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[吉兰-巴雷综合征与白塞病并存:一例报告]

[Co-existence of Guillain-Barré syndrome and Behcet syndrome: A case report].

作者信息

Yu C, Li C, Fan Y Y, Xu Y

机构信息

Department of Cardiology, Peking University People's Hospital, Beijing 100044, China.

Department of Rheumatology, Peking University People's Hospital, Beijing 100044, China.

出版信息

Beijing Da Xue Xue Bao Yi Xue Ban. 2020 Dec 18;52(6):1146-1149. doi: 10.19723/j.issn.1671-167X.2020.06.027.

Abstract

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-Barré syndrome. 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-Barré syndrome, which is easy to cause misdiagnosis. The treatment of Behcet syndrome complicated with acute Guillain-Barré syndrome includes the treatment of primary disease, plasma exchange and IVIg therapy. In addition, supportive treatment is very important for such patients. The focus of treatment is to avoid respiratory insufficiency, prevent deep vein thrombosis, monitor cardiac function and hemodynamics. Pain-relieving, physical exercise and psychological support are often under-recognized. The rehabilitation treatment is very important to improve the prognosis and quality of life of patients. What we need to learn is that when the symptoms and signs of the nervous system are difficult to be explained by neuro-Behcet syndrome alone, we should be alert to the possibility of other nervous system diseases.

摘要

一名40岁男性患者因反复口腔溃疡20余年、外阴溃疡10余年前来我科就诊。他有3个月的右侧眼外肌麻痹病史。10多天前,患者接受了神经节苷脂输注。1周前,他出现双下肢麻木、疼痛,进行性肌无力,伴有右侧上睑下垂和排尿困难。体格检查时,上下肢肌力均为0/5级。四肢腱反射减弱。入院时体格检查:血红蛋白(HGB)、白细胞及血小板计数均正常。C反应蛋白(CRP)阴性。红细胞沉降率(ESR)53mm/h。抗核抗体(ANA)、抗双链DNA抗体、抗史密斯抗体、抗心磷脂抗体及人类白细胞抗原B51均在正常范围内。甲型流感病原体、乙型流感病原体、副流感病毒、肠道病毒及细小病毒的病原学检测均为阴性。血清抗GM1 IgG检测呈阳性。脑脊液白细胞计数正常,蛋白含量升高。脑脊液中水痘-带状疱疹病毒、巨细胞病毒及单纯疱疹病毒的革兰染色、培养及PCR检测均为阴性。脑脊液中与自身免疫性脑炎及副肿瘤综合征相关的抗体均为阴性。肌电图和神经传导研究显示严重的轴索性损害累及运动神经。脑部及海绵窦的磁共振成像未观察到明显异常。该患者被诊断为白塞病合并急性吉兰-巴雷综合征。他接受了静脉注射甲泼尼龙、静脉注射免疫球蛋白(IVIg)治疗、血浆置换及利妥昔单抗治疗。治疗后,患者四肢肌力恢复至1级,上睑下垂及疼痛消失。白塞病的神经系统受累相对少见,尤其是合并吉兰-巴雷综合征时,易导致误诊。白塞病合并急性吉兰-巴雷综合征的治疗包括原发性疾病的治疗、血浆置换及IVIg治疗。此外,支持治疗对此类患者非常重要。治疗重点是避免呼吸功能不全,预防深静脉血栓形成,监测心功能及血流动力学。止痛、体育锻炼及心理支持往往未得到充分重视。康复治疗对改善患者预后及生活质量非常重要。我们需要认识到,当神经系统的症状和体征难以仅用神经白塞病解释时,应警惕其他神经系统疾病的可能性。

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