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作为混合性结缔组织病的初始表现,可能因系统性血管炎导致的脑病和严重神经病变。

Encephalopathy and severe neuropathy due to probable systemic vasculitis as an initial manifestation of mixed connective tissue disease.

作者信息

Matsui Hideaki, Udaka Fukashi, Oda Masaya, Kubori Tamotsu, Nishinaka Kazuto, Kameyama Masakuni

机构信息

Department of Neurology, Sumitomo Hospital, Osaka, Japan.

出版信息

Neurol India. 2006 Mar;54(1):83-5. doi: 10.4103/0028-3886.24716.

DOI:10.4103/0028-3886.24716
PMID:16679652
Abstract

We described a 69-year-old woman with neurological manifestations due to mixed connective tissue disease (MCTD). The patient demonstrated subacute cognitive decline, seizure and gait disturbance with no connective tissue manifestation. She had been diagnosed with dementia at another hospital, later in our hospital, serological examinations disclosed high titers of anti-RNP antibody. Cognitive dysfunction in this patient was dramatically ameliorated by steroid therapy. Three months later, she developed edema of the hands, synovitis and acrosclerosis. The patient was finally diagnosed as having MCTD. We emphasized MCTD as a rare cause of "treatable dementia".

摘要

我们描述了一位69岁患有混合性结缔组织病(MCTD)并伴有神经学表现的女性。该患者表现为亚急性认知功能衰退、癫痫发作和步态障碍,无结缔组织病表现。她在另一家医院被诊断为痴呆,后来在我院,血清学检查发现抗RNP抗体高滴度。该患者的认知功能障碍通过类固醇治疗得到显著改善。三个月后,她出现手部水肿、滑膜炎和肢端硬化。该患者最终被诊断为MCTD。我们强调MCTD是“可治疗性痴呆”的罕见病因。

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