TY - JOUR
T1 - Brain glucose metabolism in Rett syndrome
AU - Villemagne, Patricia M.
AU - Naidu, Sakkubai
AU - Villemagne, Victor L.
AU - Yaster, Myron
AU - Wagner, Henry N.
AU - Harris, James C.
AU - Moser, Hugo W.
AU - Johnston, Michael V.
AU - Dannals, Robert F.
AU - Wong, Dean F.
N1 - Funding Information:
The authors thank Harry T Chugani, MD, for providing individual absolute rates of glucose metabolism in control patients, and Hayden T Ravert, PhD, Alan A Wilson, PhD, David Clough, CNMT, Madleen Murrell, RN, and Robert Smoot, CNMT, for their excellent collaboration. This work was supported in part by Public Health Service Grants #PO1 HD24448 and PO1 HD 24061 and DA00412.
PY - 2002/8
Y1 - 2002/8
N2 - 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 [18F]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 [18F]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.
AB - 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 [18F]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 [18F]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.
UR - http://www.scopus.com/inward/record.url?scp=0036696126&partnerID=8YFLogxK
U2 - 10.1016/S0887-8994(02)00399-5
DO - 10.1016/S0887-8994(02)00399-5
M3 - Article
C2 - 12213612
AN - SCOPUS:0036696126
SN - 0887-8994
VL - 27
SP - 117
EP - 122
JO - Pediatric Neurology
JF - Pediatric Neurology
IS - 2
ER -