Perfusion status of the stroke-like lesion at the hyperacute stage in MELAS

Mitochondrial Encephalomyopathy Lactic Acidosis and Stroke-Like Episodes (MELAS): A Case Report and Critical Reappraisal of Treatment Options

“…21 Yeh et al reported hypoperfusion in a stroke-like episode using 99m Tc-HMPAO SPECT but then showed hyperperfusion using transcranial color-coded sonography. 22 …”

Increased cerebral blood flow as a predictor of episodes in MELAS using multimodal MRI

“…It predominantly involves gray matter and is not confined to vascular territories . According to previous research, hyperperfusion in acute encephalomyopathy represents an imaging landmark distinct from ischemia . Several important factors thought to contribute to hyperperfusion are vasodilation, breakdown of blood–brain barrier, and increased vascular permeability.…”

Brain uptake of Tc99m-HMPAO correlates with clinical response to the novel redox modulating agent EPI-743 in patients with mitochondrial disease

“…HMPAO SPECT imaging has also been applied to the study of mitochondrial disease showing marked decreases in regional and global uptake in the chronic setting though imaging performed during acute exacerbations is quite variable [32,33]. In contrast, our study demonstrates that the percent change in whole brain (MELAS) or cerebellar HMPAO uptake (Leigh syndrome, polymerase γ deficiency, Friedreich ataxia, Kearns-Sayre syndrome, Pearson syndrome, and mt-DNA depletion syndrome) had a highly significant linear relationship to the change in clinical status as assessed by the change in total NPMDS or NMDAS scores following three months of redox modulating therapy with EPI-743.…”