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Journal of Child Neurology
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Cerebral Blood Flow and Oxygen Metabolism in Rett Syndrome

Hideto Yoshikawa, MD

Division of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders

Noboru Fueki, MD

Division of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders

Hisaharu Suzuki, MD

Division of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders

Norio Sakuragawa, MD

Division of Inherited Metabolic Disorders, National Institute of Neuroscience

Iio Masaaki, MD

National Center of Neurology and Psychiatry, and the Department of Radiology, National Nakano Chest Hospital, Tokyo

Positron emission tomography was performed on six patients with Rett syndrome to investigate cerebral blood flow and oxygen metabolism, and the results were compared with the concurrent clinical status of the patients. The cerebral metabolic rate of oxygen (CMRO 2) was low in five patients, and oxygen extraction fraction was low in four patients; both had a tendency to decline with advancing age. Although the cause is unknown, it is suggested that impaired oxidative metabolism exists in Rett syndrome. An analysis of the distribution among brain regions showed that the ratios of values for the frontal cortex to those for the temporal cortex for both cerebral blood flow and CMRO2 were lower than those for the controls, which may indicate the loss of hyperfrontality in Rett syndrome. (J Child Neurol 1991;6:237-242).

Journal of Child Neurology, Vol. 6, No. 3, 237-242 (1991)
DOI: 10.1177/088307389100600306
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