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  • Title: Acute, reversible axonal energy failure during stroke-like episodes in MELAS.
    Author: Farrar MA, Lin CS, Krishnan AV, Park SB, Andrews PI, Kiernan MC.
    Journal: Pediatrics; 2010 Sep; 126(3):e734-9. PubMed ID: 20679297.
    Abstract:
    The pathophysiology of stroke-like episodes in MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) remains unresolved. Possible mechanisms include mitochondrial angiopathy, cytopathy, or both, collectively resulting in cellular energy depletion. To clarify disease mechanisms, axonal excitability properties were investigated in a 10-year-old child with MELAS. Serial assessments during a stroke-like episode revealed reversible depolarization of the axonal membrane consistent with disruption of energy-dependent processes. Axonal parameters correlated with the clinical assessment of central dysfunction and biochemical measures of acidosis. Novel axonal excitability techniques have established acute, reversible ischemic-like depolarization that may serve as a surrogate marker of central events that develop during stroke-like episodes in MELAS.
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