{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,12]],"date-time":"2025-12-12T13:50:09Z","timestamp":1765547409909,"version":"3.44.0"},"reference-count":15,"publisher":"BMJ","issue":"4","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2025,3,21]]},"published-print":{"date-parts":[[2025,4]]},"abstract":"<jats:p>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.<\/jats:p>","DOI":"10.1136\/bcr-2024-264278","type":"journal-article","created":{"date-parts":[[2025,4,5]],"date-time":"2025-04-05T09:56:53Z","timestamp":1743847013000},"page":"e264278","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":1,"title":["ALERD: acute leukoencephalopathy with restricted diffusion\u2014a newly emerging diagnosis"],"prefix":"10.1136","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0009-0002-9953-5576","authenticated-orcid":false,"given":"Abida","family":"Akbar","sequence":"first","affiliation":[{"name":"Pediatrics and Child Health, The Aga Khan University Hospital, Karachi, Pakistan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3184-4241","authenticated-orcid":false,"given":"Sanam Bano","family":"Rajper","sequence":"additional","affiliation":[{"name":"Pediatrics and Child Health, The Aga Khan University Hospital, Karachi, Pakistan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0001-1764-5652","authenticated-orcid":false,"given":"Sadam","family":"Hussain","sequence":"additional","affiliation":[{"name":"Pediatrics and Child Health, The Aga Khan University Hospital, Karachi, Pakistan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-9794-1107","authenticated-orcid":false,"given":"Khairunnisa","family":"Mukhtiar","sequence":"additional","affiliation":[{"name":"Pediatrics and Child Health, The Aga Khan University Hospital, Karachi, Pakistan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2025,4,5]]},"reference":[{"key":"2025082700370545000_18.4.e264278.1","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1016\/j.ejpn.2021.05.017","article-title":"The spectrum of acute leukoencephalopathy with restricted diffusion (ALERD): A case series and review of literature","volume":"33","author":"Lawrence","year":"2021","journal-title":"Eur J Paediatr Neurol"},{"key":"2025082700370545000_18.4.e264278.2","doi-asserted-by":"crossref","first-page":"20","DOI":"10.25259\/KPJ_59_2023","article-title":"Acute leukoencephalopathy with restricted diffusion \u2013 Case Series","volume":"39","author":"Jyothi","year":"2024","journal-title":"KPJ"},{"key":"2025082700370545000_18.4.e264278.3","doi-asserted-by":"publisher","DOI":"10.4103\/ijccm.IJCCM_139_18"},{"key":"2025082700370545000_18.4.e264278.4","first-page":"149","article-title":"Acute leucoencephalopathy with restriction of diffusion-a case report","volume":"17","author":"Singh","year":"2012","journal-title":"East J Med"},{"key":"2025082700370545000_18.4.e264278.5","doi-asserted-by":"crossref","first-page":"9134","DOI":"10.1038\/s41598-020-66167-7","article-title":"Determinants of outcomes for acute encephalopathy with reduced subcortical diffusion","volume":"10","author":"Sakata","year":"2020","journal-title":"Sci Rep"},{"key":"2025082700370545000_18.4.e264278.6","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0404.2007.00809.x"},{"key":"2025082700370545000_18.4.e264278.7","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1016\/j.braindev.2009.02.012","article-title":"Two newly proposed infectious encephalitis\/encephalopathy syndromes","volume":"31","author":"Takanashi","year":"2009","journal-title":"Brain and Development"},{"key":"2025082700370545000_18.4.e264278.8","doi-asserted-by":"publisher","DOI":"10.3174\/ajnr.A1247"},{"key":"2025082700370545000_18.4.e264278.9","doi-asserted-by":"publisher","DOI":"10.3174\/ajnr.A1431"},{"key":"2025082700370545000_18.4.e264278.10","doi-asserted-by":"crossref","unstructured":"Jayaramu S , Doddamani D . Radiological Insights into Acute Leukoencephalopathy with Restricted Diffusion (ALERD): A Case Series Analysis. Indian J Radiol Imaging 2024. doi:10.1055\/s-0044-1791231","DOI":"10.1055\/s-0044-1791231"},{"key":"2025082700370545000_18.4.e264278.11","doi-asserted-by":"crossref","DOI":"10.3389\/fneur.2021.730535","article-title":"Predicting the Onset of Acute Encephalopathy With Biphasic Seizures and Late Reduced Diffusion by Using Early Laboratory Data","volume":"12","author":"Maeda","year":"2021","journal-title":"Front Neurol"},{"key":"2025082700370545000_18.4.e264278.12","doi-asserted-by":"crossref","first-page":"862","DOI":"10.1016\/j.braindev.2019.07.008","article-title":"Thermolabile polymorphism of carnitine palmitoyltransferase 2: A genetic risk factor of overall acute encephalopathy","volume":"41","author":"Shibata","year":"2019","journal-title":"Brain Dev"},{"key":"2025082700370545000_18.4.e264278.13","doi-asserted-by":"crossref","first-page":"1286","DOI":"10.1111\/epi.13068","article-title":"Seizure characteristics of epilepsy in childhood after acute encephalopathy with biphasic seizures and late reduced diffusion","volume":"56","author":"Ito","year":"2015","journal-title":"Epilepsia"},{"key":"2025082700370545000_18.4.e264278.14","doi-asserted-by":"crossref","first-page":"2475","DOI":"10.1007\/s10072-023-06686-z","article-title":"Clinical and radiological spectrum of anti-myelin oligodendrocyte glycoprotein (MOG) antibody encephalitis: single-center observational study","volume":"44","author":"Salunkhe","year":"2023","journal-title":"Neurol Sci"},{"key":"2025082700370545000_18.4.e264278.15","doi-asserted-by":"crossref","unstructured":"Baby N , Ajith S , Mohammed L , et al . Be Alert to ALERD: Acute Leukoencephalopathy with Restricted Diffusion\u2014Atypical Presentation of a Rare Case. 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