Akbar Abida, Rajper Sanam Bano, Hussain Sadam, Mukhtiar Khairunnisa
Pediatrics and Child Health, The Aga Khan University Hospital, Karachi, Pakistan.
Pediatrics and Child Health, The Aga Khan University Hospital, Karachi, Pakistan
BMJ Case Rep. 2025 Apr 5;18(4):e264278. doi: 10.1136/bcr-2024-264278.
Acute leukoencephalopathy with restricted diffusion (ALERD) is a distinct, severe clinical-radiological syndrome characterised by encephalopathy, seizures and restricted diffusion in the subcortical white matter. ALERD is one of the infection-associated encephalopathy syndromes seen in childhood having a higher incidence of neurological sequelae. We report three cases of ALERD, all following a prodrome of fever and gastroenteritis, progressing to seizures and encephalopathy with varying outcomes. Case 1 was diagnosed as complicated dengue fever with multiple organ dysfunction syndrome (MODS), developed diffuse ALERD but improved with immunotherapy and had mild neurologic sequelae. Case 2 presented earlier, had normal initial MRI, developed diffuse ALERD later and had significant neurologic sequelae. Case 3, despite the central-sparing pattern, had rapid progression, MODS and severe cardiac dysfunction, and ultimately succumbed to death due to aspiration. These cases underscore ALERD as a significant cause of acute encephalopathy, requiring a high index of suspicion and prompt initiation of immunotherapy for better outcomes.
