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与米勒-费舍尔综合征相关的球麻痹及其在中国患者中的重叠表现。

Bulbar paralysis associated with Miller-Fisher syndrome and its overlaps in Chinese patients.

机构信息

Department of Neurology and Research Center of Neurology, Second Affiliated Hospital, and the Collaborative Innovation Center for Brain Science, Zhejiang University School of Medicine, 88 Jiefang Road, Hangzhou, 310009, China.

Department of Neurology and Institute of Neurology, Affiliated Hospital of School of Medicine of Ningbo University, Ningbo, 315020, China.

出版信息

Neurol Sci. 2018 Feb;39(2):305-311. doi: 10.1007/s10072-017-3184-2. Epub 2017 Nov 9.

Abstract

The study aimed to determine the incidence and the onset time of bulbar paralysis (BP) associated with Miller-Fisher syndrome (MFS) and its overlaps, to better understand the clinical characteristics among patients with MFS and its overlaps. Medical records from 48 patients with MFS and its overlaps were divided into two groups based on the presence (MFS-BP+) or absence (MFS-BP-) of BP. Their clinical features, laboratory and electrophysiological findings, neuroimaging data, and treatment plan were analyzed and compared between two groups. The incidence of BP associated with MFS and its overlaps was 48%. Eighty-two percent of the patients developed BP within 1 week after the onset of MFS and its overlaps. The cerebrospinal fluid (CSF) protein level in patients was higher in MFS-BP+ than in MFS-BP- group (67.69 ± 26.59 vs. 50.15 ± 20.44 mg/dl; P < 0.05). Frequencies of severe limb weakness, hypoglossal paralysis, disturbance of consciousness, and tracheal intubation required were also significantly higher in MFS-BP+ than in MFS-BP- group. Positive results of anti-GQ1b and anti-GT1b antibodies were all found in MFS-BP+ group. The prevalence of BP in MFS and its overlap was higher, the majority of BP occurred within 7 days after the onset of the disease, and early diagnosis of BP concurrence is helpful to decide the treatment plan.

摘要

本研究旨在确定与 Miller-Fisher 综合征(MFS)及其重叠症相关的球麻痹(BP)的发生率和发病时间,以更好地了解 MFS 及其重叠症患者的临床特征。根据是否存在 BP(MFS-BP+)将 48 例 MFS 及其重叠症患者的病历分为两组。分析和比较两组患者的临床特征、实验室和电生理检查结果、神经影像学数据和治疗方案。MFS 及其重叠症患者并发 BP 的发生率为 48%。82%的患者在 MFS 及其重叠症发病后 1 周内出现 BP。MFS-BP+患者的脑脊液(CSF)蛋白水平高于 MFS-BP-组(67.69±26.59 比 50.15±20.44 mg/dl;P<0.05)。MFS-BP+组严重肢体无力、舌下神经麻痹、意识障碍和需要气管插管的频率也明显高于 MFS-BP-组。MFS-BP+组均检测到抗-GQ1b 和抗-GT1b 抗体阳性结果。MFS 和其重叠症中并发 BP 的患病率较高,大多数 BP 发生在疾病发作后 7 天内,早期诊断 BP 并存有助于决定治疗方案。

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