Discordance Between Cerebral Oxygen and Glucose Metabolism, and Hemodynamics in a Mitochondrial Encephalomyopathy, Lactic Acidosis, and Strokelike Episode Patient

A patient with mitochondrial encephalomyopathy, lactic acidosis, and strokelike episode (MELAS) syndrome underwent serial measurement of cerebral blood flow with xenon computed tomography (Xe-CBF) while presenting with strokelike episodes accompanied by a cerebral lesion. He underwent positron emission tomography (PET) measurement of the regional cerebral blood flow (PET-CBF), metabolic rate of oxygen (CMRO2), and glucose (CMRGlu) after his symptoms and lesion disappeared. During the symptomatic period, Xe-CBF and the Xe-CBF response to acetazolamide loading were well preserved both in and outside the low-density lesion. In the PET study, decreased CMRO2 and increased PET-CBF and CMRGlu were noted in the entire brain. The strokelike episodes of patients with MELAS are more likely attributed to the failure of oxygen metabolism than to a vascular accident.