title: Acute, reversible axonal energy failure during stroke-like episodes in MELAS.
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.
authors.affiliation: Neuroscience Research Australia, Barker Street, Randwick, Sydney, New South Wales 2031, Australia.
authors.forename: Michelle A
authors.initials: MA
authors.lastname: Farrar
authors.forename: Cindy S-Y
authors.initials: CS
authors.lastname: Lin
authors.forename: Arun V
authors.initials: AV
authors.lastname: Krishnan
authors.forename: Susanna B
authors.initials: SB
authors.lastname: Park
authors.forename: Peter Ian
authors.initials: PI
authors.lastname: Andrews
authors.forename: Matthew C
authors.initials: MC
authors.lastname: Kiernan
mesh_terms: D000200:Action Potentials; D001369:Axons / Q000502:physiology*; D002648:Child; D005260:Female; D006801:Humans; D017241:MELAS Syndrome / Q000503:physiopathology*