Liewendahl K, Vanhanen S L, Heiskala H, Raininko R, Nikkinen P, Launes J, Santavuori P
Laboratory Department, University of Helsinki, Finland.
Neuropediatrics. 1997 Feb;28(1):71-3. doi: 10.1055/s-2007-973674.
Brain perfusion was studied with the Tc-99m-HMPAO SPECT method in 19 INCL patients, 21 JNCL patients and 5 patients with Jansky-Bielschowsky variant disease (JBVD). The typical SPECT findings at an early stage of INCL were bilateral anterior frontal, posterior temporoparietal and occipital hypoperfusion, whereas reduction in cerebellar perfusion appeared later. However, perfusion of basal ganglia and thalami, although atrophic on MRI, was usually well preserved up to the terminal stage. All JNCL patients except one had at least one hypoperfused area. Mild hypoperfusion was usually located in the parietal and occipital lobes and cerebellum, whereas more severe hypoperfusion was observed in the temporal lobes. In JNCL, SPECT revealed lesions not detected on CT. All JBVD patients had supra- and infratentorial hypoperfusion, which was usually bilateral. This study shows that although in NCLs brain hypoperfusion can appear prior to structural abnormalities seen on MRI or CT, such abnormalities are not always associated with significant hypoperfusion.
采用锝-99m-六甲基丙烯胺肟单光子发射计算机断层扫描（Tc-99m-HMPAO SPECT）方法，对19例婴儿晚期神经元蜡样脂褐质沉积病（INCL）患者、21例少年型神经元蜡样脂褐质沉积病（JNCL）患者和5例扬斯基-比尔绍夫斯基变异型疾病（JBVD）患者的脑灌注情况进行了研究。INCL早期典型的SPECT表现为双侧额叶前部、颞顶叶后部和枕叶灌注减低，而小脑灌注减低出现较晚。然而，基底神经节和丘脑尽管在磁共振成像（MRI）上有萎缩，但在终末期之前其灌注通常保持良好。除1例JNCL患者外，所有患者均至少有一个灌注减低区。轻度灌注减低通常位于顶叶、枕叶和小脑，而颞叶则可见更严重的灌注减低。在JNCL中，SPECT显示出CT上未检测到的病变。所有JBVD患者均有幕上和幕下灌注减低，且通常为双侧性。本研究表明，尽管在神经元蜡样脂褐质沉积病（NCLs）中脑灌注减低可先于MRI或CT上所见的结构异常出现，但这些异常并不总是与明显的灌注减低相关。
