复发性多软骨炎神经系统损害的临床特点

Clinical characteristics of neurological involvement in patients with relapsing polychondritis

目的探讨复发性多软骨炎(relapsing polychondritis RP)并发神经系统病变的临床特点。方法回顾性分析2005年12月至2019年2月在北京协和医院住院,并确诊为RP并发神经系统病变患者的临床资料。结果RP神经系统病变患者占同期住院RP患者的16.4%(33/201)。女性14例,男性19例,确诊时平均年龄为51.55岁,9例患者以神经系统病变为首发症状。24例患者为单纯中枢神经系统(central nervous system CNS)受累,其中10例患者同时有2种或2种以上CNS病变。5例患者出现单纯周围神经系统受累,均为周围感觉神经系统受累,4例患者同时出现中枢和周围神经系统病变。脑炎(包括自身免疫性边缘性脑炎)和缺血性脑卒中出现在10例患者中,是最常见的RP神经系统受累形式。结论RP神经系统受累的表现多样,脑炎和缺血性脑卒中是RP最常见的神经系统受累形式。

Objective To investigate the clinical characteristics of neurological involvement in patients with relapsing polychondritis(RP).Methods The clinical records of RP patients,hospitalized at Peking Union Medical College Hospital between December 2005 and February 2019,were retrospectively collected.Results The ratio of RP with neurological involvement in hospitalized RP patients was 16.4%(33/201).There were 14 women and 19 men.The average age at diagnosis was 55.15 years old.Nine patients initiated with neurological disorders.There were 24 patients presenting only with central nervous system(CNS)involvement.Among them,10 RP patients had two or more kinds of CNS involvement.There were 5 patients presenting with only peripheral nervous system involvement.In detail,peripheral sensory nervous system was involved in all five patients.In addition,four patients were with concurrent involvement of CNS and peripheral nervous system.Encephalitis including autoimmune limbic encephalitis and ischemic stroke occurred in 10 patients respectively,and were the most common in the RP patients with nervous system involvement.Conclusion The clinical manifestations of RP patients with nervous system involvement were varying.The encephalitis and ischemic stroke were the most common neurological manifestations in our study.