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吉兰-巴雷综合征的恢复模式。

Patterns of recovery in the Guillain-Barre syndromes.

作者信息

Ho T W, Li C Y, Cornblath D R, Gao C Y, Asbury A K, Griffin J W, McKhann G M

机构信息

Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

出版信息

Neurology. 1997 Mar;48(3):695-700. doi: 10.1212/wnl.48.3.695.

Abstract

Clinical, electrodiagnostic, and pathologic studies indicate that the Guillain-Barre syndromes (GBSs) include both primary demyelinating and primary axonal forms. The axonal forms are usually thought to have a poorer prognosis, with less chance for rapid or complete recovery. In northern China, epidemics of one axonal form, acute motor axonal neuropathy (AMAN), occur annually in the summer. Autopsy studies in some fatal cases have demonstrated wallerian-like degeneration of motor roots and motor fibers in the peripheral nerves. Recovery of such patients would require axonal regeneration along the entire length of the nerve fiber. In a 2-year prospective study of GBS at a single hospital in northern China, 42 patients were classified as having either AMAN (32 patients), acute inflammatory demyelinating polyneuropathy (AIDP) (8 patients), or as undetermined (2 patients) by electrodiagnostic criteria. Their recoveries were monitored clinically. The recovery times of AMAN and AIDP patients were similar: the median time to regain the ability to walk 5 meters with assistance was 31 days for patients classified as having AMAN and 32 days for those classified as having AIDP. These rapid recovery times are incompatible with severe wallerian degeneration of the ventral roots and motor nerve fibers. The rapid recoveries observed in AMAN patients could be explained by relatively quickly reversible immune-mediated changes at nodes of Ranvier in motor fibers, by degeneration and regeneration of intramuscular motor nerve terminals, or both.

摘要

临床、电诊断和病理研究表明,吉兰 - 巴雷综合征(GBS)包括原发性脱髓鞘型和原发性轴索性。通常认为轴索性的预后较差,快速或完全恢复的机会较少。在中国北方,一种轴索性类型,即急性运动轴索性神经病(AMAN),每年夏季都会流行。一些致命病例的尸检研究显示,周围神经的运动根和运动纤维出现了华勒氏样变性。这类患者的恢复需要神经纤维全长的轴突再生。在中国北方一家医院对GBS进行的为期2年的前瞻性研究中,根据电诊断标准,42例患者被分类为患有AMAN(32例)、急性炎症性脱髓鞘性多发性神经病(AIDP)(8例)或未明确类型(2例)。对他们的恢复情况进行了临床监测。AMAN和AIDP患者的恢复时间相似:被分类为患有AMAN的患者在辅助下能够行走5米的中位时间为31天,被分类为患有AIDP的患者为32天。这些快速的恢复时间与腹侧神经根和运动神经纤维的严重华勒氏变性不相符。AMAN患者观察到的快速恢复可以通过运动纤维郎飞结处相对快速可逆的免疫介导变化、肌内运动神经末梢的变性和再生,或两者来解释。

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