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米勒费舍尔和重叠综合征疾病进展的预测。

Prediction of disease progression in Miller Fisher and overlap syndromes.

机构信息

Department of Neurology, Erasmus MC, Rotterdam, The Netherlands.

Department of Neurology, Havenziekenhuis, Rotterdam, The Netherlands.

出版信息

J Peripher Nerv Syst. 2017 Dec;22(4):446-450. doi: 10.1111/jns.12238. Epub 2017 Nov 15.

Abstract

Patients with Miller Fisher syndrome (MFS) may have a relatively mild clinical course or progress to Guillain-Barré syndrome (GBS) with limb weakness (MFS-GBS overlap syndrome). Other variants in this spectrum are GBS with ophthalmoparesis and Bickerstaff's Brainstem encephalitis (BBE). To compare the clinical course of MFS and overlap syndromes and to identify predictors of disease progression. In a prospective study of 170 patients with GBS and variant forms, 37 (22%) had a MFS, MFS-GBS overlap syndrome, ophthalmoplegic GBS or BBE. The clinical, serological, and electrophysiological features were compared. Twenty-three patients presented with MFS, of which 10 (43%) developed limb weakness (MFS-GBS overlap syndrome). All these transitions occurred in the first week after onset of symptoms. There were no differences in the clinical, electrophysiological and serological features at entry between MFS and MFS-GBS. Twelve patients had ophthalmoplegic GBS and the disease severity at nadir and outcome was worse than in the patients with a MFS-GBS overlap syndrome. No early predictors for progression from MFS to MFS-GBS overlap syndrome were found. All transitions occurred in the first week. This finding implicates that all patients with MFS need careful monitoring for at least 1 week.

摘要

米勒费舍尔综合征(MFS)患者的临床病程可能相对较轻,或进展为伴有肢体无力的格林-巴利综合征(GBS)(MFS-GBS 重叠综合征)。该谱系中的其他变体为伴有眼肌麻痹和 Bickerstaff 脑干脑炎(BBE)的 GBS。比较 MFS 和重叠综合征的临床病程,并确定疾病进展的预测因素。在一项对 170 例 GBS 和变异型患者的前瞻性研究中,37 例(22%)为 MFS、MFS-GBS 重叠综合征、眼肌麻痹性 GBS 或 BBE。比较了其临床、血清学和电生理学特征。23 例表现为 MFS,其中 10 例(43%)出现肢体无力(MFS-GBS 重叠综合征)。所有这些转变均发生在症状出现后的第一周内。MFS 和 MFS-GBS 患者在发病时的临床、电生理和血清学特征均无差异。12 例患者为眼肌麻痹性 GBS,其疾病严重程度在最低点和预后均较 MFS-GBS 重叠综合征患者差。未发现从 MFS 进展为 MFS-GBS 重叠综合征的早期预测指标。所有转变均发生在第一周内。这一发现表明,所有 MFS 患者均需要至少 1 周的密切监测。

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