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Autoimmune Cerebellar Ataxia: Etiology and Clinical Characteristics of a Case Series from China

文献类型：期刊论文

作者Liu, Mange3,4,5; Ren, Haitao3,4,5; Zhu, Yicheng3,4,5; Fan, Siyuan3,4,5; Bai, Lin3,4,5; Wang, Jing1,2; Cui, Liying3,4,5; Guan, Hongzhi3,4,5
刊名CEREBELLUM
出版日期2022-05-26
页码7
关键词Autoimmune cerebellar ataxia Paraneoplastic cerebellar degeneration Primary autoimmune cerebellar ataxia Prognosis
ISSN号1473-4222
DOI10.1007/s12311-022-01412-5
通讯作者Guan, Hongzhi(guanhz@263.net)
英文摘要Autoimmune cerebellar ataxia (ACA) is an important and potentially treatable cause of sporadic cerebellar syndrome, but studies with large sample size are limited. This study reported a large ACA series in China and described its etiology and clinical characteristics. We reviewed all ACA patients from our hospital (2013-2021) and analyzed their clinical and paraclinical features, treatment, and outcome. ACA subtypes investigated included paraneoplastic cerebellar degeneration (PCD), primary autoimmune cerebellar ataxia (PACA), anti-glutamate decarboxylase (GAD)-associated cerebellar ataxia, opsoclonus-myoclonus syndrome (OMS), Miller Fisher syndrome (MFS), and ACA-associated with autoimmune encephalitis. A total of 127 patients were identified and 40.9% were male. The median onset age was 47.0 years. Gait ataxia was the most prevalent feature followed by limb ataxia, dizziness, and dysarthria/dysphagia. Extracerebellar manifestations included pyramidal signs (28.3%) and peripheral neuropathy/radiculopathy (15.0%). ACA subtypes were PCD (30.7%), PACA (37.8%), ACA associated with autoimmune encephalitis (12.6%), anti-GAD-associated ACA (8.7%), MFS (7.1%), and OMS (3.1%). Neuronal antibodies were positive in 67.7% of patients. Brain magnetic resonance imaging was unremarkable (55.7%) or showed atrophy (18.3%) or abnormal signal intensity (26.1%, most of which was extracerebellar). Although most patients received immunotherapy, the modified Rankin scale at last follow-up was <= 2 in only 47.3% patients. Thirteen patients died and 24 relapsed. Compared with PACA, PCD patients were older and had poorer outcome. This study illustrates the heterogeneity in the clinical features of ACA and suggests the importance of neuronal antibody testing in ACA diagnosis. PCD and PACA are the dominant ACA subtypes, and the former has a less favorable prognosis.
收录类别SCI
资助项目CAMS Innovation Fund for Medical Sciences[CIFMS 2021-1-I2M-003]
WOS研究方向Neurosciences & Neurology
语种英语
WOS记录号WOS:000805461500001
出版者SPRINGER
资助机构CAMS Innovation Fund for Medical Sciences
源URL[http://ir.psych.ac.cn/handle/311026/42733]  
专题心理研究所_中国科学院心理健康重点实验室
通讯作者Guan, Hongzhi
作者单位1.Univ Chinese Acad Sci, 19 A Yuquan Rd, Beijing 100049, Peoples R China
2.Chinese Acad Sci, Inst Psychol, CAS Key Lab Mental Hlth, 16 Lincui Rd, Beijing 100101, Peoples R China
3.Chinese Acad Med Sci, Beijing 100730, Peoples R China
4.Peking Union Med Coll, Beijing 100730, Peoples R China
5.Peking Union Med Coll Hosp, Dept Neurol, Beijing, Peoples R China
推荐引用方式
GB/T 7714		 Liu, Mange,Ren, Haitao,Zhu, Yicheng,et al. Autoimmune Cerebellar Ataxia: Etiology and Clinical Characteristics of a Case Series from China[J]. CEREBELLUM,2022:7.
APA Liu, Mange.,Ren, Haitao.,Zhu, Yicheng.,Fan, Siyuan.,Bai, Lin.,...&Guan, Hongzhi.(2022).Autoimmune Cerebellar Ataxia: Etiology and Clinical Characteristics of a Case Series from China.CEREBELLUM,7.
MLA Liu, Mange,et al."Autoimmune Cerebellar Ataxia: Etiology and Clinical Characteristics of a Case Series from China".CEREBELLUM (2022):7.

入库方式： OAI收割

来源：心理研究所

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