复发性多软骨炎相关神经系统损害28例临床分析  

Clinical analysis of 28 cases of relapsing polychondritis with nervous system involvement

作　　者：刘子悦 姜楠[2] 彭斌[1] 曾小峰[2] 崔丽英[1] 朱以诚[1] Liu Ziyue;Jiang Nan;Peng Bin;Zeng Xiaofeng;Cui Liying;Zhu Yicheng(Department of Neurology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China;Department of Rheumatology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医院神经科,100730 [2]中国医学科学院北京协和医院风湿免疫科,100730

出　　处：《中华神经科杂志》2020年第12期1021-1027,共7页Chinese Journal of Neurology

摘　　要：目的探讨复发性多软骨炎(RP)相关神经系统损害的临床、影像、生化检查改变、治疗方案及预后特点,以提高临床医生对本病的认识。方法搜集2005年12月至2019年11月北京协和医院收治的28例RP患者的临床资料并随访其预后信息,进行回顾性分析。结果患者中位年龄50岁,57.1%(16/28)为男性。其中26例为中枢神经系统受累,常见症状为头痛(69.2%,18/26)、认知下降(38.5%,10/26)、精神行为异常(38.5%,10/26)等;仅2例出现周围神经系统损害。头颅MRI检查显示常见受累部位是皮质下(13/18)及皮质(8/18)组织。脑脊液常表现为白细胞升高(中位数55×106/L)、蛋白正常或轻度升高[(0.54±0.17)g/L]。全部患者接受了糖皮质激素治疗,24例(85.7%)接受了环磷酰胺等免疫抑制剂治疗;27例呈现不同程度的症状缓解,但24例好转后曾出现病情复发;认知功能障碍是最常见的遗留症状。有16例得到随访信息,平均随访时间3.3年,10例患者病情稳定,共3例死亡,死亡病例平均总病程8.6年。结论RP病程中可能出现诸多神经系统损害表现,以急性或亚急性边缘叶脑炎、脑膜脑炎或脑膜炎等临床综合征较为多见。但其临床表现及辅助检查均缺乏特异性,诊断具有一定难度,需全面排除其他病因并尽早使用免疫抑制治疗。Objective To explore the clinical profile and prognostic features of relapsing polychondritis(RP)with nervous system involvement.Methods The clinical data of 28 RP patients with nervous system involvement admitted to Peking Union Medical College Hospital from December 2005 to November 2019 were collected and analyzed.Results The median age of the patients was 50 years and 57.1%(16/28)were men.Patients with central nervous system involvement accounted for 92.9%(26/28)of the total.Common manifestations included headache(69.2%,18/26),cognitive impairment(38.5%,10/26),and mental disorders(38.5%,10/26);only two cases had peripheral nervous system damage.Brain magnetic resonance imaging revealed that subcortical(13/18)and cortical(8/18)tissues were most affected.Cerebrospinal fluid tests showed that the median white blood cell count was 55×106/L,while the protein level was also slighted elevated((0.54±0.17)g/L).All patients were treated with glucocorticoids,and 85.7%(24/28)of patients with immunosuppressants;27 patients showed optimistic responses to the treatment,while 24 had recurrent courses.Cognitive dysfunction was the most common residual symptom.Follow-up data of 16 patients were available with an average follow-up time of 3.3 years.Ten of the patients were in stable condition and three patients died.Conclusions Nervous system damage,especially limbic encephalitis and meningitis may present in course of RP.However,it is a challenge to diagnose for the non-specific clinical manifestations.Treatment with corticosteroids and immunosuppressants can lead to a favorable response.

关 键 词：多软骨炎 复发性 神经系统 诊断 治疗 

分 类 号：R747.9[医药卫生—神经病学与精神病学] R681.3[医药卫生—临床医学]