Atypical spinal amyotrophy in adults
Dubinskaia, E.E.; Viatkina, S.Ia.
Zhurnal Nevropatologii i Psikhiatrii Imeni S.S. Korsakova 91(3): 18-21
1991
ISSN/ISBN: 0044-4588 PMID: 1646529 Document Number: 375363
The authors describe the clinical pleomorphism of atypical spinal amyotrophy of adults (ASAA) characterized by lesions of the anterior horn structures of the cervical part of the spinal cord as well as by the spreading of the process along the entire length of the spinal cord and to the bulbar part of the spine. This is supported by the electrophysiological and morphological data. The disease begins at the 3rd and 4th decades of life and progresses to the lethal outcome due to cardiopulmonary insufficiency. Inheritance occurs by the autosomal dominant type with the high penetrance and by the autosomal recessive type stemming from the long-term inbreeding. ASAA describe is identical to the late spinal amyotrophy depicted by S. N. Davidenkov according to the data obtained by Browning, Bernhardt and Strumpel. The appearance of an isolated form of ASAA in the Saratov region may be related to the migration of the population 200 years ago from Western Europe to the central land along the Volga.