白塞综合征合并急性格林巴利综合征1例  

Co-existence of Guillain-Barrésyndrome and Behcet syndrome:A case report

作　　者：禹琛 李春[2] 范洋溢[3] 徐燕[3] YU Chen;LI Chun;FAN Yang-yi;XU Yan(Department of Cardiollgy,Peking University People's Hospstal,Beijing 100044,China;Department of Rheumatology,Peking University People's Hospstal,Beijing 100044,China;Department of Neurology,Peking University People's Hospstal,Beijing 100044,China)

机构地区：[1]北京大学人民医院心血管内科,北京100044 [2]北京大学人民医院风湿免疫科,北京100044 [3]北京大学人民医院神经内科,北京100044

出　　处：《北京大学学报（医学版）》2020年第6期1146-1149,共4页Journal of Peking University:Health Sciences

摘　　要：白塞综合征(Behcet syndrome,BS)是一种复杂的多系统疾病,临床特征为口腔溃疡、生殖器溃疡、皮肤病变,以及眼、神经等受累。中东和地中海地区的BS患病率高、病情重,因此该病又称“丝绸之路病”。BS属于罕见病的一种,而急性格林巴利综合征同样也属于罕见病。本文报道1例BS合并急性格林巴利综合征的患者,结合以往文献复习,以期进一步提高临床对两种罕见疾病的认识。A 40-year-old male patient was referred to our department with complains of recurrent oral ulcer for more than 20 years and vulvar ulcer for more than 10 years.He presented with a 3-month history of right external ophthalmoplegia.More than 10 days ago,the patient received ganglioside infusion.And one week ago,he developed numbness and pain of his lambs,and progressive myasthenia,accompanied by right blepharoptosis and dysuria.On exam,motor strength was graded 0/5 in the lower and the upper extremities.Deep tendon reflexes were diminished in extremities.His admission medical examination:hemoglobin(HGB),white cell and platelet counts were normal.C-reactive protein(CRP)was negative.Erythrocyte sedimentation rate(ESR)53 mm/h.Antinuclear antibody(ANA),anti-dsDNA antibody,anti-Smith antibody,anti-cardiolipin antibody and human leucocyte antigen B51 were all within normal range.The etiological tests of influenza A pathogen,influenza B pathogen,parainfluenza virus,enterovirus and parvovirus were all negative.He tested positive for serum anti-GM1 IgG.Cerebrospinal fluid had a normal white cell count,an elevated protein content.Gram staining,culture and PCR detection for varicella-zoster virus,cytomegalovirus and herpes simplex virus were all negative.Antibodies associated with autoimmune encephalitis and paraneoplastic syndrome were negative in cerebrospinal fluid.Electromyography and nerve conduction studies showed a severe axonal damage affecting motor nerves.No obvious abnormalities were observed in his magnetic resonance imaging of brain and cavernous sinus.The patient was diagnosed with Behcet syndrome complicated with acute Guillain-Barrésyndrome.He received intravenous methylprednisolone,intravenous immunoglobulin(IVIg)therapy,plasma exchange and rituximab treatment.After treatment,the patient’s muscle strength of limbs was restored to grade 1,blepharoptosis and pain disappeared.The nervous system involvement of Behcet syndrome is relatively rare,especially combined with Guillain-Barrésyndrome,which is easy to

关 键 词：白塞综合征 格林-巴利综合征 病例报告 免疫球蛋白类 静脉内 

分 类 号：R745[医药卫生—神经病学与精神病学]