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Consensus-based statements for the management of mitochondrial stroke-like episodes [version 1; peer review: 2 approved]

Ng, YS; Bindoff, LA; Gorman, GS; Horvath, R; Klopstock, T; Mancuso, M; Martikainen, MH; ... Turnbull, DM; + view all (2019) Consensus-based statements for the management of mitochondrial stroke-like episodes [version 1; peer review: 2 approved]. Wellcome Open Research , 4 , Article 201. 10.12688/wellcomeopenres.15599.1. Green open access

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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.

Type: Article
Title: Consensus-based statements for the management of mitochondrial stroke-like episodes [version 1; peer review: 2 approved]
Open access status: An open access version is available from UCL Discovery
DOI: 10.12688/wellcomeopenres.15599.1
Publisher version: https://doi.org/10.12688/wellcomeopenres.15599.1
Language: English
Additional information: © 2019 Ng YS et al. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/).
Keywords: MELAS, m.3243A>G, POLG, epilepsy, status epilepticus, encephalopathies, stroke, neurodegenerative disorders (other than dementia), antiepileptic drugs
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Department of Neuromuscular Diseases
URI: https://discovery.ucl.ac.uk/id/eprint/10088813
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