A group of international experts has drawn up recommendations to standardise the diagnosis and management of MELAS myopathy and associated stroke-like episodes (SLEs). Consensus was reached on several statements (using the Delphi method), notably:
- this myopathy is defined by the occurrence of at least one SLE, associated with mitochondrial dysfunction caused by a mutation in mitochondrial DNA. The use of imprecise terminology such as ‘MELAS spectrum’ or ‘MELAS-like’ is discouraged;
- the efficacy of supplementation with L-arginine, L-citrulline, L-taurine, coenzyme Q10, vitamins and other dietary supplements has not yet been demonstrated, either for acute treatment or as prophylaxis, which highlights the need for future randomised controlled trials,
- in SLE, antiepileptic drugs should be initiated as soon as epileptic seizures are suspected (even without EEG confirmation) and intravenous corticosteroids may be considered on a case-by-case basis. However, stroke treatments (fibrinolysis, antiplatelet agents) are not indicated;
- multidisciplinary management of neurological, neuropsychiatric and systemic complications has been recommended.
This international consensus addresses the lack of recommendations but highlights the gaps in knowledge that still need to be filled.
Voir aussi « MELAS : vers un diagnostic et une prise en charge harmonisés »
