Launes J, Heiskala H, Nikkinen P, Santavuori P
Department of Neurology, University Central Hospital, Helsinki, Finland.
Neuropediatrics. 1996 Apr;27(2):84-7. doi: 10.1055/s-2007-973754.
The juvenile neuronal ceroid-lipofuscinosis (JNCL) is a recessively inherited progressive encephalopathy. We studied 21 JNCL patients with a duration of illness of 1 to 17 years by 99mTc-HM-PAO single photon emission computed tomography (SPECT) and correlated the findings with clinical parameters. All patients had at least one hypoperfused brain area, the median number of such areas was 5 per patient. Parietally, occipitally, and in the cerebellar lobes hypoperfusion was usually mild whereas it was temporally more severe. Right parietal hypoperfusion correlated to neurological dysfunction.
青少年神经元蜡样脂褐质沉积症（JNCL）是一种隐性遗传的进行性脑病。我们通过99mTc-HM-PAO单光子发射计算机断层扫描（SPECT）对21例病程为1至17年的JNCL患者进行了研究，并将结果与临床参数进行了关联分析。所有患者至少有一个脑灌注不足区域，每位患者此类区域的中位数为5个。在顶叶、枕叶和小脑叶，灌注不足通常较轻，而在颞叶则更严重。右侧顶叶灌注不足与神经功能障碍相关。
