Department of Medical Sciences and Public Health, Institute of Neurology, University of Cagliari and AOU Cagliari, 09042, Cagliari, Italy.
Cerebellum. 2019 Oct;18(5):932-940. doi: 10.1007/s12311-019-01059-9.
Thyroid disorders, including hypothyroidism, hyperthyroidism and Hashimoto encephalopathy, are considered the most common cause of cerebellar dysfunction due to hormonal imbalance. Typically, cerebellar impairment occurs in the course of hypothyroidism and Hashimoto encephalopathy. Information about demographic, clinical and laboratory features of cerebellar disease associated with thyroid disorders is poor. Our review of the literature (1965 to 2018) identified 28 cases associated with hypothyroidism and 37 cases associated with Hashimoto encephalitis. Both patients with hypothyroidism and Hashimoto encephalopathy presented with signs of ataxia that were similarly distributed in the two groups and were mostly predictive of vermis involvement and frequent impairment of cerebellar hemispheres. Additional neurological signs, like dystonia, psychiatric symptoms, ocular disturbances and myoclonus, could be found in the Hashimoto encephalopathy group alone. When present, atrophy of vermis and often of both cerebellar hemispheres was the main imaging abnormality in both hypothyroidism and Hashimoto encephalopathy. Anti-thyroid antibodies could be detected in three quarters of patients with hypothyroidism and in all patients with Hashimoto encephalopathy. In the patients with hypothyroidism, thyroid replacement therapy yielded complete or partial remission of ataxia. In the Hashimoto encephalopathy group, immunosuppressive treatment provided complete remission of ataxia in about 60% of patients, partial remission in the remaining cases. Owing to the treatable nature of the condition and the high prevalence of thyroid disease among general population, cerebellar syndrome associated with thyroid disorders should be considered an important clinical entity. Information from this review will hopefully stimulate and strengthen awareness of thyroid-associated ataxia among clinicians.
甲状腺疾病,包括甲状腺功能减退症、甲状腺功能亢进症和桥本脑病,被认为是最常见的因激素失衡导致小脑功能障碍的原因。通常,小脑损伤发生在甲状腺功能减退症和桥本脑病的过程中。关于与甲状腺疾病相关的小脑疾病的人口统计学、临床和实验室特征的信息很少。我们对文献(1965 年至 2018 年)的回顾确定了 28 例与甲状腺功能减退症相关的病例和 37 例与桥本脑病相关的病例。甲状腺功能减退症和桥本脑病患者均表现出共济失调的迹象,这些迹象在两组中分布相似,主要预测蚓部受累和小脑半球频繁受损。在桥本脑病组中还可以发现其他神经学迹象,如肌张力障碍、精神症状、眼部障碍和肌阵挛。当存在时,小脑蚓部和常伴有小脑半球萎缩是甲状腺功能减退症和桥本脑病的主要影像学异常。甲状腺功能减退症患者中有四分之三可检测到抗甲状腺抗体,而所有桥本脑病患者均可检测到。在甲状腺功能减退症患者中,甲状腺替代治疗使共济失调完全或部分缓解。在桥本脑病组中,约 60%的患者接受免疫抑制治疗后共济失调完全缓解,其余患者部分缓解。由于该疾病具有可治疗性,且甲状腺疾病在普通人群中高发,因此应将与甲状腺疾病相关的小脑综合征视为一种重要的临床实体。希望本次综述提供的信息能激发并增强临床医生对与甲状腺相关的共济失调的认识。