Lozeron Pierre, Adams David
Department of Neurology, Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris-Sud, Paris, France.
Curr Opin Neurol. 2007 Oct;20(5):536-41. doi: 10.1097/WCO.0b013e3282ef79e3.
To provide clinically useful guidelines in the management of neuropathy associated with monoclonal gammopathy from a review of the most recent literature and our own experience.
Recent data on neuropathy associated with monoclonal gammopathy come from better descriptions of subgroups, and from new treatment compounds that have shown encouraging results in different entities.
Neuropathies associated with monoclonal gammopathy are relatively rare and most often the neuropathy reveals the monoclonal gammopathy. These conditions require combined neurological and haematological assessments. Their clinical presentations are highly heterogeneous but most have an electrophysiological demyelinating pattern. The main described subgroup is IgM anti-(myelin-associated glycoprotein) neuropathy, which presents as a relatively benign, slowly progressive sensory neuropathy. Nerve biopsy should be considered in patients with progressive and disabling axonal neuropathy. Neuropathies associated with monoclonal gammopathy have various neurological and general outcomes, including life-threatening entities such as light-chain amyloid neuropathy and POEMS syndrome. Treatment choice is wide and depends both on the underlying haematological disorder and severity of the neuropathy. Intravenous immunoglobulin should be assessed in demyelinating monoclonal gammopathy of undetermined significance neuropathy. Malignant haematological disorders should be treated per se. The possibility of a malignant evolution of monoclonal gammopathy of undetermined significance warrants regular haematological monitoring.
通过回顾最新文献及我们自身的经验,为单克隆丙种球蛋白病相关神经病变的管理提供临床实用指南。
关于单克隆丙种球蛋白病相关神经病变的最新数据来自对亚组的更详尽描述,以及在不同类型中显示出令人鼓舞结果的新治疗化合物。
与单克隆丙种球蛋白病相关的神经病变相对罕见,且神经病变常揭示单克隆丙种球蛋白病。这些情况需要神经科和血液科联合评估。它们的临床表现高度异质性,但大多数具有电生理脱髓鞘模式。主要描述的亚组是IgM抗(髓鞘相关糖蛋白)神经病变,表现为相对良性、进展缓慢的感觉神经病变。对于进行性和致残性轴索性神经病变患者应考虑神经活检。与单克隆丙种球蛋白病相关的神经病变有各种神经学和总体预后,包括危及生命的类型,如轻链淀粉样变神经病变和POEMS综合征。治疗选择广泛,取决于潜在的血液系统疾病和神经病变的严重程度。对于意义未明的脱髓鞘性单克隆丙种球蛋白病相关神经病变,应评估静脉注射免疫球蛋白。恶性血液系统疾病应本身进行治疗。意义未明的单克隆丙种球蛋白病发生恶性演变的可能性需要定期进行血液学监测。