Green Alison J E, Sivtseva Tatiana M, Danilova Al'bina P, Osakovsky Vladimir L, Vladimirtsev Vsevolod A, Zeidler Martin, Knight Richard S, Platonov Feodor A, Shatunov Alexey, Alekseev Vasiliy P, Krivoshapkin Vadim G, Masters Colin L, Gajdusek D Carleton, Goldfarb Lev G
The National CJD Surveillance Unit, Western General Hospital, Edinburgh EH4 2XU, UK.
J Neurol Sci. 2003 Aug 15;212(1-2):69-73. doi: 10.1016/s0022-510x(03)00107-2.
Viliuisk encephalomyelitis (VE) is a neurodegenerative disorder expressed as subacute meningo-encephalitis progressing to a more prolonged pan-encephalitic syndrome with a fatal outcome within 1 to 10 years. Some patients survive to a steady state of global dementia and severe spasticity that may last for over 20 years. Multiple micronecrotic foci surrounded by inflammatory infiltrates are observed throughout the cerebral cortex and other gray matter areas. Infectious etiology of VE is strongly suspected, but the causative agent has not been identified. We conducted a search for assays that might be helpful for VE diagnosis and established for the first time that the majority of patients with definite VE show evidence for intrathecal IgG synthesis correlating with the clinical manifestations of the disease. This indicates that the detection of oligoclonal IgG banding in the cerebrospinal fluid is a valuable diagnostic assay for VE. Implications of these findings for a possible etiology of VE are discussed.
维柳伊斯克脑脊髓炎（VE）是一种神经退行性疾病，表现为亚急性脑膜脑炎，进而发展为更为迁延的全脑炎综合征，1至10年内会导致死亡。一些患者存活下来，进入全面痴呆和严重痉挛的稳定状态，这种状态可能持续20多年。在整个大脑皮层和其他灰质区域观察到多个被炎性浸润包围的微坏死灶。强烈怀疑VE的病因具有传染性，但致病因子尚未确定。我们进行了一项可能有助于VE诊断的检测方法的研究，并首次证实，大多数确诊为VE的患者显示出鞘内IgG合成的证据，且与该疾病的临床表现相关。这表明脑脊液中寡克隆IgG条带的检测是VE的一种有价值的诊断方法。本文讨论了这些发现对VE可能病因的意义。
