Singhi Pratibha D, Ray Munni, Singhi Sunit, Kumar Khandelwal Narendra
Department of Pediatrics, PGIMER, Chandigarh, India.
J Child Neurol. 2006 Oct;21(10):851-7. doi: 10.1177/08830738060210100201.
Acute disseminated encephalomyelitis in children is not uncommon in developing countries, yet there is little systematic documentation of its clinical profile and follow-up. We studied the clinical and neuroradiologic features of acute disseminated encephalomyelitis in 52 consecutive children. Clinical details, magnetic resonance imaging (MRI) findings, and the results of other investigations were recorded, and children were followed up from 6 to 48 months. A repeat MRI was done after 3 to 4 months, and in those with persistent lesions, another MRI was done after 6 to 7 months of discharge. The mean age at presentation was 6.14 +/- 3.17 years, 73.1% were male, and 17 children had a history of antecedent infectious illness or vaccination. Most children had a meningoencephalitic presentation, with sudden-onset motor weakness in 76.9% and seizures in 36.5%. Altered sensorium and pyramidal signs were seen in 55.8% and 80.7% of children, respectively. On MRI, scattered T(2)-weighted hyperintense lesions were seen, mainly in the subcortical white matter, especially in the parietal (53.8%) and frontal (30.17%) regions. Thalamic, basal ganglia, and callosal lesions were seen in 30.76%, 17.3%, and 13.46% of cases, respectively. Variable contrast enhancement was seen in 48% of those who had contrast MRIs. The response to methylprednisolone was good, with dramatic recovery in 26.9% and marked improvement in 51.9% at discharge. On follow-up, of 44 children, residual smaller MRI lesions were seen in 30. The MRI was repeated at 6 months in children with residual lesions, and it was found that the lesions either disappeared or were significantly reduced after 6 months in 75% of cases. Four children had relapse of acute disseminated encephalomyelitis with new lesions on MRI. All of them responded to methylprednisolone. None of the clinical or neuroradiologic factors at presentation had any significant correlation with relapse. Six months after discharge, no deficits could be found in 61.3% of cases; 15.9% and 4.5% had motor and cognitive deficits, and 9% had multiple deficits. The presentation of pediatric acute disseminated encephalomyelitis in developing countries is similar to that in developed countries. In spite of an aggressive presentation, most children respond well to corticosteroids. MRI lesions disappear or are significantly reduced at 6 months in the majority of cases.
急性播散性脑脊髓炎在发展中国家的儿童中并不少见，但关于其临床特征和随访情况的系统记录却很少。我们对52例连续的儿童急性播散性脑脊髓炎患者的临床和神经放射学特征进行了研究。记录了临床细节、磁共振成像（MRI）结果及其他检查结果，并对患儿进行了6至48个月的随访。在3至4个月后进行了重复MRI检查，对于病变持续存在的患儿，在出院后6至7个月又进行了一次MRI检查。发病时的平均年龄为6.14±3.17岁，73.1%为男性，17名儿童有前驱感染性疾病或疫苗接种史。大多数儿童表现为脑膜脑炎，76.9%的患儿突发运动无力，36.5%的患儿有癫痫发作。分别有55.8%和80.7%的患儿出现意识改变和锥体束征。MRI检查可见散在的T2加权高信号病变，主要位于皮质下白质，尤其是顶叶（53.8%）和额叶（30.17%）区域。分别有30.76%、17.3%和13.46%的病例出现丘脑、基底节和胼胝体病变。在进行增强MRI检查的患儿中，48%可见不同程度的强化。患儿对甲泼尼龙反应良好，出院时26.9%显著恢复，51.9%明显改善。随访中，44名患儿中有30名残留较小的MRI病变。对有残留病变的患儿在6个月时再次进行MRI检查，发现75%的病例在6个月后病变消失或明显缩小。4名儿童急性播散性脑脊髓炎复发，MRI出现新病变。他们均对甲泼尼龙有反应。发病时的临床或神经放射学因素与复发均无显著相关性。出院6个月后，61.3%的病例无功能缺损；15.9%和4.5%的患儿有运动和认知功能缺损，9%的患儿有多种功能缺损。发展中国家儿童急性播散性脑脊髓炎的表现与发达国家相似。尽管起病急，但大多数儿童对皮质类固醇反应良好。大多数病例在6个月时MRI病变消失或明显缩小。
