Viliuisk encephalomyelitis--review of the spectrum of pathological changes.

Abstract:

:Viliuisk encephalomyelitis (VE) is an unique neurological disease occurring in the Iakut (Sakha) people of Siberia. Evolution of the disease follows one of three broad clinical forms: subacute, slowly progressive or chronic. Death occurs within 3 to 6 months in subacute cases and within 6 years in the slowly progressive cases. Chronic cases lack a subacute phase but show a slowly progressive dementia associated with bradykinesia, dysarthria and spastic paraparesis that stabilizes late in the disease process. In subacute and slowly progressive cases, focal necrotizing encephalomyelitis is seen at necropsy. Chronic cases show multifocal areas of lysis with a gliotic margin, predominantly within grey matter, lacking associated chronic inflammatory changes seen in the other forms of the disease. Epidemiological studies are consistent with a disease of low-grade communicability, but laboratory studies have so far failed to reveal an infectious organism. The spectrum of neuropathological changes are reviewed in this examination of 11 cases. Although the aetiology of VE remains obscure, further studies are warranted since it may represent a novel disease process.

authors

McLean CA,Masters CL,Vladimirtsev VA,Prokhorova IA,Goldfarb LG,Asher DM,Vladimirtsev AI,Alekseev VP,Gajdusek DC

subject

Has Abstract

pub_date

1997-06-01 00:00:00

pages

212-7

issue

3

eissn

0305-1846

issn

1365-2990

journal_volume

23

pub_type

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