米勒费雪综合征和血浆置换。

Chiaki Kambara, Hidenori Matsuo, Takayasu Fukudome, Hirofumi Goto, Noritoshi Shibuya
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引用次数: 31

摘要

米勒费雪综合征(MFS)的治疗存在争议,甚至其自然病史和预后也不完全清楚。我们回顾性地回顾了过去3年的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,他们的症状在发病后长达50天内得到缓解(共济失调改善20-35天,眼麻痹改善25-50天),1例患者被诊断为格林-巴-罗综合征,并出现了严重的肌肉无力。我们还发现MFS患者有偏离的辅助性t -1型(Th1)/辅助性t -2型(Th2)极化,血浆置换可以将MFS患者的Th2显性状态转变为Th1显性状态。尽管血浆置换可以去除体液因子,包括抗gq1b,并可能诱导外周血中产生Th1/Th2细胞因子的细胞平衡的改变,但其治疗原理尚未确定。因此,需要对照临床试验来证明血浆置换是否能使MFS患者更早恢复并减少神经功能缺陷。
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Miller Fisher syndrome and plasmapheresis.

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.

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Presidential Address: PRESIDENTIAL ADDRESS Fluctuations in the peripheral blood leukocyte and platelet counts in leukocytapheresis in healthy volunteers. Mobilization factors of peripheral blood stem cells in healthy donors. Cytokine removal by plasma exchange with continuous hemodiafiltration in critically ill patients. In vitro evaluation of newly developed adsorbent for selective removal of glycosylated low-density lipoprotein.
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