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米勒-费雪综合征与血浆置换术

Miller Fisher syndrome and plasmapheresis.

作者信息

Kambara Chiaki, Matsuo Hidenori, Fukudome Takayasu, Goto Hirofumi, Shibuya Noritoshi

机构信息

Department of Neurology, Kawatana National Hospital, Nagasaki, Japan.

出版信息

Ther Apher. 2002 Dec;6(6):450-3. doi: 10.1046/j.1526-0968.2002.00466.x.

Abstract

Treatment for Miller Fisher syndrome (MFS) is controversial, and even the natural history and prognosis are not fully understood. We retrospectively reviewed our cases of MFS for the last 3 years. The analysis of 4 MFS cases revealed that we had performed plasmapheresis or additional immunotherapy to each of 4 patients, and their symptoms resolved for up to 50 days after the onset (ataxia improved 20-35 days and ophthalmoplegia for 25-50 days) except for 1 patient, and that Guillain-Barré syndrome had been diagnosed in 1 patient who had developed profound muscle weakness. We also discovered that MFS patients had a deviated T-helper Type-1 (Th1)/T-helper Type-2 (Th2) polarization and that plasmapheresis can shift Th2-dominant status to Th1-dominant status in patients with MFS. Although plasmapheresis may remove humoral factors, including anti-GQ1b, and may induce a shift of the Th1/Th2 cytokine-producing cell balance in peripheral blood, the therapeutic rationale has not yet been established. Therefore, controlled clinical trials are required to show whether plasmapheresis leads to earlier recovery with fewer neurologic deficits in patients with MFS.

摘要

米勒-费希尔综合征(MFS)的治疗存在争议,甚至其自然病程和预后也尚未完全明确。我们回顾性分析了过去3年中我们收治的MFS病例。对4例MFS病例的分析显示,我们对4例患者均进行了血浆置换或额外的免疫治疗,除1例患者外,其余患者在发病后长达50天内症状均得到缓解(共济失调在20 - 35天改善,眼肌麻痹在25 - 50天改善),且1例出现严重肌肉无力的患者被诊断为吉兰-巴雷综合征。我们还发现,MFS患者存在辅助性T细胞1型(Th1)/辅助性T细胞2型(Th2)极化异常,血浆置换可使MFS患者的Th2优势状态转变为Th1优势状态。虽然血浆置换可能清除包括抗GQ1b在内的体液因子,并可能在外周血中诱导Th1/Th2细胞因子产生细胞平衡的转变,但其治疗原理尚未确立。因此,需要进行对照临床试验,以证明血浆置换是否能使MFS患者更早康复且神经功能缺损更少。

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