Semenza Carlo, Pignatti Riccardo, Bertella Laura, Ceriani Francesca, Mori Ileana, Molinari Enrico, Giardino Daniela, Malvestiti Francesca, Grugni Graziano
Department of Psychology, University of Trieste, Italy.
Neuropsychologia. 2008 Jan 15;46(1):206-12. doi: 10.1016/j.neuropsychologia.2007.07.017. Epub 2007 Aug 2.
Mathematical abilities were tested in people with Prader-Willi syndrome (PWS), using a series of basic mathematical tasks for which normative data are available. The difference between the deletion and the disomy variants of this condition was explored. While a wide phenotypic variation was found, some basic findings emerge clearly. As expected from previous literature, deletion and disomy participants were found to differ in their degree of impairment, with disomy being overall the most spared condition. However, the tasks selectively spared in the disomy condition are not necessarily the easiest ones and those that discriminate less the PWS group from controls. It rather seems that disomy patients are spared, with respect to deletion, in tasks entailing transcoding and comparison of numbers in the Arabic code. Overall a particular difficulty was detected in reliably performing parity judgments. This task has been shown to be very frequently spared after a brain injury, even in severe aphasic conditions. The most interesting result is the sparing in analog number scale, whereby PWS seem, overall, to outperform controls. This finding may help in understanding previously reported, surprising results about cognitive skills in PWS. Elevated performances in PWS may result from life-long hyper-reliance on one visuo-spatial system in presence of underdevelopment of the other.
研究人员使用一系列具备规范数据的基础数学任务，对普拉德-威利综合征（PWS）患者的数学能力进行了测试。同时，探究了该病症的缺失型和二体型变体之间的差异。尽管发现了广泛的表型变异，但一些基本发现清晰显现。正如先前文献所预期的那样，研究发现缺失型和二体型参与者在损伤程度上存在差异，总体而言二体型是受影响最小的情况。然而，在二体型情况下选择性保留的任务不一定是最容易的任务，也不是那些在区分PWS组和对照组方面差异较小的任务。似乎二体型患者在涉及阿拉伯数字编码的数字转码和比较任务中，相对于缺失型患者受到的影响较小。总体而言，在可靠地进行奇偶判断时检测到了特别的困难。即使在严重失语的情况下，该任务也经常在脑损伤后得以保留。最有趣的结果是在模拟数字量表任务中表现出的保留，总体而言PWS患者似乎比对照组表现更好。这一发现可能有助于理解先前报道的关于PWS患者认知技能的惊人结果。PWS患者表现提升可能是由于在一个视觉空间系统发育不全的情况下，终生过度依赖另一个视觉空间系统所致。
