Kamate Mahesh, Detroja Mayank, Hattiholi Virupaxi
Professor of Pediatrics and In.Charge Child Development Centre, KAHER University's J N Medical College, Belgaum, Karnataka, India.
Senior resident, Child Development Centre, KLE Prabhakar Kore Hospital, Belgaum, Karnataka, India.
Neurol India. 2021 Mar-Apr;69(2):466-469. doi: 10.4103/0028-3886.314577.
To study the clinico-radiological profile of children with acute leukoencephalopathy with restricted diffusion.
A retrospective chart review of children with acute leukoencephalopathy with restricted diffusion was done from July 2015 to July 2018. The clinical details, neuroimaging findings, sequelae, and the final outcome on modified Rankin Score were analyzed.
Sixteen children with a mean age of 4.4 years were diagnosed with acute leukoencephalopathy with restricted diffusion. All, except one, had fever, seizure, and altered sensorium. The median duration of hospital stay was 3 weeks. Only one out of 16, had biphasic clinical picture that is characteristic of acute encephalopathy with biphasic seizures and restricted diffusion. Magnetic resonance imaging showed restriction diffusion in all. While it was symmetric in 13 children, in 3 children it was asymmetric, and in 2 children there was patchy involvement. Seven children (43.7%) had post-encephalopathic epilepsy. While complete neurological recovery was seen in 2 children, behavioral problems like hyperactivity in 10 (62.5%), speech problems in 8 (50%), and cognitive delay in 3 (18.8%) children were noted.
Acute leukoencephalopathy with restricted diffusion is emerging as an important cause of acute encephalopathy in children with a protracted course and long-term sequelae such as cognitive impairment and refractory postencephalopathic epilepsy.
研究具有弥散受限的儿童急性白质脑病的临床放射学特征。
对2015年7月至2018年7月期间患有弥散受限的儿童急性白质脑病的病例进行回顾性图表审查。分析临床细节、神经影像学表现、后遗症以及改良Rankin评分的最终结果。
16名平均年龄为4.4岁的儿童被诊断为具有弥散受限的急性白质脑病。除1名儿童外，所有儿童均有发热、癫痫发作和意识改变。住院时间中位数为3周。16名儿童中只有1名具有双相临床表现，这是急性脑病伴双相癫痫发作和弥散受限的特征。磁共振成像显示所有患儿均有弥散受限。其中13名儿童的表现为对称，3名儿童不对称，2名儿童有斑片状受累。7名儿童（43.7%）患有脑病后癫痫。2名儿童实现了完全神经功能恢复，10名儿童（62.5%）出现了如多动等行为问题，8名儿童（50%）有言语问题，3名儿童（18.8%）有认知延迟。
具有弥散受限的急性白质脑病正逐渐成为儿童急性脑病的一个重要病因，其病程迁延且有认知障碍和难治性脑病后癫痫等长期后遗症。
