Acute, Reversible Axonal Energy Failure During Stroke-Like Episodes in MELAS

作者：Farrar Michelle A; Lin Cindy S Y; Krishnan Arun V; Park Susanna B; Andrews Peter Ian; Kiernan Matthew C^*

来源：Pediatrics, 2010, 126(3): E734-E739.

DOI：10.1542/peds.2009-2930

• mitochondria
• stroke
• axon
• excitability
• MELAS

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. Pediatrics 2010;126:e734-e739

• 出版日期2010-9