Salmaggi A, Carella F, Ciano C, Binelli S, Giovannini P, Palazzini E, Girotti F
Istituto Nazionale Neurologico C. Besta, Milano, Italy.
Mov Disord. 1995 Mar;10(2):207-10. doi: 10.1002/mds.870100212.
Three patients displaying a clinical picture of progressively evolving multifocal action myoclonus and cerebellar ataxia showed a marked intrathecal immune activation, which was persistent over a 2- to 5-year time span in the two serially investigated patients. A thorough search for metabolic, toxic, infectious, or degenerative causes of myoclonus was unsuccessful. The presence of intrathecal immune activation in at least a subgroup of patients with the clinical features of progressive myoclonic ataxia suggests the possibility of immune-mediated damage within the central nervous system in this condition.
三名表现出逐渐发展的多灶性动作性肌阵挛和小脑共济失调临床表现的患者显示出明显的鞘内免疫激活，在两名接受系列研究的患者中，这种激活在2至5年的时间跨度内持续存在。对肌阵挛的代谢、中毒、感染或退行性病因进行的全面排查未获成功。在至少一部分具有进行性肌阵挛性共济失调临床特征的患者中存在鞘内免疫激活，这提示在这种情况下中枢神经系统内存在免疫介导损伤的可能性。
