副蛋白血症相关周围神经病20例临床、电生理特点分析  

Clinicoelectrophysiological characteristics of peripheral nerves with paraproteinemia

作　　者：唐鹤飞[1] 张在强[1] 

机构地区：[1]首都医科大学附属北京天坛医院神经内科,100050

出　　处：《中华医学杂志》2015年第4期283-285,共3页National Medical Journal of China

摘　　要：目的 总结副蛋白血症相关周围神经病临床及电生理特点,加深对此病的认识.方法 回顾性分析2008年3月至2012年7月在天坛医院神经内科就诊的明确诊断的副蛋白血症相关周围神经病患者的临床、实验室及神经电生理检查资料,并在不同亚型之间比较.结果 20例患者入组本研究.男12例,女8例,平均年龄52岁.病程2个月至3年,平均病程14个月.其中IgM型2例（10％）、IgG型10例（50％）、IgA型7例（35％）,λ轻链1例（5％）.本组患者以感觉性、轴索性、多发性神经病为主要表现,远端受累为主.其中IgA型及IgG型均以感觉性、轴索性、多发性神经病为主要表现.结论 本组患者以感觉性、轴索性、远端对称性多发性神经病为主要表现.临床表现异质性明显,即使不以感觉性、脱髓鞘性损害为主要表现,亦应考虑此病因可能.在周围神经病病因筛查中,应常规筛查副蛋白血症.Objective To explore the clinicoelectrophysiological characteristics of paraproteinemic neuropathy （PPN）.Methods Retrospective analyses were performed for the clinical data,including clinical characteristics,laboratory and neural electrophysiological examinations,of hospitalized patients with definitively diagnosed PPN from 2008 to 2012.Their objective parameters were compared between different sub-groups.Results There were 12 males and 8 females with an average age of 52 years.The average clinical course was 14 （2-36） months.The types were IgM monoclonal gammopathy （MG） （n =2,10%）,IgG MG （n =10,50%）,IgA MG （n =7,35%） and λ light chain MG （n =1,5%）.This cohort had predominant sensory and axonal polyneuropathy with distal involvement.And the subtype of IgA and IgG manifested predominant sensory and axonal polyneuropathy.Conclusion The PPN patients have predominant sensory,axonal and distal symmetric polyneuropathy with clinical heterogeneities.PPN must be suspected even if sensory impairment and demyelination are not the dominant features.For patients with peripheral neuropathy,typing of monoclonal immunoglobulins should be routinely performed.

关 键 词：副蛋白血症 单克隆丙种球蛋白病 周围神经病 

分 类 号：R745[医药卫生—神经病学与精神病学]