Cerebral metabolism of oxygen and glucose in a patient with MELAS syndrome
Corresponding Author
M. Sano
Department of Neurology, Showa General Hospital
Motoki Sano, Department of Neurology, Showa General Hospital, 2-450 Tenjin, Kodaira, Tokyo 187, JapanSearch for more papers by this authorK. Ishii
Positron Medical Center, Tokyo Metropolitan Institute of Gerontology, Japan
Search for more papers by this authorM. Senda
Positron Medical Center, Tokyo Metropolitan Institute of Gerontology, Japan
Search for more papers by this authorCorresponding Author
M. Sano
Department of Neurology, Showa General Hospital
Motoki Sano, Department of Neurology, Showa General Hospital, 2-450 Tenjin, Kodaira, Tokyo 187, JapanSearch for more papers by this authorK. Ishii
Positron Medical Center, Tokyo Metropolitan Institute of Gerontology, Japan
Search for more papers by this authorM. Senda
Positron Medical Center, Tokyo Metropolitan Institute of Gerontology, Japan
Search for more papers by this authorAbstract
We studied cerebral oxygen and glucose metabolism as well as cerebral blood flow using positron emission tomography (PET) in a case with MELAS showing dementia, diabetes mellitus, ataxia and lactic acidosis without any signs of stroke. This case, confirmed to have a point mutation at position 3243 in the transfer RNA gene of mitochondrial DNA, developed a stroke-like episode 8 months after the PET study. Uncoupling was observed between cerebral oxygen metabolism and cerebral blood flow with reduced fractional oxygen extraction ratio, indicating “hyperemia”, not ischemia. The “hyperemia” may be closely related to the malfunction of mitochondria in aerobic energy production. A drastic decrease in cerebral oxygen metabolism (CMR2) was found globally in contrast to preserved cerebral glucose metabolism (CMRglu), resulting in a remarkable decrease in the metabolic ratio (CMRO2/CMRglu). The dissociation between cerebral glucose and oxygen metabolism may be characteristic of MELAS.
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