POPULATION GENETIC-STUDY OF HEREDITARY MO TOR AND SENSORY NEUROPATHY IN KIROV REGION

All the cases of hereditary motor and sensory neuropathy (HMSN) in an eastern part of Kirov region (Russian north-east) were ascertained (N = 42 including 11 persons with pre/subclinical forms; m: f = 1). HMSN prevalence is 15,95+/-2,47.10(-5) being higher in rural than in urban populations. The distribution of HMSN families (total 16) in 9 distric ts of the region is uneven. HMSN is the most common of all hereditary muscular disorders in the region. Autosomal dominant inheritance was e stablished in 12 families, AD gene frequency is 10,90+/-2,90.10(-5) ge ne penetrance being 90%. Sporadic cases were few (N = 4; 9,76%). No pr oven autosomal recessive or X-linked inheritance was found out.