Donofrio P D, Kelly J J
Department of Neurology, Bowman Gray School of Medicine, Wake Forest University, Winston-Salem, NC.
Muscle Nerve. 1989 Jan;12(1):1-8. doi: 10.1002/mus.880120102.
Monoclonal gammopathy of undetermined significance (MGUS) is the most common paraproteinemia associated with polyneuropathy. Although the clinical and electrodiagnostic manifestations most resemble those of chronic inflammatory demyelinating polyneuropathy, some patients manifest a pure sensory neuropathy or neuronopathy. The M protein is usually IgM, and its concentration in serum is low. Nerve pathology from patients with demyelinating disease shows a reduction of large myelinated fibers and segmental demyelination with remyelination. In some cases, the M protein possesses antibody activity against components of the myelin sheath or axon. These neuropathies may respond to treatment with steroids, immunosuppressant agents, and plasma exchange.
意义未明的单克隆丙种球蛋白病（MGUS）是与多发性神经病相关的最常见的副蛋白血症。虽然其临床和电诊断表现与慢性炎症性脱髓鞘性多发性神经病最为相似，但一些患者表现为纯感觉性神经病或神经元病。M蛋白通常为IgM，其血清浓度较低。脱髓鞘疾病患者的神经病理学表现为大的有髓纤维减少以及节段性脱髓鞘伴髓鞘再生。在某些情况下，M蛋白具有针对髓鞘或轴突成分的抗体活性。这些神经病可能对类固醇、免疫抑制剂和血浆置换治疗有反应。
