Turner Martin R, Hammers Alexander, Al-Chalabi Ammar, Shaw Christopher E, Andersen Peter M, Brooks David J, Leigh P Nigel
Department of Neurology, The Radcliffe Infirmary, Oxford, UK.
J Neurol. 2007 Aug;254(8):1033-6. doi: 10.1007/s00415-006-0482-7. Epub 2007 Feb 9.
Four PLS patients underwent cerebral [(11)C]-flumazenil PET. They were compared firstly with a group of controls, then later directly with a group of sporadic ALS patients and a familial ALS group homozygous for the 'D90A' SOD1 gene mutation. There was a similar pattern of decreased binding in PLS patients when compared to controls as that seen in a previous study of sporadic ALS patients, supporting the concept that PLS is part of the same overall spectrum of MND. However, in direct group comparisons, both sporadic and homD90A ALS patients demonstrated relative decreases in anterior and orbito-frontal binding compared to PLS patients, suggesting that there may be differences in cortical vulnerability between phenotypic groups.
四名进行性核上性麻痹（PLS）患者接受了脑[(11)C]-氟马西尼正电子发射断层扫描（PET）。首先将他们与一组对照组进行比较，随后又直接与一组散发性肌萎缩侧索硬化症（ALS）患者以及一组携带“SOD1基因‘D90A’突变的纯合子家族性ALS患者进行比较。与对照组相比，PLS患者的结合减少模式与先前对散发性ALS患者的研究中所见相似，这支持了PLS是运动神经元病（MND）同一总体谱系一部分的概念。然而，在直接的组间比较中，与PLS患者相比，散发性和D90A纯合子ALS患者在前额叶和眶额结合方面均表现出相对减少，这表明表型组之间可能存在皮质易损性差异。
