Muscle glycogen depletion and increased oxidative phosphorylation following status epilepticus.
We describe complete glycogen depletion and increased respiratory chain enzyme activity in a muscle biopsy obtained prior to the demise of a patient in multiorgan failure following status epilepticus. These findings validate the theoretical basis of muscle energy turnover during status epilepticus: the increased demand for energy leads to complete depletion of glycogen reserves. The attempt to preserve adenosine triphosphate requirements results in increased activity of respiratory chain enzymes.
Michelson-Kerman
Marina
M
Mitochondrial Disease Clinic, Wolfson Medical Center, Holon, Sackler School of Medicine, Tel-Aviv University, Israel.
Watemberg
Nathan
N
Nissenkorn
Andreea
A
Gilad
Eli
E
Sadeh
Menachem
M
Lerman-Sagie
Tally
T
D001706:Biopsy; D002648:Child; D004579:Electron Transport / Q000502:physiology; D004734:Energy Metabolism / Q000502:physiology; D004831:Epilepsies, Myoclonic / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D006003:Glycogen / Q000378:metabolism*; D006801:Humans; D009102:Multiple Organ Failure / Q000473:pathology; D018482:Muscle, Skeletal / Q000473:pathology*; D013226:Status Epilepticus / Q000473:pathology*
D006003:Glycogen