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急性免疫性多发性神经病的其他区域变异型。伴有感觉异常的双侧面神经麻痹或展神经麻痹、腰骶部多发性神经根病以及伴有咽-颈-臂肌无力的共济失调。

Further regional variants of acute immune polyneuropathy. Bifacial weakness or sixth nerve paresis with paresthesias, lumbar polyradiculopathy, and ataxia with pharyngeal-cervical-brachial weakness.

作者信息

Ropper A H

机构信息

Neurology Service, St Elizabeth's Medical Center, Boston, Mass.

出版信息

Arch Neurol. 1994 Jul;51(7):671-5. doi: 10.1001/archneur.1994.00540190051014.

Abstract

OBJECTIVE

To describe four syndromes of acute regional weakness with clinical, spinal fluid, and electrophysiologic similarities to the acute immune polyneuropathy of Guillain-Barré syndrome.

DESIGN

Case series of personally examined patients.

RESULTS

Seven patients are described: four with facial diplegia and distal limb paresthesias, one with sixth nerve palsy and distal paresthesias, one with bilateral lumbar polyradiculopathy, and one with combined Fisher's syndrome and pharyngeal-cervical-brachial weakness. These self-limited illnesses, which evolved over days or weeks, involved acellular cerebrospinal fluid with raised protein concentration and electrophysiologic findings that were consistent with a demyelinating polyneuropathy.

CONCLUSIONS

The first three regional variants of Guillain-Barré syndrome may cause diagnostic difficulty, particularly at the onset of illness, and the fourth links Fisher's syndrome with the typical syndrome. The consistently bilateral weakness of Guillain-Barré syndrome and its regional variants and the absence of a monoparetic or hemiparetic pattern suggest that the pathologic process occurs in the same single or contiguous groups of nerves on both sides of the sagittal plane but is not randomly distributed in the peripheral nervous system.

摘要

目的

描述四种急性局部无力综合征,其在临床、脑脊液及电生理方面与吉兰 - 巴雷综合征的急性免疫性多发性神经病相似。

设计

对亲自检查的患者进行病例系列研究。

结果

描述了7例患者:4例有双侧面瘫和肢体远端感觉异常,1例有第六脑神经麻痹和远端感觉异常,1例有双侧腰骶神经根病,1例合并费舍尔综合征和咽 - 颈 - 臂肌无力。这些自限性疾病在数天或数周内进展,脑脊液无细胞但蛋白浓度升高,电生理检查结果与脱髓鞘性多发性神经病一致。

结论

吉兰 - 巴雷综合征的前三种局部变异型可能导致诊断困难,尤其是在疾病发作时,第四种变异型将费舍尔综合征与典型综合征联系起来。吉兰 - 巴雷综合征及其局部变异型始终存在双侧无力,且无单瘫或偏瘫模式,这表明病理过程发生在矢状面两侧相同的单个或相邻神经组中,但并非随机分布于周围神经系统。

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