白塞病并发脊髓病变6例临床分析并文献复习  

Spinal Cord Involvement in Behet's Disease:a Case Series and Literature Review

作　　者：袁伟 严冬[1,3] 张跃华 彭琳一 吴迪[1] 沈敏[1] 吴庆军[1] 郑文洁[1] YUAN Wei;YAN Dong;ZHANG Yue-hua;PENG Lin-Yi;WU Di;SHEN Min;WU Qing-jun;ZHENG Wen-jie(Department of Rheumatology,Peking Union Medical College Hospital,Chinese Academy of Medical sciences&Peking Union Medical College,Beijing l00730,China)

机构地区：[1]中国医学科学院,北京协和医学院,北京协和医院风湿免疫科,北京100730 [2]华北理工大学附属开溧总医院风湿免疫科,河北唐山063000 [3]苏州大学附属第二医院风湿免疫科,苏州215004 [4]河北医科大学第四医院肿瘤科(免疫),石家庄050000

出　　处：《中华临床免疫和变态反应杂志》2017年第4期333-338,共6页Chinese Journal of Allergy & Clinical Immunology

基　　金：国家自然科学基金(81571598)

摘　　要：目的研究白塞病(Behet's disease,BD)并发脊髓病变的临床特点、治疗及预后。方法报道北京协和医院2004年2月至2016年12月住院的BD并发脊髓病变患者的临床资料,并复习相关文献。结果 BD并发脊髓病变患者6例,占同期神经BD患者的8%(6/71)。均为男性,脊髓病变发病年龄13~37岁,平均(28±9)岁。均以下肢无力为首发表现,临床表现为感觉异常2例,尿失禁5例,尿潴留2例,大便失禁2例,性功能障碍1例,4例伴有不同程度发热,6例患者均有锥体束征阳性。脑脊液:细胞数升高5例,细胞学检查5例为淋巴细胞性炎症,1例为淋巴中性粒细胞炎症,脑脊液生化:6例患者均有蛋白轻度升高。磁共振成像(magnetic resonance imaging,MRI)示6例均有颈髓损害,1例并发胸髓损害。均给予糖皮质激素联合免疫抑制剂治疗,生物制剂治疗2例,5例好转,1例反复复发。结论 BD并发脊髓损害较为少见,临床常表现为下肢无力、感觉异常及括约肌功能障碍,脑脊液表现为细胞数升高,蛋白轻度升高,脊髓MRI有助于明确诊断,早期诊断、积极治疗,有助于改善预后。Objective To evaluate the clinical characteristics,treatment,and prognosis of Beh et s disease(BD)patients with spinal cord involvement.Methods The clinical features,laboratory tests,imaging studies,treatment,and outcome of 6 patients with BD complicated with spinal cord involvement at Peking Union Medical College Hospital from February 2004 to December 2016 were retrospectively analyzed.Results Among 71 patients with Neuro-BD,6 patients(8%)had spinal cord involvement.All patients were male,and the average age of disease onset was(28±9)years old(range,13-37 years old).The most frequent neurological signs were paraparesis(6 patients),sensory deficits(2 patients),urinary incontinence(5 patients),urinary retention(2 patients),fecal incontinence(2 patients),sexual dysfunction(1 patient),and febrile(4 patients).All the patients had pyramidal signs.Cerebrospinal fluid(CSF)showed pleocytosis and elevated protein levels.All the patients had cervical cord involved,and one patient had thoracic cord involved as well.Six patients were all treated with corticosteroids and immunosuppressants,while 2 patients were treated with biological agents.Five patients were improved,but one patient relapsed.Conclusions Spinal cord involvement is a relatively rare complication of BD,which manifests mainly as paraparesis,sensory deficits and sphincter dysfunction.CSF reveals pleocytosis and elevated protein levels.Spinal MRI is useful for early diagnosis of spinal cord lesions.Early diagnosis and aggressive management is advocated.

关 键 词：白塞病 脊髓受累 临床特点 

分 类 号：R597.9[医药卫生—内科学] R744[医药卫生—临床医学]