[Variability of hereditary spinal amyotrophies]

Under conditions of the former isolate in 13 patients from 8 families hereditary spinal amyotrophy of adults was revealed, which is not in compliance to any other forms of this disease. The disease manifests in several generations of families at the age of 30--40 years in the form of anterior horn lesion to the cervical level in combination with the moderate "bulbar" and pyramidal disorders. The development of the disease is slow. The main type of inneritance is dominant. Histological study of a lethal case demonstrated the anterior horn lesion and the secondary character of amyotrophy.