Ataxia associated with Hashimoto's disease: progressive non-familial adult onset cerebellar degeneration with autoimmune thyroiditis
Ataxia associated with Hashimoto's disease: progressive non-familial adult onset cerebellar degeneration with autoimmune thyroiditis
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DOI：
10.1136/jnnp.71.1.81
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发表时间：
2001-07-01
影响因子：
11
通讯作者：
Drachman, DA
中科院分区：
文献类型：
--
作者：
Selim, M;Drachman, DA
获得性小脑性共济失调已被描述为伴有甲状腺功能减退症，通常可通过甲状腺激素替代疗法逆转。小脑功能障碍归因于内分泌失调的代谢和生理效应。然而，在少数患者中，尽管进行了甲状腺替代治疗，共济失调仍然持续存在。其他机制可能涉及与甲状腺疾病相关的共济失调。 目的-记录六名抗甲状腺抗体升高（桥本氏/自身免疫性甲状腺炎）患者发生的进行性非家族性成人发病小脑变性（PNACD），以及在没有甲状腺功能减退的情况下的其他自身免疫表现；方法——对 6 名 PNACD 患者进行案例研究，回顾临床病程以及与内分泌和自身免疫状态的关系。结果——所有 6 名患者出现症状时甲状腺功能正常；出现与桥本自身免疫性甲状腺炎一致的抗甲状腺抗体；并且有强烈的器官特异性自身免疫素质的个人或家族史。脑部 MRI 显示四名患者小脑蚓部萎缩，两名患者橄榄脑桥小脑萎缩。排除了小脑变性的其他可能原因。用L-甲状腺素进行从头治疗（两名患者）或继续治疗（三名患者）并没有改变共济失调的进展。结论-这些抗甲状腺抗体升高的患者的小脑变性可能是免疫介导的。抗甲状腺抗体的存在可能发出信号或导致自身免疫过程产生小脑变性。 “桥本相关性共济失调”似乎代表了一种可识别且并不罕见的病症；应考虑对这些患者尝试免疫调节治疗。
Acquired cerebellar ataxia has been described with hypothyroidism, and is typically reversible by thyroid hormone replacement therapy. The cerebellar dysfunction has been attributed to metabolic and physiological effects of the endocrine disorder. In a few patients, however, ataxia has persisted despite thyroid replacement therapy. Other mechanisms may be involved in ataxia associated with thyroid disorders.Objective-To document progressive nonfamilial adult onset cerebellar degeneration (PNACD) occurring in six patients with raised antithyroid antibodies (Hashimoto 's/autoimmune thyroiditis), and other autoimmune manifestations, in the absence of hypothyroidism; and to document the independence of the cerebellar disorder from the endocrine dysfunction.Methods-A case study of six patients with PNACD reviewing the clinical course and relation to endocrine and autoimmune status.Results-All six patients were euthyroid when they developed their symptoms; had raised antithyroid antibodies consistent with Hashimoto's autoimmune thyroiditis; and had strong personal or family histories of organ specific autoimmune diatheses. Brain MRI disclosed atrophy of the cerebellar vermis in four patients and olivopontocerebellar atrophy in two. Other possible causes of cerebellar degeneration were excluded. De novo treatment (two patients) or continued treatment (three patients) with L-thyroxine did not modify the progression of the ataxia.Conclusions-Cerebellar degeneration in these patients with raised antithyroid antibodies may be immune mediated. The presence of antithyroid antibodies may signal or cause the autoimmune process producing cerebellar degeneration. "Hashimoto's associated ataxia" seems to represent a recognisable and not uncommon condition; a trial of immunomodulating therapy should be considered in these patients.