Clinical and radiologic reversal of stroke-like episodes in MELAS with high-dose <scp>l</scp> -arginine

Abstract

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome ( MT-TL1 gene) is a progressive neurologic disorder with stroke-like episodes (SLEs), which are recurrent neurologic deficits resembling vasoocclusive strokes.1 However, SLEs are not restricted to vascular territories and have a predilection for the occipital and posterior parietal and temporal cortices,2 may evolve subacutely over hours to days,3 and have greater potential for reversibility.4 Their pathophysiology is incompletely understood. Current literature suggests a combination of neuronal mitochondrial energy failure and cerebrovascular angiopathy with dysregulated perfusion.5