International consensus definitions for infection-triggered encephalopathy syndromes

Sakuma H 1 , Thomas T 2 , Debinski C 3 , Eyre M 4 , Han VX 5 , Jones HF 6 Show all authors , Kawano G 7 , Lee VW 8 , Malone S 9 , Matsuishi T 7 , Mohammad SS 10 , Mori T 1 , Nishida H 1 , Nosadini M 11 , Takanashi JI 12 , Mizuguchi M 13 , Lim M 8 , Dale RC 14

Affiliations 

  • 1 Department of Brain & Neurosciences, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan
  • 2 Department of Paediatrics, Neurology Service, KK Women's and Children's Hospital, Singapore
  • 3 Monash Children's Hospital, Melbourne, VIC, Australia
  • 4 School of Biomedical Engineering and Imaging Sciences, King's College London, London, UK
  • 5 Khoo Teck Puat-National University Children's Medical Institute, National University Health System, Singapore
  • 6 Department of Neuroservices, Starship Children's Hospital, Auckland, New Zealand
  • 7 Department of Paediatrics, St Mary's Hospital, Fukuoka, Japan
  • 8 Children's Neurosciences, Evelina London Children's Hospital, London, UK
  • 9 Neuroscience Department, Queensland Children's Hospital, South Brisbane, QLD, Australia
  • 10 Kids Neuroscience Centre and Brain and Mind Centre, Faculty of Medicine and Health, University of Sydney, Westmead, NSW, Australia
  • 11 Paediatric Neurology and Neurophysiology Unit, Department of Women's and Children's Health, University Hospital of Padova, Padova, Italy
  • 12 Department of Pediatrics and Pediatric Neurology, Tokyo Women's Medical University Yachiyo Medical Center, Chiba, Japan
  • 13 Department of Developmental Medical Sciences, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
  • 14 Kids Neuroscience Centre, The Children's Hospital at Westmead, Faculty of Medicine and Health, University of Sydney, Westmead, NSW, Australia
Dev Med Child Neurol, 2024 Aug 14.
PMID: 39143740 DOI: 10.1111/dmcn.16067

Abstract

AIM: To develop standardized diagnostic criteria for 'infection-triggered encephalopathy syndrome (ITES)' and five specific clinical syndromes of ITES.

METHOD: The draft definitions were based on existing criteria, standardized, and discussed by a panel of international experts using nominal group technique over 18 months to achieve consensus. All criteria use the same format: (1) presence of infection/fever; (2) clinical features including encephalopathy; (3) neuroradiological features on magnetic resonance imaging; (4) exclusion of other causes.

RESULTS: We first highlighted differences between ITES and infectious and autoimmune encephalitis, which is the most important differential diagnosis. Consensus was achieved to define five specific ITESs: acute encephalopathy with biphasic seizures and late reduced diffusion; acute necrotizing encephalopathy; mild encephalopathy with a reversible splenial lesion; acute fulminant cerebral oedema; and acute shock with encephalopathy and multiorgan failure. Two further conditions that are currently classified as epilepsy syndromes but have similar features to ITES, namely febrile infection-related epilepsy syndrome and hemiconvulsion-hemiplegia-epilepsy syndrome, are also discussed.

INTERPRETATION: The consensus definition is expected to improve awareness of this disease concept, provide diagnostic framework, and facilitate future international research and clinical trials.

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

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