Hereditary diseases of the nervous system in the Hissar district of Tajikistan

R.A. Rakhmonov, T.V. Madaminova

Chair of Neurology and Bases of Medical Genetics Avicenna TSMU

This paper presents the results of a study of hereditary diseases of nervous system (HDNS) in the Hissar district of Tajikistan.

Diseases were revealed totally in 74 patients (men – 51, women – 23) from 42 families. Nine clinical entities of NBNS areestablished. In result of related marriage were born 33,8% of patients. Predominant form was limb girdle muscular dystrophy (LGMD) (n=22). Least of all was patients with Becker progressive muscular dystrophy (PMD) and atypical form of PMD (1 patient).

The prevalence of LGMD in Hissar district exceeds the data obtained in Khatlon region of Tajikistan (8,4 and 2,7 per 100 000 population, respectively), and Duchenne PMD – is lower (2,3 and 5,7). Mostly nosology had autosomal recessive inheritance (n=26), less – X-linked type (n=2). Autosomal dominant pattern of inheritance is often observed in patients with neural amyotrophy of Charcot-Marie-Toots and autosomal recessive – in LGMD (10 and 11, respectively). NBNS prevalence in districtwhole was 28.1 per 100 000 population.

Keywords: hereditary disease of the nervous system, related marriages, progressive muscular dystrophy, neural amyotrophy of Charcot-Marie-Toots.

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