Proximal monomelic amyotrophy of the upper limb.

A 30-year-old patient of Central European origin, suffering from monomelic amyotrophy, is presented. The disease was characterized by proximal weakness of one upper limb, mainly of the shoulder girdle, accompanied by atrophy. The electrodiagnostic examination revealed signs of partial denervation in the presence of normal motor and sensory conduction. The disease, which is probably of the anterior horn cells, had a benign course and good prognosis, as evident from repeated examinations during a follow-up of eight years.