Eur J Paediatr Neurol. 2025 Oct 17;59:82-91. doi: 10.1016/j.ejpn.2025.10.007. Online ahead of print.
ABSTRACT
INTRODUCTION: The evolving spectrum of acute leukoencephalopathy with restricted diffusion (ALERD) in Indian children is distinct from that of their Japanese counterparts.
METHODS: This study enrolled children presenting with febrile infection-related epilepsy syndrome (FIRES) and radiological features of ALERD between May 2022 and April 2024. Intergroup comparisons were performed between the ‘diffuse’ versus ‘multifocal’ and the ‘symmetrical’ versus ‘asymmetrical’ diffusion restriction groups. Seizures recurring three months after discharge were designated as post-encephalopathic epilepsy (PEE). The outcome measures included the paediatric modified Rankin score (mRS) and development/intelligence quotients at the 12-month follow-up.
RESULTS: This study enrolled 38 children, whose mean (standard deviation) age was 6.77 (3.22) years. Diffuse and multifocal ALERD occurred in 29 % and 71 % of children, respectively; the lesions were symmetrical and asymmetrical among 37 % and 63 % of children, respectively. The mRS score was <2 in 18 % of children, whereas intellectual disabilities and PEE were noted among 50 % and 42 % of children, respectively. Age under six years was a significant predictor of diffuse and symmetrical ALERD. Pearson analysis revealed significant positive correlations of the diffusion-weighted imaging scores, prolonged persistence of diffusion restriction, and seizure duration exceeding 2 h with the mRS scores.
CONCLUSION: The children had a monophasic course and a higher frequency of multifocal and asymmetric ALERD. The nature of ALERD lesions did not influence the occurrence of PEE, although unilateral lesions had better outcomes. Children with a radiographic signature of ALERD constitute a unique subset of FIRES, with distinct therapeutic responses and long-term outcomes.
PMID:41129842 | DOI:10.1016/j.ejpn.2025.10.007
