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非肿瘤性病因自身免疫性脑炎青少年及成年患者中运动障碍的比例及谱系

Proportion and spectrum of movement disorders in adolescent and adult patients of autoimmune encephalitis of non-neoplastic aetiology.

作者信息

Dash Deepa, Ihtisham Kavish, Tripathi Madhavi, Tripathi Manjari

机构信息

Department of Neurology, All India Institute of Medical Sciences, New Delhi 110029, India.

Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi 110029, India.

出版信息

J Clin Neurosci. 2019 Jan;59:185-189. doi: 10.1016/j.jocn.2018.10.076. Epub 2018 Nov 16.

DOI:10.1016/j.jocn.2018.10.076
PMID:30455136
Abstract

We aimed to study the proportion of patients with movement disorders in seropositive autoimmune encephalitis of non-neoplastic aetiology and also to describe the spectrum of movement disorders in them. We prospectively screened 362 patients of age >12 years with encephalitis of unknown aetiology for a panel of antibodies for autoimmune encephalitis. Demographic and clinical characteristics with focus on the movement disorders were recorded. We also evaluated the differences in the spectrum of movement disorder based on various age groups and antibody positivity. Patients were treated with immune modulating drugs and were followed up for 6 months. Out of the 41 patients, 21 (51.2%) patients presented with movement disorder as a part of their clinical presentation. The commonest movement disorder encountered in our cohort was orofaciolingual dyskinesia (OFLD) 57.1% followed by tremor (38.1%), choreoathetosis (33.3%), paroxysmal dyskinesia (23.8%) stereotypies (14.3%), bradykinesia (13.1%), followed by dystonia (13.1%), catatonia (4.7%), neuromyotonia (4.7%) ballism (4.7%), ataxia (4.7%) and stiff person phenotype (4.7%). The hyperkinetic movement disorders were more commonly seen compared to hypokinetic disorders. All patients received immunomodulatory therapy. On follow, 17 (80.1%) patients had good response with total remission of the movement disorder. Four patients did not have total remission but significant improvement in the symptoms after 6 months of follow up. Our study shows that >50% of patients with antibody positive autoimmune encephalitis have movement disorder as a part of their clinical feature. Timely institution of immunotherapy leads to good outcome in majority of patients.

摘要

我们旨在研究非肿瘤病因的血清学阳性自身免疫性脑炎患者中运动障碍患者的比例,并描述其中运动障碍的范围。我们前瞻性地筛查了362例年龄大于12岁、病因不明的脑炎患者,检测其自身免疫性脑炎抗体谱。记录了以运动障碍为重点的人口统计学和临床特征。我们还评估了基于不同年龄组和抗体阳性情况的运动障碍范围差异。患者接受免疫调节药物治疗,并随访6个月。在41例患者中,21例(51.2%)患者出现运动障碍作为其临床表现的一部分。在我们的队列中最常见的运动障碍是口面部运动障碍(OFLD),占57.1%,其次是震颤(38.1%)、舞蹈手足徐动症(33.3%)、阵发性运动障碍(23.8%)、刻板动作(14.3%)、运动迟缓(13.1%),其次是肌张力障碍(13.1%)、紧张症(4.7%)、神经性肌强直(4.7%)、投掷症(4.7%)、共济失调(4.7%)和僵人综合征(4.7%)。与运动减少性障碍相比,运动增多性运动障碍更常见。所有患者均接受免疫调节治疗。随访时,17例(80.1%)患者反应良好,运动障碍完全缓解。4例患者虽未完全缓解,但随访6个月后症状有明显改善。我们的研究表明,抗体阳性的自身免疫性脑炎患者中有超过50%有运动障碍作为其临床特征的一部分。及时进行免疫治疗可使大多数患者获得良好预后。

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