Demography of several hereditary diseases of the nervous system among the population of the Fergan valley
Dahuraev, A.
Zhurnal Nevropatologii i Psikhiatrii Imeni S.S. Korsakova 77(8): 1163-1166
1977
ISSN/ISBN: 0044-4588 PMID: 143177 Document Number: 122245
The author studied some hereditary diseases of the nervous system among the population of the Fergan Valley. An analysis of 19751 case histories made it possible to estimate the frequency of separate forms of neuroheredodegenerative diseases (Huntington's chorea, Strümpel's paraplegia and different forms of myopathy). A clinico-genetical study of 50 families of the probands permitted to define some clinical and genetical traits of these diseases. It was established that in families of the probands there was a recessive type of inheritance of Strümpel's paraplegia and Erbb's myopathy. Huntington's chorea was characterized by a dominant type of inheritance. It was also established that in famalies with a higher inbreeding, Strümpel's paraplegia was characterized by an early onset of the disease and a rapid development. The author established the frequency of mutations in these diseases which in Hutington's chorea was 2.8X10(-6), for Strümpel's paraplegia--5.8 X 10(-5), Erbb's myopathy 1.12 X 10(5) and Duchenne's myopathy--2.4 X 10(-5).