Idrissova Zh R, Boldyreva M N, Dekonenko E P, Malishev N A, Leontyeva I Y, Martinenko I N, Petrukhin A S
First Clinical Infections Hospital, Moscow, Russia.
Eur J Neurol. 2003 Sep;10(5):537-46. doi: 10.1046/j.1468-1331.2003.00639.x.
This study includes 90 children (41 female and 49 male) in the age range of 2-16 years with acute disseminated encephalomyelitis (ADEM). Thirty-three patients developed ADEM following rubella infection, 26 children following varicella infection, 20 suspected viral aetiology ADEM and 11 multiphasic disseminated encephalomyelitis (MDEM). All patients had neurological, routine laboratory and viral serology study with an enzyme-linked immunosorbent assay. Brain and/or spinal cord magnetic resonance imaging (MRI) were performed in 14 children. A follow-up study was in 1-5 years. Typing of DRB1 gene HLA class II was performed in 38 patients. We established that the varicella and rubella groups had preferential patterns. Rubella ADEM is characterized by acute explosive onset, seizures, coma and moderate pyramidal signs, whereas varicella infection is characterized by cerebella ataxia and mild pyramidal dysfunction. The suspected viral aetiology ADEM was characterized by polisymptomatic presentation. MDEM was characterized by older age of patients (11.6 +/- 2.8 years), more severe and prolonged local neurological symptoms, including myelitis symptoms and marked extrapyramidal signs, with distinct demyelination in MRIs. As a whole, ADEM is associated with DRB101 and DRB1017(03) in the Russian population. Thus, ADEM is a separate autoimmune condition with a specific mechanism due to the type of genetic immunoregulatory base and specificity of viral trigger.
本研究纳入了90名年龄在2至16岁之间患有急性播散性脑脊髓炎（ADEM）的儿童（41名女性和49名男性）。33例患者在风疹感染后发生ADEM，26例儿童在水痘感染后发病，20例疑似病毒病因的ADEM以及11例多相性播散性脑脊髓炎（MDEM）。所有患者均进行了神经学、常规实验室检查以及采用酶联免疫吸附测定法的病毒血清学研究。14名儿童接受了脑和/或脊髓磁共振成像（MRI）检查。随访研究为期1至5年。对38例患者进行了DRB1基因HLA II类分型。我们发现水痘组和风疹组具有优先模式。风疹相关性ADEM的特征为急性暴发性起病、癫痫发作、昏迷和中度锥体束征，而水痘感染则以小脑共济失调和轻度锥体束功能障碍为特征。疑似病毒病因的ADEM以多症状表现为特征。MDEM的特征为患者年龄较大（11.6±2.8岁）、局部神经症状更严重且持续时间更长，包括脊髓炎症状和明显的锥体外系体征，MRI显示有明显的脱髓鞘改变。总体而言，在俄罗斯人群中，ADEM与DRB101和DRB1017(03)相关。因此，由于遗传免疫调节基础的类型和病毒触发因素的特异性，ADEM是一种具有特定机制的独立自身免疫性疾病。
