Consensus-based statements for the management of mitochondrial stroke-like episodes
Open Access
- 13 December 2019
- journal article
- Published by F1000 Research Ltd in Wellcome Open Research
Abstract
Background: Focal-onset seizures and encephalopathy are prominent features of a stroke-like episode, which is a severe neurological manifestation associated with subtypes of mitochondrial disease. Despite more than 30 years of research, the acute treatment of stroke-like episodes remains controversial. Methods: We used the modified Delphi process to harness the clinical expertise of a group of mitochondrial disease specialists from five European countries to produce consensus guidance for the acute management of stroke-like episodes and commonly associated complications. Results: Consensus on a new definition of mitochondrial stroke-like episodes was achieved and enabled the group to develop diagnostic criteria based on clinical features, neuroimaging and/or electroencephalogram findings. Guidelines for the management of strokelike episodes were agreed with aggressive seizure management strongly recommended at the outset of stroke-like episodes. Conclusions: Our consensus statement defines stroke-like episodes in terms of an epileptic encephalopathy and we have used this to revise both diagnostic criteria and guidelines for management. A prospective, multi-centre, randomised controlled trial is required for evaluating the efficacy of any compound on modifying the trajectory of stroke-like episodes.Keywords
Funding Information
- Bundesministerium für Bildung und Forschung (01GM1603, 01GM1113A)
- Wellcome Trust (203105)
- National Institute for Health Research (CL-2016-01-003)
- Biotechnology and Biological Sciences Research Council (L016354)
- Sigrid Juselius Foundation
- Medical Research Council
- UK NHS Specialist Commissioners
This publication has 39 references indexed in Scilit:
- Mitochondrial Encephalomyopathy With Lactic Acidosis and Strokelike Episodes Presenting Before 50 Years of AgeJAMA Neurology, 2016
- Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) in the older adultPractical Neurology, 2014
- Localized cerebral energy failure in DNA polymerase gamma-associated encephalopathy syndromesBrain, 2010
- The G13513A Mutation in the ND5 Gene of Mitochondrial DNA as a Common Cause of MELAS or Leigh SyndromeArchives of Neurology, 2008
- MELAS ASSOCIATED WITH MUTATIONS IN THE POLG1 GENENeurology, 2007
- Mitochondrial Encephalomyopathy with Elderly Onset of Stroke-Like Episodes.Internal Medicine, 1996
- MELAS: An original case and clinical criteria for diagnosisNeuromuscular Disorders, 1992
- Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke‐like episodes (MELAS)Neurology, 1992
- A mutation in the tRNALeu(UUR) gene associated with the MELAS subgroup of mitochondrial encephalomyopathiesNature, 1990
- Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes: A distinctive clinical syndromeAnnals of Neurology, 1984