CAJ | 학술논문

目的：探讨以周围神经病为首发症状的意义未明的单克隆丙种球蛋白病（ MGUS）的临床特征、实验室检查结果及治疗。方法选取2012年1月-2013年7月浙江大学医学院附属第二医院神经内科住院诊治的以周围神经病为首发症状，经骨髓穿刺及实验室检查证实为MGUS患者4例。回顾性分析患者的临床特征、实验室检查结果、治疗过程及效果。结果4例患者均为中年男性；亚急性及慢性起病，1例以双下肢麻木起病，1例以双下肢麻木伴乏力起病，1例以四肢乏力起病，1例以四肢麻木伴乏力起病；病程为3个月~10年。4例患者血常规、肝功能、肾功能、血钾、血钙均在参考范围内；均有免疫球蛋白增高及不同程度脑脊液蛋白升高；尿本周蛋白阴性；免疫固定电泳提示IgAλ型2例，IgGλ型和IgMλ型各1例；1例患者血λ轻链增高；3例患者血β2微球蛋白增高；骨髓穿刺结果显示浆细胞增多，部分伴有异型及幼稚形态；肌电图检查以轴索损害为主2例，以脱髓鞘及轴索损害并存2例。4例患者中1例应用中药治疗，最终病情恶化死亡；2例采用沙利度胺及泼尼松治疗，其中1例症状无加重，1例病情仍进展；1例单用泼尼松治疗，症状无加重。结论以周围神经病为首发症状的MGUS常呈亚急性及慢性起病，表现为四肢麻木乏力，肌电图提示存在周围神经损害表现，免疫固定电泳提示单克隆丙种球蛋白增高，可试用皮质类固醇激素、静脉免疫球蛋白和血浆置换治疗。
목적：탐토이주위신경병위수발증상적의의미명적단극륭병충구단백병（ MGUS）적림상특정、실험실검사결과급치료。방법선취2012년1월-2013년7월절강대학의학원부속제이의원신경내과주원진치적이주위신경병위수발증상，경골수천자급실험실검사증실위MGUS환자4례。회고성분석환자적림상특정、실험실검사결과、치료과정급효과。결과4례환자균위중년남성；아급성급만성기병，1례이쌍하지마목기병，1례이쌍하지마목반핍력기병，1례이사지핍력기병，1례이사지마목반핍력기병；병정위3개월~10년。4례환자혈상규、간공능、신공능、혈갑、혈개균재삼고범위내；균유면역구단백증고급불동정도뇌척액단백승고；뇨본주단백음성；면역고정전영제시IgAλ형2례，IgGλ형화IgMλ형각1례；1례환자혈λ경련증고；3례환자혈β2미구단백증고；골수천자결과현시장세포증다，부분반유이형급유치형태；기전도검사이축색손해위주2례，이탈수초급축색손해병존2례。4례환자중1례응용중약치료，최종병정악화사망；2례채용사리도알급발니송치료，기중1례증상무가중，1례병정잉진전；1례단용발니송치료，증상무가중。결론이주위신경병위수발증상적MGUS상정아급성급만성기병，표현위사지마목핍력，기전도제시존재주위신경손해표현，면역고정전영제시단극륭병충구단백증고，가시용피질류고순격소、정맥면역구단백화혈장치환치료。
Objective To investigate the clinical features,laboratory findings and treatment monoclonal gammopathy of undetermined significance( MGUS)with peripheral neuropathy as its first symptom. Methods Four cases of MGUS with pe-ripheral neuropathy as its first symptom admitted to the neurology department of the Second Affiliated Hospital of Zhejiang Univer-sity School of Medicine from January 2012 to July 2013 were selected,and their clinical features,laboratory findings and treat-ment were retrospectively analyzed. Results The four patients were all middle-aged men with subacute or chronic onset between 40-50 years old. Four cases firstly presented respectively as numbness of lower limbs,numbness and weakness of lower limbs, weakness of four limbs and numbness and weakness of four limbs. The course of the disease lasted from three months to ten years. The blood routine,liver function,kidney function,serum potassium and serum calcium of the four patients were all within normal limits. All patients had increased Ig and different increased levels of protein in cerebrospinal fluid,and the Bence-Jonces protein was negative. The immunofixation electrophoresis tests revealed two cases of IgAλ type,one case of IgGλ type and one case of IgMλ type. One case showed increased serum λ light chain, and three cases showed increased serum β2 microglobu-lin. Bone marrow aspiration showed increased plasma cell,with some demonstrating abnormal and immature shape. The electro-myography examination showed the axonal damage in two cases and demyelination with axonal damage in the other two cases. One case out of the four patients was given traditional Chinese medicine treatment,and ultimately died of disease progression;two ca-ses were given thalidomide and prednisone therapy,and one case showed non-aggravated symptoms and one case had progressive disease;one case was given treatment of prednisone alone,and showed no development of the symptoms. Conclusion MGUS with peripheral neuropathy as the first symptom often has subacute and chronic onset, and often shows numbness and weak-ness. Electromyography examination suggests the presence of peripheral nerve damage. Immunofixation electrophoresis showed in-creased monoclonal gamma globulin. Corticosteroids,intravenous immunoglobulin and plasma exchange therapy can be treatment approaches.