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Acute Leucoencephalopathy with Restricted Diffusion in Children – A case series

Objective: To study the clinico-radiological profile of children with acute leukoencephalopathy with restricted diffusion. Methods: 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. Results: 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. Conclusion: 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.