REGIONAL CEREBRAL BLOOD-FLOW ABNORMALITIES IN RETT-SYNDROME

Sixteen Rett syndrome (RS) girls and 1 24 year old RS woman were compa red with 16 neurologically healthy children as to the regional cerebra l blood flow (rCBF). Hypoperfusion of the frontal lobes and parts of t he midbrain was found in RS, with significant differences to controls at latest by the age of three to four years. The findings were in acco rdance with previous reports of morphological, neuroradiological and f unctional abnormalities in these areas, compatible with a statement th at structures in the midbrain and in the frontal lobes may have partic ular pathophysiological relevance in RS.