6例Melkersson-Rosenthal综合征的临床、病理及电生理特点  

Clinical, pathological and electrophysiological characteristics of Melkersson-Rosenthal syn- drome： report of 6 cases

作　　者：井峰[1] 黄旭升[1] 陈朝晖[1] 贾渭泉[1] 崔荣太[1] 杨飞[1] 

机构地区：[1]中国人民解放军总医院神经内科,北京100853

出　　处：《癫痫与神经电生理学杂志》2011年第1期28-31,共4页Journal of Epileptology and Electroneurophysiology(China)

摘　　要：目的：分析梅-罗（Melkersson-Rosenthal）综合征（MRS）的临床、病理及电生理特点，以提高对本病的认识和诊断水平。方法：对6例MRS患者的临床表现、病理改变及神经电生理检查结果进行回顾性研究。结果：按MRS的诊断标准，6例中4例为完全型。全部患者均有唇、面部水肿；5例出现周围性面瘫；4例有皱襞舌表现。其中2例家族史阳性。2例患者的病理活检结果支持MRS诊断。神经电生理检查提示：面神经运动传导复合肌动作电位潜伏期延长、波幅降低或引不出波形；面神经支配的肌肉针电极肌电图无运动单位电位，或募集相呈单纯相、峰值减低。结论：MRS的主要临床特征为唇、面部水肿，周围性面瘫及皱襞舌。病程长、反复发作者多预后不佳。神经电生理检查对判断该病的预后有重要意义。Objeetive：To promote the diagnostic level of Melkersson-Rosenthal syndrome （MRS） by analyzing the clinical, pathological and electrophysiological characteristics of MRS. Methods： A retrospective study was made for the clinical features, histopathological findings and electrophysiological re- sults of 6 MRS cases. Results： Four of the six patients with MRS had the classical triad of symptoms ac- cording to the diagnostic criteria of MRS. All the patients had the symptoms of orofacial edema; 5 pa- tients had peripheral facial paralysis; 4 patients had fissured tongues, and 2 of them had positive family history. Pathological findings of two patients supported the diagnosis of MRS. Electrophysiological study of facial nerve demonstrated the distal motor latency was prolonged and the amplitude of CMAPs de- creased or could not be evoked. Needle EMG showed no MUP, reduced recruitment and decreased spike potentials on muscles that were innervated by facial nerves. Conclusion： The most important clinical mani festations of MRS are orofacial edema, peripheral facial paralysis and fissured tongue. A chronically re current course usually indicates a dissatisfactory recovery. Electrophysiological test of facial nerves are valuable in predicting the prognosis.

关 键 词：梅-罗(Melkersson-Rosenthal)综合征(MRS) 病理 肌电图 神经传导 

分 类 号：R747.9[医药卫生—神经病学与精神病学] R741.044[医药卫生—临床医学]