This is a 48 year old right-handed male who presented with a six month history of fatigue and weight loss. For the past six weeks, he had noted progressive difficulty in walking, slurring of his speech, and weakness in both upper extremities. He reported difficulty in combing his hair, writing, and climbing the stairs. During the week prior to presentation, he had fallen repeatedly and was using a wheelchair. The weakness was worse on the left side. He had also noticed a "strange abnormal sensation" over his entire face, and numbness on the dorsal aspect of his left hand.
Patient #8.
He denied pain, problems with memory or affect, visual changes, diplopia, dysphagia, breathing difficulties, or bowel and bladder dysfunction.
His past history was unremarkable. Family history includes a cousin with Addison's disease. He was previously married, but never had children. His occupation was "in the oil business". He denied drug abuse, only drinks socially, and smoked two packs of cigarettes per week for 26 years.
Physical examination was within normal limits with no evidence of cardiopulmonary abnormalities, organomegaly, or arthropathy.
Mental Status: The patient was awake, alert, and oriented with a mini-mental exam of 27/30. He could not recall 2/3 objects and misspelled while writing a sentence. His speech was mildly slurred; comprehension, repetition, and fluency were normal.
Cranial Nerves:
Motor: There was mild generalized wasting, especially in the hands and feet. Tone was mildly spastic in all extremities, left more than the right.
Strength | Right | Left |
|---|---|---|
Upper Extremities | ||
| Deltoid | 4 | 4 |
| Biceps | 4+ | 4 |
| Triceps | 5 | 5 |
| Wrist Extensors | 5 | 5 |
| Wrist Flexors | 4 | 4 |
| Finger Extensors | 4- | 3 |
| Finger Flexors | 4 | 4 |
Lower Extremities | ||
| Iliopsoas | 4- | 4- |
| Knee Extensors | 4 | 4 |
| Knee Flexors | 4+ | 4+ |
| Ankle Extensors | 4+ | 4+ |
| Ankle Flexors | 5 | 5 |
Reflexes: Jaw jerk was positive; Hoffmans and crossed adductors positive bilaterally. Glabella, snout and palmo-mental reflexes were negative. The biceps, triceps, brachioradialis, knee, and ankle reflexes were 2+ on the right and 3+ on the left.
Sensation: Abnormal graphesthesia, stereognosis, and extinction in the left hand. Light touch over the entire face was abnormal. Pin and touch perception was decreased on the left side of the body. Vibration was decreased in the toes; proprioception was normal.
Cerebellar: Finger-to-nose and heel-to-shin were done slowly, but not with prominent ataxia. Rapid alternating movements were slow in the left hand.
Gait: The patient was barely able to take a few steps, and only with support. His legs moved stiffly, exhibiting circumduction bilaterally, left more than right. Heel walking was more difficult than toe walking. Tandem-walking was abnormal, Romberg was negative, and he was unable to walk on his toes or his heels.
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