Case report and literature analysis: Autoimmune cerebellar ataxia associated with homer-3 antibodies

作者全名："Wu, Qisi; Gong, Beibei; Jiang, Anan; Qin, Xinyue"

作者地址："[Wu, Qisi; Jiang, Anan; Qin, Xinyue] Chongqing Med Univ, Dept Neurol, Affiliated Hosp 1, Chongqing, Peoples R China; [Gong, Beibei] Chongqing Med Univ, Dept Radiol, Affiliated Hosp 1, Chongqing, Peoples R China"

通信作者："Qin, XY (通讯作者)，Chongqing Med Univ, Dept Neurol, Affiliated Hosp 1, Chongqing, Peoples R China."

来源：FRONTIERS IN NEUROLOGY

ESI学科分类：CLINICAL MEDICINE

WOS号：WOS:000838289300001

JCR分区：Q2

影响因子：3.4

年份：2022

卷号：13

期号：　

开始页：　

结束页：　

文献类型：Article

关键词：autoimmune cerebellar ataxia; Homer-3 antibody; brain MRI enhanced abnormalities; relapse; rituximab

摘要："ObjectiveWe present a case of autoimmune cerebellar ataxia (ACA) associated with Homer protein homolog 3 (Homer-3) antibodies. Then, a review of the literature was conducted to summarize its clinical spectrum to improve clinicians' understanding of this rare entity. Case presentationA 25-year-old man suffered from the subacute onset of cerebellar ataxia and psychiatric symptoms with abnormalities in the cerebellum on initial brain MRI and Homer-3 antibodies titers of 1:100 in the serum. His neurological symptoms did not improve after intravenous methylprednisolone but significantly improved following plasma exchange with a modified Rankin Scale (mRS) score of 1. However, 5 months later, he experienced relapse during oral prednisone tapering with enhanced cerebellar lesions and obvious cerebellar atrophy on repeated MRI. Various immunomodulatory approaches, including corticosteroids and plasma exchange, were utilized with no improvement. Then rituximab was given for the first time to treat Homer-3 autoimmunity with partial improvement of symptoms. However, the patient remained profoundly disabled with an mRS score of 4. ConclusionACA associated with Homer-3 antibodies may have a suboptimal response to corticosteroid therapy. More intense immunotherapy such as rituximab may contribute to the improvement of cerebellar syndrome. Relapsing courses and presentation of cerebellar atrophy may suggest a poor prognosis in this entity."

基金机构：　

基金资助正文：