Lappalainen R, Liewendahl K, Sainio K, Nikkinen P, Riikonen R S
Department of Child Neurology, Children's Castle Hospital, Helsinki, Finland.
Acta Neurol Scand. 1997 Jan;95(1):44-50. doi: 10.1111/j.1600-0404.1997.tb00067.x.
Thirteen patients (mean age 8.4 + 5.3 years) with Rett syndrome (RS) were studied with EEG and 99mTc-HMPAO SPECT. Eleven patients had background abnormalities and 10 patients paroxysmal activity in EEG. Hypoperfusion of varying severity was detected in 11 patients, 7 patients having multiple lesions. Bifrontal hypoperfusion, observed in 6 patients, was the most distinctive finding. Hypoperfusion was observed also in other cortical regions, except for the occipital lobes. There was no correlation between severity of the background abnormality or presence of paroxysmal activity in EEG and grade of hypoperfusion. There was, however, an association between the severity of hypoperfusion and early manifestation of symptoms in patients with RS. Whether this early-onset group of patients represents a different disease entity or only reflects disease variability the basic pathology being the same, is a possibility that deserves further clarification.
对13例雷特综合征（RS）患者（平均年龄8.4±5.3岁）进行了脑电图（EEG）和99mTc-六甲基丙二胺肟单光子发射计算机断层扫描（99mTc-HMPAO SPECT）研究。11例患者脑电图有背景异常，10例患者有阵发性活动。11例患者检测到不同程度的灌注不足，7例患者有多处病变。6例患者出现的双侧额叶灌注不足是最显著的发现。除枕叶外，其他皮质区域也观察到灌注不足。脑电图背景异常的严重程度或阵发性活动的存在与灌注不足的程度之间没有相关性。然而，RS患者灌注不足的严重程度与症状的早期表现之间存在关联。这组早发型患者是代表不同的疾病实体，还是仅反映疾病变异性而基本病理相同，这是一个值得进一步阐明的可能性。
