Mathematical skills in Prader–Willi Syndrome

Abstract

Failure of mathematical skills is the most distinctive feature in the cognitive profile of PWS. However, to determine whether this is indeed a specific pattern of performance related to PWS, results must be compared with those obtained with patients manifesting other genetic disorders.

Developmental Origins of Low Mathematics Performance and Normal Variation in Twins from 7 to 9 Years

“…That is, when genes are found that contribute to the heritability of low mathematics performance, the same genes would be expected to contribute to the heritability of normal variation in mathematics performance. Of course there are rare monogenetic disorders that result in low mathematics performance, for example, individuals with Prader-Willi syndrome generally present with, among other problems, poor mathematical abilities (Bertella et al, 2005). Such individuals have been excluded from this analysis, and therefore the genetic effects found here are not likely to be the result of such severe and rare monogenic disorders.…”

Anatomical substrates and neurocognitive predictors of daily numerical abilities in mild cognitive impairment

“…In fact, if the intrinsic difficulty of the tasks were influencing the level of performance, one should expect that patients with other neurological conditions should also fail in this or in other similar tests. Rather, it is now well-established that different degenerative diseases show characteristic impairment profiles which follow particular lesional/degeneration patterns, such as in the case of Alzheimer disease (Carlomagno et al, 1999;Delazer, Karner, Proell & Benke, 2006;Helpern, McMilla, Moore, Dennis & Grossman, 2003;Martini, Domahs, Benke & Delazer, 2003;Zamarian et al, 2007b), Parkinson disease and other basal ganglia dysfunctions (Benke, Delazer, Bartha & Auer, 2003;Delazer et al, 2004;Tamura et al, 2003;Zamarian et al, 2006), semantic dementia (Cappelletti, Butterworth & Kopelman, 2001, 2012, amyothrophic lateral sclerosis (Palmieri et al, 2013), and genetic defects (e.g., Bertella et al, 2005;Semenza et al, 2008;Semenza et al, 2012). Each degenerative disease seems to affect aspects of number processing and calculation in a distinct way.…”

Genetic Research on Sleep, Sleep Disturbances and Associated Difficulties

“…Это означает, что если обнаруживаются гене-тические и средовые факторы, влияющие на математические способности, то значительная их часть влияет и на трудности в изучении математики . Однако отдельные редкие мутации, например при синдроме Дауна или синдроме Вильямса (Bertella et al, 2005;Mazzocco, 1998;Murphy, Mazzocco, 2008), могут также вносить вклад в нормальную изменчивость математических способностей/нарушений. Далее в этой главе данный вопрос рассмотрен подробнее.…”

“…При сравнении всех показателей теста Векс-лера у людей с СПВ и контрольной группы с таким же уровнем интеллек-та только показатели арифметических субтестов были значимо ниже у людей с СПВ. Эти результаты свидетельствуют, что низкий уровень IQ может являться не единственной причиной нарушения математических способностей (Bertella et al, 2005).…”