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

被引：15

作者：

Farrar, Michelle A. ^{[1
,2
,5
]}

Lin, Cindy S. -Y. ^{[1
,3
]}

Krishnan, Arun V. ^{[1
,3
]}

Park, Susanna B. ^{[1
,4
]}

Andrews, Peter Ian ^{[2
,5
]}

Kiernan, Matthew C. ^{[1
,3
,4
]}

机构：

[1] Neurosci Res Australia, Sydney, NSW 2031, Australia

[2] Univ New S Wales, Sch Womens & Childrens Hlth, Sydney, NSW, Australia

[3] Univ New S Wales, Translat Neurosci Facil, Sch Med Sci, Sydney, NSW, Australia

[4] Univ New S Wales, Prince Wales Clin Hosp, Sydney, NSW, Australia

[5] Sydney Childrens Hosp, Dept Neurol, Sydney, NSW, Australia

来源：

PEDIATRICS | 2010年 / 126卷 / 03期

基金：

英国医学研究理事会;

关键词：

mitochondria; stroke; axon; excitability; MELAS; EXCITABILITY PROPERTIES; NERVE EXCITABILITY; MOTOR AXONS; MUTATION; NEUROPATHIES; DEPENDENCE; ISCHEMIA;

D O I：

10.1542/peds.2009-2930

中图分类号：

R72 [儿科学];

学科分类号：

100202 [儿科学];

摘要：

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

引用

页码：E734 / E739

页数：6

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