Gol'dfarb L G, Fedorova N I, Chumakov M P, Petrov P A, Vladimirtsev A I
Genetika. 1979;15(8):1502-12.
Viljuisk encephalomyelitis (VE) is a severe neurologic disease characterized by slow progressive dementia', oligobradykinesia, low spastic paraparesis and speech disturbances. It develops in persons of 20-50 years old. VE occurs in a small region of middle Viljui, but for last years the focus has considerably expanded. Etiology of VE is still obscure. 194 families with VE patients were examined. The data obtained contradict the hypothesis of simple recessive inheritance of VE. The value of the heredity coefficient, calculated on the basis of the Falconer - Edwards model, is 22-29% for relatives of the first relation degree. It suggests the existence of individual hereditary determined susceptibility to VE. 14 secondary cases were observed in affected families among adopted relatives (adopted children, husbands and wives of patients) with a rate exceeding random possible frequency. These observations have been evaluated as an evidence of horizontal transmission of the disease from patients with chronic forms to healthy persons. Obligatory condition for the transmission consists in a long-term contact (as a rule, more than one year). According to all known characteristics VE should be refferred to slow infections.
维柳伊斯克脑脊髓炎(VE)是一种严重的神经系统疾病,其特征为缓慢进展性痴呆、少动性运动迟缓、下肢轻度痉挛性轻瘫和言语障碍。该病发生于20至50岁的人群。VE在维柳伊中部的一个小区域出现,但在过去几年中,病灶已大幅扩大。VE的病因仍不明确。对194个有VE患者的家庭进行了检查。所获得的数据与VE简单隐性遗传的假说相矛盾。根据法尔科纳-爱德华兹模型计算得出的遗传系数,对于一级亲属而言为22%至29%。这表明存在个体遗传决定的对VE的易感性。在受影响家庭的收养亲属(收养子女、患者的丈夫和妻子)中观察到14例二代病例,其发生率超过随机可能频率。这些观察结果被评估为该疾病从慢性形式患者向健康人水平传播的证据。传播的必要条件是长期接触(通常超过一年)。根据所有已知特征,VE应归类为慢感染。