Consensus-based statements for the management of mitochondrial stroke-like episodes.

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Bindoff, Laurence A 
Gorman, Gráinne S 
Horvath, Rita 
Klopstock, Thomas 

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.

MELAS, POLG, antiepileptic drugs, encephalopathies, epilepsy, m.3243A>G, neurodegenerative disorders (other than dementia), status epilepticus, stroke
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Wellcome Open Res
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F1000 Research Ltd
Wellcome Trust (109915/A/15/Z)
Medical Research Council (MR/N025431/2)