[Chronic spinal amyotrophy with paralysis of the vocal cords: Young-Harper syndrome].

A 59 year old man had a 20 year history of proximal muscle weakness and proximal and distal amyotrophy with areflexia and vocal cords paralysis. The EMG and muscular biopsy were compatible with a chronic spinal atrophy. The sister of this patient was said to be suffering from a similar syndrome. The only similar cases published are the cases inherited as an autosomal dominant trait reported by Young and Harper. They are differentiated by the distal predominance of the amyotrophy. These cases can be compared with the rare cases of hereditary laryngeal palsy which have also an autosomal dominant inheritance.