title: Genetics and mathematics: evidence from Prader-Willi syndrome.
abstract: 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.
authors.affiliation: Department of Psychology, University of Trieste, Italy. semenza@univ.trieste.it
authors.forename: Carlo
authors.initials: C
authors.lastname: Semenza
authors.forename: Riccardo
authors.initials: R
authors.lastname: Pignatti
authors.forename: Laura
authors.initials: L
authors.lastname: Bertella
authors.forename: Francesca
authors.initials: F
authors.lastname: Ceriani
authors.forename: Ileana
authors.initials: I
authors.lastname: Mori
authors.forename: Enrico
authors.initials: E
authors.lastname: Molinari
authors.forename: Daniela
authors.initials: D
authors.lastname: Giardino
authors.forename: Francesca
authors.initials: F
authors.lastname: Malvestiti
authors.forename: Graziano
authors.initials: G
authors.lastname: Grugni
mesh_terms: D000328:Adult; D002872:Chromosome Deletion; D005260:Female; D006801:Humans; D007360:Intelligence / Q000502:physiology; D008297:Male; D008433:Mathematics; D008606:Mental Processes / Q000502:physiology*; D009483:Neuropsychological Tests / Q000706:statistics & numerical data; D011218:Prader-Willi Syndrome / Q000235:genetics* / Q000503:physiopathology*; D012016:Reference Values; D024182:Uniparental Disomy / Q000235:genetics*