Assessment of brain perfusion by ECD-Tc99m SPET in Rett Syndrome

Rett syndrome (RS) is a progressive neurological pediatric disorder which affects only girls. The aim of our study is to determinate if brain perfusion abnormalities might be able to explain the clinical manifestations and progression of the disease. Quantitative global and regional brain blood flow was evaluated in 25 RS girls and compared with a reference group of 9 children. SPET revealed a considerable global reduction in cerebral perfusion in the group of RS girls and a great statistical difference was recorded when compared with the control group. The reduction of cerebral perfusion reflects the functional disturbances in the brains of RS girls, which might be associated with precocious brain atrophy, even when morphologic imaging (MRI) appears normal.