Acute, reversible axonal energy failure during stroke-like episodes in MELAS

Pediatrics

Volumn 126, Issue 3, 2010, Pages

  Farrar, Michelle A ^a,b,d   Lin, Cindy S Y ^a,b   Krishnan, Arun V ^a,b   Park, Susanna B ^a,c   Andrews, Peter Ian ^b,d   Kiernan, Matthew C ^a,b,c  

^a NEUROSCIENCE RESEARCH AUSTRALIA   (Australia)

^b UNIVERSITY OF NEW SOUTH WALES   (Australia)

^c UNIVERSITY OF NEW SOUTH WALES   (Australia)

^d SYDNEY CHILDREN'S HOSPITAL   (Australia)

Author keywords

Axon; Excitability; MELAS; Mitochondria; Stroke

Indexed keywords

ALPHA TOCOPHEROL; ARGININE; ASCORBIC ACID; BENZODIAZEPINE DERIVATIVE; GLUCOSE; LACTIC ACID; LEUCINE; LIPID EMULSION; RIBOFLAVIN; RNA; UBIQUINONE;

ABDOMINAL PAIN; ACUTE BRAIN DISEASE; ARTICLE; BRAIN DISEASE; CASE REPORT; CELL MEMBRANE DEPOLARIZATION; CEREBRAL BLINDNESS; CHILD; COGNITIVE DEFECT; CONFUSION; CONTINUOUS INFUSION; DROWSINESS; ELECTROENCEPHALOGRAM; EPILEPTIC STATE; FATIGUE; FEMALE; FOCAL EPILEPSY; HEADACHE; HOMONYMOUS HEMIANOPIA; HUMAN; LACTATE BLOOD LEVEL; LACTIC ACIDOSIS; MEDIAN NERVE; MELAS SYNDROME; MITOCHONDRIAL GENOME; MOTOR NERVE CONDUCTION; MUSCLE ACTION POTENTIAL; MUSCLE STRENGTH; MYOPATHY; NERVE CONDUCTION; NERVE EXCITABILITY; NERVE FIBER MEMBRANE; PERCEPTION DEAFNESS; PH; POINT MUTATION; PRIORITY JOURNAL; PROSPECTIVE PAYMENT; PTOSIS; RECURRENT DISEASE; REFRACTORY PERIOD; SCHOOL CHILD; SEIZURE; SENSORY NERVE CONDUCTION; SHORT STATURE; STIMULUS; STROKE; SURAL NERVE; TIBIAL NERVE; VOMITING; WEAKNESS; ACTION POTENTIAL; NERVE FIBER; PATHOPHYSIOLOGY; PHYSIOLOGY;

ACTION POTENTIALS; AXONS; CHILD; FEMALE; HUMANS; MELAS SYNDROME;

EID: 77956277009     PISSN: 00314005     EISSN: 10984275     Source Type: Journal    
DOI: 10.1542/peds.2009-2930     Document Type: Article

References (16)

1. Kaufmann P, Pascual JM, Anziska Y, et al. Nerve conduction abnormalities in patients with MELAS and the A3243G mutation. Arch Neurol. 2006;63(5):746-748
2. Koga Y. L-Arginine therapy on MELAS [in Japanese]. Rinsho Shinkeigaku. 2008;48(11):1010-1012
3. Krishnan AV, Lin CS, Park SB, Kiernan MC. Assessment of nerve excitability in toxic and metabolic neuropathies. J Peripher Nerv Syst. 2008;13(1):7-26
4. Vucic S, Kiernan MC. Axonal excitability properties in amyotrophic lateral sclerosis. Clin Neurophysiol. 2006;117(7):1458-1466 (Pubitemid 43947716)
5. Krishnan AV, Bostock H, Ip J, Hayes M, Watson S, Kiernan MC. Axonal function in a family with episodic ataxia type 2 due to a novel mutation. J Neurol. 2008;255(5):750-755
6. Kiernan MC, Krishnan A, Lin C, Burke D, Berkovic SF. Mutation in the Na+ channel subunit SCN1B produces paradoxical changes in peripheral nerve excitability. Brain. 2005;128(pt 8):1841-1846
7. Kiernan MC, Burke D, Andersen KV, Bostock H. Multiple measures of axonal excitability: a new approach in clinical testing. Muscle Nerve. 2000;23(3):399-409
8. Kiernan MC, Bostock H. Effects of membrane polarization and ischaemia on the excitability properties of human motor axons. Brain. 2000;123(pt 12):2542-2551
9. Kaji R. Physiology of conduction block in multifocal motor neuropathy and other demyelinating neuropathies. Muscle Nerve. 2003;27(3):285-296 (Pubitemid 36255388)
10. Rakowski RF, Gadsby DC, De Weer P. Voltage dependence of the Na/K pump. J Membr Biol. 1997;155(2):105-112
11. Kaji R, Sumner AJ. Ouabain reverses conduction disturbances in single demyelinated nerve fibers. Neurology. 1989;39(10):1364-1368
12. Baker MD, Bostock H. The pH dependence of late sodium current in large sensory neurons. Neuroscience. 1999;92(3):1119-1130 (Pubitemid 29287555)
13. Mogyoros I, Kiernan MC, Burke D, Bostock H. Excitability changes in human sensory and motor axons during hyperventilation and ischaemia. Brain. 1997;120(pt 2):317-325
14. Alvarez S, Moldovan M, Krarup C. Acute energy restriction triggers Wallerian degeneration in mouse. Exp Neurol. 2008;212(1):166-178
15. Iizuka T, Sakai F, Kan S, Suzuki N. Slowly progressive spread of the stroke-like lesions in MELAS. Neurology. 2003;61(9):1238-1244
16. James AM, Sheard PW, Wei YH, Murphy MP. Decreased ATP synthesis is phenotypically expressed during increased energy demand in fibroblasts containing mitochondrial transfer RNA mutations. Eur J Biochem. 1999;259(1):-2):462-469