Amyotrophy of the hands and pyramidal features of predominantly the legs segregating within one large family.

Eleven patients of one kindred are described with amyotrophy of the hands or pyramidal features of predominantly the legs or both. Clinical examination indicated neuronal origin of wasting and weakness of the hands, whereas electrophysiological investigations suggested motor neuropathy. It is suggested that both lower and motor neuron signs are segregating as independent autosomal dominant traits.