Li Minglei, Han Zongming, Li Jinlei, Wang Qianyun, Lv Zufang
First Department of Pediatrics, Weifang People's Hospital Affiliated to Shandong Second Medical University, Weifang, China.
School of Clinical Medicine, Shandong Second Medical University, Weifang, China.
Front Immunol. 2025 Jan 14;15:1480212. doi: 10.3389/fimmu.2024.1480212. eCollection 2024.
Autoimmune cerebellar ataxia (ACA) is a cerebellar syndrome induced by autoimmune reactions and its onset is induced by malignant tumors, prodromic infection, and gluten allergy. Its clinical symptoms include gait disorder, limb ataxia, dysarthria, and dysphagia. According to , the diagnosis of ACA is based on the following points: 1. subacute or acute onset of the disease, with cerebellar syndrome as the main manifestation; 2. The cranial magnetic resonance imaging (MRI) in the early stage of the disease (within three months) does not show significant atrophy of the cerebellum and brainstem; 3. presence of either of the following: 1) positive anti-cerebellar antibodies in serum and/or cerebrospinal fluid cell-based assay (CBA), 2) at least two of the following are present: ① the patient or first-degree relative has a history of autoimmune disease, ② cerebrospinal fluid leukocytes >5×10/L, or positive for cerebrospinal fluid specific oligoclonal bands, ③ tissue-based assay (TBA) revealing the characteristic fluorescent form of Purkinje cell antibody, and ④ the presence of systemic autoimmune disease-related antibodies; and 4. the absence of other diseases. Currently, fewer instances of ACA have been associated with positive results for carbonic anhydrase-related protein VIII (CARP VIII). Three case reports have been detected by this antibody in adults with ovarian cancer, breast cancer, or melanoma, and there is no report on this antibody in children. Moreover, neurological diseases associated with mycoplasma pneumoniae infection are increasingly being reported. Therefore, the correlation between this infection and autoimmune encephalitis antibodies needs to be further investigated.
自身免疫性小脑共济失调（ACA）是一种由自身免疫反应引起的小脑综合征，其发病由恶性肿瘤、前驱感染和面筋过敏诱发。其临床症状包括步态障碍、肢体共济失调、构音障碍和吞咽困难。根据[具体依据未给出]，ACA的诊断基于以下几点：1. 疾病亚急性或急性起病，以小脑综合征为主要表现；2. 疾病早期（三个月内）头颅磁共振成像（MRI）未显示小脑和脑干明显萎缩；3. 存在以下情况之一：1）血清和/或脑脊液细胞分析（CBA）中抗小脑抗体阳性，2）至少存在以下两项：①患者或一级亲属有自身免疫性疾病史，②脑脊液白细胞>5×10/L，或脑脊液特异性寡克隆带阳性，③组织分析（TBA）显示浦肯野细胞抗体的特征性荧光形式，④存在系统性自身免疫性疾病相关抗体；4. 不存在其他疾病。目前，与碳酸酐酶相关蛋白VIII（CARP VIII）阳性结果相关的ACA病例较少。已有三例该抗体在患有卵巢癌、乳腺癌或黑色素瘤的成人中检测到的病例报告，但在儿童中尚无该抗体的报告。此外，越来越多的支原体肺炎感染相关神经疾病被报道。因此，这种感染与自身免疫性脑炎抗体之间的相关性需要进一步研究。
