Wu Qisi, Gong Beibei, Jiang Anan, Qin Xinyue
Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, China.
Department of Radiology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, China.
Front Neurol. 2022 Jul 26;13:951659. doi: 10.3389/fneur.2022.951659. eCollection 2022.
We 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.
A 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.
ACA 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.
我们报告一例与荷马蛋白同源物3（Homer-3）抗体相关的自身免疫性小脑共济失调（ACA）病例。然后，对文献进行综述以总结其临床谱，以提高临床医生对这种罕见疾病的认识。
一名25岁男性亚急性起病，出现小脑共济失调和精神症状，初始脑部MRI显示小脑异常，血清中Homer-3抗体滴度为1:100。静脉注射甲泼尼龙后其神经症状未改善，但血浆置换后显著改善，改良Rankin量表（mRS）评分为1分。然而，5个月后，他在口服泼尼松减量期间复发，重复MRI显示小脑病变加重和明显的小脑萎缩。采用了包括皮质类固醇和血浆置换在内的各种免疫调节方法，但均无改善。然后首次给予利妥昔单抗治疗Homer-3自身免疫，症状部分改善。然而，患者仍严重残疾，mRS评分为4分。
与Homer-3抗体相关的ACA对皮质类固醇治疗的反应可能欠佳。更强化的免疫治疗如利妥昔单抗可能有助于改善小脑综合征。复发病程和小脑萎缩的表现可能提示该疾病预后不良。
