摘要
目的 总结血管炎性周围神经病的临床电生理及周围神经活检病理特点.方法 回顾性分析北京大学第三医院神经科15例血管炎性周围神经病患者临床表现、电生理特点及腓肠神经活检病理特点.结果 15例患者中,男8例,女7例,年龄38～82岁,周围神经病病程0.5～60个月.12例患者诊断为系统性血管炎性周围神经病,5例为原发性系统性血管炎,其中Churg-Strass综合征（CSS）2例,抗中性粒细胞胞浆抗体（ANCA）相关性血管炎3例;其余7例为继发性系统性血管炎,其中系统性红斑狼疮（SLE）1例,干燥综合征（SS）2例,类风湿关节炎（RA）3例,白塞病伴发甲状腺乳头状癌1例,乙型肝炎1例,1例患者SS伴发RA.3例患者诊断为非系统性血管炎性周围神经病.血管炎病理类型：1型4例,2型2例,3型9例.神经纤维病变类型：轴索变性为主8例,轴索变性伴脱髓鞘7例,其中2例伴局部神经束膜增厚.结论 血管炎性周围神经病的临床特点以多数性单神经病变和非对称性多神经病变多见;电生理特点以轴索病变为主,感觉、运动神经均受累,部分患者伴发脱髓鞘改变;神经病理特点为以轴索病变为主,可伴发脱髓鞘改变.
Objective To summarize the clinical features,electrophysiology and neuropathological characteristics of peripheral nerves in patients with vasculitic neuropathy.Methods We retrospectively analyzed the clinical,electrophysiology and neuropathological characteristics of 15 patients with vasculitic neuropathy who underwent electrophysiology and sural nerve biopsy in our department from January 2009 to June 2013.Results There were 8 males and 7 females,aged from 38 to 82 years old,with a peripheral neuropathy course ranged from 0.5 month to 60 months.In the total of 15 patients,3 patients were diagnosed as nonsystemic vasculitic neuropathy,while the other 12 patients were diagnosed as systemic vasculitis neuropathy （SVN） including 5 cases of primary systemic vasculitis and 7 cases of secondary systemic vasculitis.In patients diagnosed as primary systemic vasculitis,there were 2 cases of Churg-Strass syndrome （CSS） and 3 cases of ANCA associated vasculitis.In patients diagnosed as secondary systemic vasculitis,there were 1 case of systemic lupus erythematosus （SLE）,2 cases of sicca syndrome （SS）,3 cases of rheumatoid arthritis （RA）,1 case of Behcet＇ s disease associated with thyroid papillary carcinoma,1 case of hepatitis B and 1 case of RA-associated SS.For the pathological features of vasculitic neuropathy,type 1 lesion was found in 4 patients,type 2 lesion in 2 patients,and type 3 lesion in 9 patients.Axon degeneration was observed in 8 patients,while 7 patients manifested as axon degeneration associated with demyelination and local thickening of the perineurium was found in 2 patients.Conclusion Multiple mononeuropathy and asymmetric polyneuropathy are the common clinical presentations of vasculitic neuropathy.Electrodiagnostic testing almost always reveals the evidence of a predominantly axonal and sensorimotor process with associated demyelination presented in some cases.Sural nerve biopsy showes changes indicative of an axonopathy.
出处
《中华内科杂志》
CAS
CSCD
北大核心
2014年第5期384-389,共6页
Chinese Journal of Internal Medicine
