1. The paternal grandfather (II.1), born in 1924, was said to be more severely affected than III.2 with a hoarse voice and marked wasting of his hands. He had wasted feet, an abnormal gait with foot drop and a tendency to trip, and recent difficulty in climbing and
2. Table 3 compares our clinical findings and electrophysiological studies with those of other groups.'-3 Our family is comparable with the Welsh family described by Young and Harper';in; They share many clinical and electrophysiological features. Upper limb involvement preceded lower limb changes in most patients. Only one subject in the family of Young and Harper had a normal voice and indirect laryngoscopy showed unilateral or bilateral vocal cord paralysis in the hoarse subjects examined,1980