本文报告一例40岁女性,缓慢发生的多发性神经病,皮肤色素沉着,浮肿、肝脏肿大、腹水、无月经、视乳头水肿、脑脊液蛋白细胞解离,血清中有IgA型M蛋白,骨髓中浆细胞增加,内分泌机能低下,腓浅神经病理检查示轴索变性,粗经有髓神经纤维明显减少,肌电为神经原性改变。诊断为Crow-Fukase氏综合征,经肾上腺皮质激素及为期三周的血浆置换疗法,病情缓解。 This article reports a 40-year-old female with a slow onset of polyneuropathy, skin pigmentation, edema, liver enlargement, ascites, no menstruation, papilledema, dissociation of cerebrospinal fluid protein cells, serum IgA-type M protein, Increased cells, endocrine dysfunction, shallow peroneal neuropathology showed axonal degeneration, coarse myelinated nerve fibers decreased significantly, EMG neurogenic changes. Diagnosis of Crow-Fukase’s syndrome, the adrenal cortex hormones and three-week plasmapheresis therapy, remission.