Wei Chunxiao, Shen Yanxin, Zhai Weijie, Shang Tianling, Wang Zicheng, Wang Yongchun, Li Mingxi, Zhao Yang, Sun Li
Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Jilin University, Changchun, China.
Front Neurol. 2022 Aug 23;13:970141. doi: 10.3389/fneur.2022.970141. eCollection 2022.
Hashimoto's encephalopathy (HE), also known as steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT), has a variety of clinical manifestations, with various neuropsychiatric characteristics, including tremors, transient aphasia, seizures, altered consciousness, myoclonus, cognitive impairment, and psychiatric manifestations. The hallmark presenting feature is a non-specific encephalopathy characterized by alteration of mental status and consciousness ranging from confusion to coma and impaired cognitive function, while those with cerebellar ataxia as the main manifestation is rare. We reported a case of Hashimoto's encephalopathy with cerebellar ataxia as the main manifestation, elevated anti-thyroid antibodies (anti-TPO/TG), and normal thyroid function. The symptoms of cerebellar ataxia improved after steroid treatment. Meanwhile, we reviewed the clinical features of 20 representative cases of HE with cerebellar ataxia as the core symptoms. In conclusion, based on our case findings and literature review, the diagnosis of HE should be suspected in cases of encephalopathy without an obvious cause, to quickly start an effective treatment.
桥本脑病（HE），也称为与自身免疫性甲状腺炎相关的类固醇反应性脑病（SREAT），有多种临床表现，具有各种神经精神特征，包括震颤、短暂性失语、癫痫发作、意识改变、肌阵挛、认知障碍和精神症状。其标志性的主要特征是一种非特异性脑病，表现为精神状态和意识改变，从意识模糊到昏迷，以及认知功能受损，而以小脑性共济失调为主要表现的情况较为罕见。我们报告了一例以小脑性共济失调为主要表现、抗甲状腺抗体（抗TPO/TG）升高且甲状腺功能正常的桥本脑病病例。类固醇治疗后小脑性共济失调症状改善。同时，我们回顾了20例以小脑性共济失调为核心症状的代表性桥本脑病病例的临床特征。总之，根据我们的病例发现和文献综述，对于无明显病因的脑病病例应怀疑桥本脑病的诊断，以便迅速开始有效的治疗。
