in bed that afternoon and night. The following morning he was barely able to walk to the bathroom. A friend was called who brought the patient to the Emergency Room.

On exam, the patient had no fever. The neck was supple, and there was no skin eruption, or enlargement of the liver or spleen. The cranial nerves and mental status were normal. There was moderate weakness of the legs and distal equal to proximal and mild weakness of the intrinsic muscles of the hands. No sensory defect could be demonstrated, although the patient still complained of tingling of the hands and feet. Deep tendon  reflexes were absent throughout. The plantar responses were flexor.

Although the examining physician was uncertain what was going on, a decision was made to admit the patient for observation. A complete blood count, urinalysis, chest x-ray, electrocardiogram, electrolytes, blood sugar, and BUN were all normal. A lumbar puncture, done the day of admission, showed a normal opening pressure, clear fluid, a protein of 48 mg % and no cells. A Tensilon test, done the following morning, was negative. On the second hospital day, the legs were severely weak with only flickers of movements of both thighs and the toes. Weakness of the upper extremities had also progressed.

On the third hospital day, the patient was almost quadriplegic and bilateral facial paralysis (facial diplegia) had appeared. The patient was unable to handle his secretions well and complained of breathlessness. His voice sounded weak. Vital capacity was 1100 cc. An elective tracheostomy was performed later that day. A repeat lumbar puncture now showed a protein of 120 mg % and no cells. On the night of the third hospital day, respirations appeared to be labored even with tracheostomy, and ventilatory assistance with a respirator was necessary. The patient was now completely quadriplegic and could barely lift his head from the pillow. Extraocular movements and pupillary light reflexes were preserved. Ventilatory assistance was required for the next 13 days. By the tenth hospital day, movement in the shoulders began to return. Over the next three weeks, steady improvement occurred, so that by five weeks in the hospital, the patient was going to physical therapy every day, and was  walking in parallel bars with assistance. After two more months of Rehabilitation Service, the patient was discharged with only minimal weakness of both arms and legs. An electromyogram, at four weeks after admission, showed moderate slowing of conduction velocities in the ulnar and peroneal nerves and markedly increased distal latencies. There was only minimal evidence of denervation in the abductor digiti quinti.

A repeat lumbar puncture, after four weeks in the hospital, showed a protein of 35 mg %. When the patient was seen in follow-up, 8 months after initial hospitalization, there was no residual weakness of limbs demonstrable, although the ankle jerks could not be elicited. All of the other tendon reflexes were normal and there was no sensory deficit.

 QUESTIONS: CASE SUMMARY 2

1. What features of the physical exam tend to distinguish a myopathy from a neuropathy from a disorder of neuromuscular junction?

2. What is albumin-cytologic dissociation and what does this finding in the spinal fluid suggest about which type of illness is responsible for the muscle weakness?

3. What is a Tensilon test and why was it done?

4. What category of disease do the electrical studies suggest?

5. What is the significance of the previous viral-like illness?

 6. What is the specific diagnosis?