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甲状腺功能减退性肌病

Authors
Marc L Miller, MD
Devon I Rubin, MD
Section Editors
Michael J Aminoff, MD, DSc
Jeremy M Shefner, MD, PhD
Douglas S Ross, MD
Deputy Editor
Janet L Wilterdink, MD
Translators
潘慧, 主任医师，教授

引言

在先天性和成人发作型甲状腺功能减退症中，各种形式的肌肉受累很常见。本专题将讨论甲状腺功能减退性肌病的临床表现、发病机制和治疗。甲状腺功能减退症的其他神经系统表现将单独讨论。 (参见“甲状腺功能减退症的神经系统表现”和“甲状腺功能减退症的临床表现”)

先天性甲状腺功能减退症

Kocher-Debre-Semelaigne综合征描述了具有典型克汀病特征的婴儿伴有弥漫性肌肥大和以近端为主的肌无力。这些婴儿存在运动和认知发育迟缓、便秘、黏液性水肿、舌胖和毛发皮肤粗糙等典型克汀病表现。虽然他们外表看起来肌肉非常发达(几乎是看起来强健)，但实际上却无力，常常难以坐下和控制头部运动[1]。 (参见“碘缺乏病”，关于‘克汀病’一节)

这些婴儿的血清肌酸激酶(creatine kinase, CK)和其他肌酶常常升高。受累肌肉肌电图可能正常，或显示散在的运动单位动作电位波幅减低。肌肉活检显示肌肉纤维大小不等，主要为Ⅰ型纤维萎缩。还存在与甲状腺功能减退持续时间有关的间质结缔组织增多以及异常糖原累积。肌纤维坏死和炎症并不是该病的突出特征[2]。

使用甲状腺激素治疗可使肌力迅速改善和血清CK浓度恢复正常，对于长期先天性甲状腺功能减退症的婴儿也是如此[3]。

成人甲状腺功能减退症

甲状腺功能减退症成人常出现肌肉受累，在一项病例系列研究中，79%的甲状腺功能减退症患者出现肌肉主诉(肌无力、痉挛、肌痛)[4]。更为严重或长期未经治疗的甲状腺功能减退症患者更可能发生有临床意义的肌肉疾病。

           

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Literature review current through: 2017-06 . | This topic last updated: 2015-12-16.
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