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[桥本脑病的临床特征与治疗]

[Clinical Features and Treatment of Hashimoto Encephalopathy].

作者信息

Maki Yoshimitsu, Takashima Hiroshi

机构信息

Department of Neurology and Geriatrics, Kagoshima University Graduate School of Medical and Dental Sciences.

出版信息

Brain Nerve. 2016 Sep;68(9):1025-1033. doi: 10.11477/mf.1416200549.

Abstract

Hashimoto encephalopathy (HE) is characterized by heterogeneous neurological symptoms. HE is diagnosed based on three criteria-the presence of antithyroid antibodies, neurological symptoms from the cerebrum and/or cerebellum, and a positive response to immunotherapy. We clinically analyzed 18 patients (3 men, 15 women; age range, 38-81years) diagnosed with HE in our hospital from May 2013 to January 2016. Eleven patients showed sensory abnormalities such as strong pain, deep muscle pain, dysesthesia, paresthesia, or neuralgia. Surprisingly, the majority of the pain was distributed in a manner that was not explainable anatomically. Seventeen patients showed motor disturbances, such as weakness, paresis of extremities, or dexterity movement disorder, and eight patients showed give-way weakness, which is disruption of continuous muscle contraction. Other symptoms indicative of brain-related anomalies such as tremor, dystonia, involuntary movements, cerebellar ataxia, parkinsonism, memory loss, and chronic fatigue were also seen. In most patients, such motor, sensory, or higher brain functions were markedly improved with immunosuppressive therapies such as prednisolone, azathioprine, or immunoadsorption therapy. Although give-way weakness and anatomically unexplainable pain are typically considered as being psychogenic in origin, the presence of these symptoms is indicative of HE. HE exhibits diffuse involvement of the entire brain and thus, these symptoms are explainable. We propose that physicians should not diagnose somatoform disorders without first excluding autoimmune encephalopathy.

摘要

桥本脑病(HE)的特点是神经症状多种多样。HE的诊断基于三个标准:存在抗甲状腺抗体、大脑和/或小脑出现神经症状以及对免疫治疗有阳性反应。我们对2013年5月至2016年1月在我院诊断为HE的18例患者(3例男性,15例女性;年龄范围38 - 81岁)进行了临床分析。11例患者出现感觉异常,如剧痛、深部肌肉疼痛、感觉异常、感觉异常或神经痛。令人惊讶的是,大多数疼痛的分布方式无法从解剖学上解释。17例患者出现运动障碍,如虚弱、肢体轻瘫或灵活性运动障碍,8例患者出现折刀样无力,即持续肌肉收缩的中断。还观察到其他提示脑相关异常的症状,如震颤、肌张力障碍、不自主运动、小脑共济失调、帕金森综合征、记忆力减退和慢性疲劳。在大多数患者中,通过泼尼松龙、硫唑嘌呤或免疫吸附治疗等免疫抑制疗法,这些运动、感觉或高级脑功能有明显改善。尽管折刀样无力和解剖学上无法解释的疼痛通常被认为是心因性的,但这些症状的存在提示为HE。HE表现为全脑的弥漫性受累,因此,这些症状是可以解释的。我们建议医生在未首先排除自身免疫性脑病之前,不应诊断为躯体形式障碍。

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