Donofrio Peter D
Department of Neurology, Wake Forest University School of Medicine, Medical Center Boulevard, Winston-Salem, North Carolina 27157-1078, USA.
Muscle Nerve. 2003 Sep;28(3):273-92. doi: 10.1002/mus.10402.
Evaluation of peripheral neuropathy is a common reason for referral to a neurologist. Recent advances in immunology have identified an inflammatory component in many neuropathies and have led to treatment trials using agents that attenuate this response. This article reviews the clinical presentation and treatment of the most common subacute inflammatory neuropathies, Guillain-Barré syndrome (GBS) and Fisher syndrome, and describes the lack of response to corticosteroids and the efficacy of treatment with plasma exchange and intravenous immunoglobulin (IVIG). Chronic inflammatory demyelinating polyneuropathy, although sharing some clinical, electrodiagnostic, and pathologic similarities to GBS, improves after treatment with plasma exchange and IVIG and numerous immunomodulatory agents. Controlled trials in multifocal motor neuropathy have shown benefit after treatment with IVIG and cyclophosphamide. Also discussed is the treatment of less common inflammatory neuropathies whose pathophysiology involves monoclonal proteins or antibodies directed against myelin-associated glycoprotein or sulfatide. Little treatment data exist to direct the clinician to proper management of rare inflammatory neuropathies resulting from osteosclerotic myeloma; POEMS syndrome; vasculitis; Sjögren's syndrome; and neoplasia (paraneoplastic neuropathy).
评估周围神经病变是转诊至神经科医生处的常见原因。免疫学的最新进展已在许多神经病变中识别出炎症成分，并引发了使用能减轻这种反应的药物进行治疗试验。本文综述了最常见的亚急性炎症性神经病变——吉兰-巴雷综合征（GBS）和费舍尔综合征的临床表现及治疗方法，并描述了其对皮质类固醇治疗无反应以及血浆置换和静脉注射免疫球蛋白（IVIG）治疗的疗效。慢性炎症性脱髓鞘性多发性神经病变虽然在临床、电诊断和病理方面与GBS有一些相似之处，但在接受血浆置换、IVIG及多种免疫调节药物治疗后病情有所改善。多灶性运动神经病变的对照试验表明，IVIG和环磷酰胺治疗后有益。文中还讨论了较少见的炎症性神经病变的治疗，其病理生理学涉及单克隆蛋白或针对髓鞘相关糖蛋白或硫脂的抗体。目前几乎没有治疗数据能指导临床医生对由骨硬化性骨髓瘤、POEMS综合征、血管炎、干燥综合征和肿瘤（副肿瘤性神经病变）引起的罕见炎症性神经病变进行恰当管理。
