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系统性硬化症(硬皮病)的周围神经肌肉表现

Peripheral neuromuscular manifestations in systemic sclerosis (scleroderma).

作者信息

Hietaharju A, Jääskeläinen S, Kalimo H, Hietarinta M

机构信息

Department of Clinical Sciences, University of Tampere, Finland.

出版信息

Muscle Nerve. 1993 Nov;16(11):1204-12. doi: 10.1002/mus.880161110.

Abstract

Systemic sclerosis (scleroderma) is thought to be the least likely of the collagen vascular disorders to cause nervous system damage. We evaluated the peripheral neuromuscular manifestations in 32 patients with scleroderma. A clinically defined peripheral nervous system (PNS) lesion was manifest in 5 of 32 patients (16%), including 2 patients with trigeminal neuropathy and single cases of polyneuropathy, brachial plexopathy, and lumbosacral radiculopathy. Neurophysiological studies suggested subclinical PNS involvement in 6 additional patients (3 with distal axonal polyneuropathy, 1 with probable myopathy and superimposed polyneuropathy, 1 with trigeminal neuropathy, and 1 with focal ulnar neuropathy). Even though subjective muscular complaints were numerous (16 patients, 50%), a defined primary muscular disease could be demonstrated only in 5 patients (16%). Our results indicate that peripheral neuropathy in scleroderma is not as uncommon as previously estimated.

摘要

系统性硬化症(硬皮病)被认为是胶原血管疾病中最不可能导致神经系统损害的疾病。我们评估了32例硬皮病患者的周围神经肌肉表现。32例患者中有5例(16%)出现了临床定义的周围神经系统(PNS)病变,其中包括2例三叉神经病变患者以及单发性多发性神经病、臂丛神经病变和腰骶神经根病变各1例。神经生理学研究表明,另有6例患者(3例为远端轴索性多发性神经病,1例可能为肌病合并多发性神经病,1例为三叉神经病变,1例为局灶性尺神经病变)存在亚临床PNS受累。尽管主观肌肉症状很多(16例患者,50%),但仅5例患者(16%)可证实存在明确的原发性肌肉疾病。我们的结果表明,硬皮病中的周围神经病变并不像先前估计的那样罕见。

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