以神经束衣炎为首发表现的肺非朗格汉斯细胞组织细胞增生症的临床特点(附1例报告)  

Clinical features of lung non-langerhans cell histiocytosis with the first clinical manifestation of perineuritis(report of 1 case)

作　　者：吕鹤[1] 张巍[1] 王朝霞[1] 左越焕[1] 刘靖[1] 袁云[1] 

机构地区：[1]北京大学第一医院神经内科,100034

出　　处：《临床神经病学杂志》2016年第3期203-205,共3页Journal of Clinical Neurology

基　　金：国家自然科学基金(81541118)

摘　　要：目的探讨以神经束衣炎为首发表现的肺非朗格汉斯细胞组织细胞增生症的临床特点。方法回顾性分析1例以神经束衣炎为首发表现的肺非朗格汉斯细胞组织细胞增生症患者的临床资料。结果患者为36岁男性,出现多发性单神经炎以及多发性周围神经病,同时出现严重的肺部病变。抗神经节苷酯2(GM2)IgM抗体增高。神经传导速度证实存在广泛感觉、运动神经病。血清GM2 IgM抗体显著升高。对此患者进行神经活检以及肺组织活检。腓肠神经活检证实显著的神经束衣炎伴随大量神经纤维丢失。肺组织活检为非郎格汉斯组织细胞增生症。结论本病例首次证实神经束衣炎可以出现在肺非郎格汉斯组织细胞增生症,运动、感觉神经均可受累。GM2 IgM抗体可能参与疾病的发生。Objective To explore the clinical features of lung non-langerhans cell histiocytosis with the first clinical manifestation of perineuritis. Methods The clinical data of 1 lung non-langerhans cell histiocytosis with the first clinical manifestation of perineuritis patient was retrospectively analyzed. Results A 36-year-old man suffered from multiple mononeuritis at beginning and then developed polyneuropathy and pulmonary infection. Nerve conduction study showed widespread sensorimotor neuropathy. Anti-gangliosidosis 2（GM2） IgM antibody elevated significantly in his serum. Sural nerve biopsy and lung biopsy were performed for diagnosis. Sural nerve biopsy demonstrated considerable perineuritis with loss of nerve fibers. The lung biopsy confirmed the diagnosis of nonlangerhans cell histiocytosis. Conclusions The presented case confirmed the concomitant of perineuritis and lung non-Langerhans cell histiocytosis for the first time. Both sensory and motor nerve could be involved. Anti-GM2 IgM antibody might play a role in the pathogenesis.

关 键 词：神经束衣炎 肺非朗格汉斯组织细胞增生症 抗GM2 IGM抗体 免疫性周围神经病 

分 类 号：R563[医药卫生—呼吸系统]