Burroni L, Aucone A M, Volterrani D, Hayek Y, Bertelli P, Vella A, Zappella M, Vattimo A
Department of Nuclear Medicine, University of Siena, Policlinico Le Scotte, Italy.
Nucl Med Commun. 1997 Jun;18(6):527-34. doi: 10.1097/00006231-199706000-00005.
Rett syndrome is a progressive neurological paediatric disorder associated with severe mental deficiency, which affects only girls. The aim of this study was to determine if brain blood flow abnormalities detected with 99Tc(m)-ethyl-cysteinate-dimer (99Tc[m]-ECD) single photon emission tomography (SPET) can explain the clinical manifestation and progression of the disease. Qualitative and quantitative global and regional brain blood flow was evaluated in 12 girls with Rett syndrome and compared with an aged-matched reference group of children. In comparison with the reference group, SPET revealed a considerable global reduction in cerebral perfusion in the groups of girls with Rett syndrome. A large statistical difference was noted, which was more evident when comparing the control group with girls with stage IV Rett syndrome than girls with stage III Rett syndrome. The reduction in cerebral perfusion reflects functional disturbance in the brain of children with Rett syndrome. These data confirm that 99Tc(m)-ECD brain SPET is sensitive in detecting hypoperfused areas in girls with Rett syndrome that may be associated with brain atrophy, even when magnetic resonance imaging appears normal.
雷特综合征是一种与严重智力缺陷相关的进行性儿科神经疾病，仅影响女孩。本研究的目的是确定用99锝（m）-乙半胱氨酸二聚体（99Tc[m]-ECD）单光子发射断层扫描（SPET）检测到的脑血流异常是否能解释该疾病的临床表现和进展。对12名雷特综合征女孩的全脑和局部脑血流进行了定性和定量评估，并与年龄匹配的儿童参照组进行了比较。与参照组相比，SPET显示雷特综合征女孩组全脑灌注显著降低。观察到有很大的统计学差异，与III期雷特综合征女孩相比，对照组与IV期雷特综合征女孩比较时差异更明显。脑灌注减少反映了雷特综合征患儿大脑的功能障碍。这些数据证实，99Tc（m）-ECD脑SPET在检测雷特综合征女孩可能与脑萎缩相关的灌注不足区域方面很敏感，即使磁共振成像显示正常。
