IGG MONOCLONAL PARAPROTEINEMIA AND PERIPHERAL NEUROPATHY
IGG MONOCLONAL PARAPROTEINEMIA AND PERIPHERAL NEUROPATHY
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DOI：
10.1136/jnnp.56.1.52
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发表时间：
1993-01-01
影响因子：
11
通讯作者：
MCLEOD, JG
中科院分区：
文献类型：
--
作者：
BLEASEL, AF;HAWKE, SHB;MCLEOD, JG
本文报告5例周围神经病伴良性IgG单克隆副蛋白血症患者，均为感觉运动神经病，有缓解期和复发期。血清副蛋白水平与患者的临床状态无关。电生理检查显示4例患者传导速度明显减慢，传导阻滞，另1例轻度。腓肠神经活检显示，在每五名患者的炎性细胞浸润的脱髓鞘神经病变。三名患者的髓鞘/雪旺细胞反应的免疫荧光研究的证据，并在所有的神经密集表达的主要组织相容性复合物I类和II类分子是明显的神经内膜，入侵的单核细胞，内皮细胞和雪旺细胞。所有的病人都对治疗有反应，血浆置换法特别有效。4名患者在停止所有治疗后获得长期缓解。这5例周围神经病和IgG副蛋白血症的临床、电生理和病理特征与慢性炎性脱髓鞘性多发性神经病患者相同。
Five patients with peripheral ne uropathy and benign IgG monoclonal paraproteinaemia are reported, all of whom had a sensorimotor neuropathy with a remitting and relapsing course. The serum paraprotein level did not correlate with the patient's clinical status. Electrophsyiological studies showed marked slowing of conduction velocity and conduction block in four of the patients and mild showing in the other. Sural nerve biopsies demonstrated a demyelinating neuropathy with inflammatory cell infiltrates in each of the five patients. Three of the patients had evidence of myelin/Schwann cell reactivity on immunofluorescence studies and in all nerves dense expression of major histocompatability complex class I and II molecules was evident within the endoneurium, on invading mononuclear cells, endothelial cells and Schwann cells. All the patients responded to treatment, plasmapheresis being particularly effective. Four patients have achieved prolonged remissions after all treatment had ceased. These five cases of peripheral neuropathy and IgG paraproteinaemia were identical in their clinical, electrophysiological and pathological features to patients with chronic inflammatory demyelinating polyneuropathy.