Brain glucose metabolism in Rett Syndrome.
Rett syndrome is a progressive neurologic disorder affecting girls in early childhood with loss of achieved psychomotor abilities and mental retardation. Six sedated female patients (4 to 15 years of age) with a diagnosis of Rett syndrome were studied with [(18)F]fluorodeoxyglucose (FDG) and underwent positron emission tomography scanning of the brain. Relative tracer concentrations between different areas of the brain were assessed, and results were compared with 18 age-matched control subjects. Patients were divided into two age groups: 3 to 8 years of age and 9 to 15 years of age. A relative decrease in [(18)F]FDG uptake in the lateral occipital areas in relation with the whole brain and a relative increase in the cerebellum was evident in both age groups (P < 0.001, unpaired Student t test). A relative increase in frontal tracer uptake was observed in the younger group. Sensorimotor areas and relations between cortical and subcortical structures were preserved in all patients. Changes in glucose cerebral metabolism resemble the regional distribution of normal children less than 1 year of age, likely reflecting a maturational arrest. Changes in frontal areas parallel those in postmortem N-methyl-D-aspartate receptor densities and could correlate with different clinical stages of the disease. This pattern differs from those described in Down syndrome, autism, and Alzheimer's disease.
Villemagne
Patricia M
PM
Department of Radiology, Johns Hopkins Medical Institutions;, Baltimore, Maryland 21287-0807, USA.
Naidu
Sakkubai
S
Villemagne
Victor L
VL
Yaster
Myron
M
Wagner
Henry N
HN
Harris
James C
JC
Moser
Hugo W
HW
Johnston
Michael V
MV
Dannals
Robert F
RF
Wong
Dean F
DF
D000293:Adolescent; D001921:Brain / Q000000981:diagnostic imaging / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D005260:Female; D019788:Fluorodeoxyglucose F18 / Q000493:pharmacokinetics; D005625:Frontal Lobe / Q000000981:diagnostic imaging / Q000378:metabolism; D005947:Glucose / Q000378:metabolism*; D006801:Humans; D008297:Male; D010296:Parietal Lobe / Q000000981:diagnostic imaging / Q000378:metabolism; D019275:Radiopharmaceuticals / Q000493:pharmacokinetics; D015518:Rett Syndrome / Q000175:diagnosis / Q000378:metabolism*; D013702:Temporal Lobe / Q000000981:diagnostic imaging / Q000378:metabolism; D014055:Tomography, Emission-Computed; D014057:Tomography, X-Ray Computed
D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18; D005947:Glucose