HEREDITARY SPASTIC PARAPLEGIAS - A COMPAR ATIVE-STUDY IN RUSSIAN POPULATIONS

15 families (27 patients) with hereditary spastic paraplegia (HSP) wer e found In the course of monogenic disorders investigation in 6 Russia n populations. High HSP prevalence (7,21+1,61). 10(-5) was found In Kl rov region [the frequency of the gene of autosomal-dominant form was ( 3,61+/-1,14). 10(-5), autosomal-recessive-(64,5+/-9,74). 10(-4)]. The pronounced interfamillal polymorphism of HSP was observed. Two familie s with rare autosomal-recessive variation of ''clear'' HSP as well as two families with HSP associated with peroneal amyotrophias were revea led. Accumulation of cases with unusual combination of autosomal-domin ant HSP together with mental deficiency was remarkable In Klrov region .