Pagano Miguel A, Allievi Alberto, Muñoz Sebastián A, Marroquín Verónica, Biaggioni Milton, Vallejos Lucas F
Unidad de Neurología, Hospital General de Agudos Juan A. Fernández, Buenos Aires, Argentina.
Medicina (B Aires). 2011;71(3):254-6.
Acute disseminated encephalomyelitis (ADEM) and Guillain-Barré Syndrome (GBS) are commonly recognized as separated entities involving different parts of the nervous system. However, they share some features such as: autoimmune pathogenesis, myelin injury and previous history of viral infections or vaccination. We report the case of a 41 year-old man who developed fever, lower limbs weakness and obtundation fifteen days after an acute gastroenteritis. Neurological examination showed patellar hypereflexia, bilateral Babinski and neurogenic bladder. Twenty-four hours later he developed flaccid paraparesis, generalized areflexia and respiratory failure that was supported by mechanical ventilation. Cerebrospinal fluid showed mononuclear pleocytosis and elevated proteins. Electrodiagnosis showed important reduction of conduction velocity on both peroneal nerves. Magnetic Resonance Imaging revealed white matter lesions in brain, pons and thoracic levels of the spinal cord. Diagnosis of the association between ADEM and GBS (ASADEM-GBS) was made and treatment with corticosteroids and intravenous immunoglobulin was started. The patient recovered motor, sensory and bladder functions and he was able to walk six months later. ASADEM-GBS is an uncommon entity generally considered of poor outcome; however a rapid diagnosis and treatment can substantially improve the prognosis.
急性播散性脑脊髓炎(ADEM)和吉兰-巴雷综合征(GBS)通常被认为是涉及神经系统不同部位的独立疾病实体。然而,它们具有一些共同特征,如自身免疫发病机制、髓鞘损伤以及既往病毒感染或疫苗接种史。我们报告一例41岁男性病例,该患者在急性胃肠炎后15天出现发热、下肢无力和意识障碍。神经系统检查显示髌阵挛亢进、双侧巴宾斯基征阳性及神经源性膀胱。24小时后,他出现弛缓性截瘫、全身反射消失及呼吸衰竭,需机械通气支持。脑脊液检查显示单核细胞增多及蛋白升高。电诊断显示双侧腓总神经传导速度显著降低。磁共振成像显示脑、脑桥和脊髓胸段水平有白质病变。诊断为ADEM与GBS合并(ADEM-GBS),并开始使用皮质类固醇和静脉注射免疫球蛋白治疗。患者运动、感觉和膀胱功能恢复,6个月后能够行走。ADEM-GBS是一种罕见疾病,通常认为预后较差;然而,快速诊断和治疗可显著改善预后。