Briemberg Hannah R, Levin Kerry, Amato Anthony A
From the *Department of Neurology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts; and the daggerDepartment of Neurology, Cleveland Clinic, Cleveland, Ohio, U.S.A.
J Clin Neuromuscul Dis. 2002 Jun;3(4):153-8. doi: 10.1097/00131402-200206000-00004.
Peripheral nervous system (PNS) vasculitis and Guillain-Barré syndrome (GBS) are two distinct entities. Although there may be similarities in clinical presentation, the two are rarely confused. PNS vasculitis typically presents as a mononeuritis multiplex, as an overlapping mononeuritis multiplex, or as a distal symmetric sensorimotor polyneuropathy. Electrophysiologic studies are consistent with a primary axonal pathophysiologic process. In contrast, GBS typically presents with variable, mild sensory symptoms followed by symmetric progressive weakness. Early electrophysiologic studies, when abnormal, usually demonstrate findings consistent with demyelination. We describe two cases of PNS vasculitis in which the initial clinical presentation and the presence of multifocal conduction block on electrophysiologic studies led to the incorrect diagnosis of GBS early in the hospital course. Although GBS must always be considered in patients with rapidly progressive weakness, physicians must remain vigilant for alternative diagnoses, as illustrated by our cases.
周围神经系统（PNS）血管炎和吉兰-巴雷综合征（GBS）是两种不同的疾病。尽管临床表现可能有相似之处，但二者很少被混淆。PNS血管炎通常表现为多发性单神经炎、重叠性多发性单神经炎或远端对称性感觉运动性多发性神经病。电生理研究结果与原发性轴索性病理生理过程一致。相比之下，GBS通常先出现多变的轻度感觉症状，随后出现对称性进行性肌无力。早期电生理研究若有异常，通常显示与脱髓鞘相符的结果。我们描述了2例PNS血管炎病例，其最初的临床表现以及电生理研究中多灶性传导阻滞的存在导致在病程早期被误诊为GBS。尽管对于快速进展性肌无力的患者必须始终考虑GBS，但正如我们的病例所示，医生必须对其他诊断保持警惕。
