Avtsyn A P, Zhavoronkov A A, Alekseev V P, Istomin A A
Arkh Patol. 1994 Jul-Aug;56(4):39-44.
Viliuisk encephalomyelitis (VEM) appears to be endemic disease, affecting native population in Yakutia (Yakut, Even, Evenk). The infectious nature of VEM is very likely, but not yet established with certainty. The mortality rate caused by VEM from 1950 to 1990 was 17.4 per 10000 of native population. The maximum morbidity can be seen in Viliui and Central zones of Yakutia. Women were affected one and a half times more than men. VEM is characterized by multiple micronecrotic foci with marked inflammatory reactions and gliosis in the brain tissue. The vascular component in VEM is manifested by vasculitis, plasmarhagies, obliteration of the capillary lumen with development of capillarofibrosis, progressive reduction of the microcirculatory bed, the deformation of walls in major vessels with atrophy of their muscular layer, in some cases--by hyaline thrombi. The data presented give no grounds to consider the vascular component as secondary, relating only to the brain atrophy.
维柳伊斯克脑脊髓炎(VEM)似乎是一种地方病,影响着雅库特地区(雅库特人、埃文人、鄂温克人)的当地居民。VEM极有可能具有传染性,但尚未得到确切证实。1950年至1990年期间,VEM导致的死亡率为每10000名当地居民中有17.4人死亡。发病率最高的地区出现在雅库特的维柳伊地区和中部地区。女性受影响的人数比男性多一倍半。VEM的特征是脑组织中有多个微坏死灶,并伴有明显的炎症反应和胶质细胞增生。VEM的血管病变表现为血管炎、血浆渗出、毛细血管腔闭塞并伴有毛细血管纤维化、微循环床逐渐减少、大血管壁变形且肌肉层萎缩,在某些情况下还会出现透明血栓。所提供的数据无法证明血管病变是继发性的,仅与脑萎缩有关。