Literature DB >> 21191642

Neuropsychological attention skills and related behaviours in adults with tuberous sclerosis complex.

Kevin M Tierney1, Deborah L McCartney, Jaco R Serfontein, Petrus J de Vries.   

Abstract

Tuberous sclerosis complex (TSC) is a genetic disorder associated with mTOR over-activation and disruption of MAPK, PI3K and AMPK signalling. Children with TSC have significant deficits on neuropsychological attention tasks, particularly dual tasking. Here we investigated attentional skills and related behaviours in daily life in normally intelligent adults with TSC and matched controls using the Test of Everyday Attention for Children (TEA-Ch) and the Attention-Deficit Scales for Adults (ADSA). No group differences were demonstrated on selective or sustained attention tasks carried out alone. However, adults with TSC performed significantly worse when these tasks were combined in a cross-modal dual task condition. On the ADSA the TSC group had significantly worse scores on several subscales (attention/concentration, behaviour/disorganization, academic and emotional behaviours) compared to controls and these correlated with dual task performance, indicating a clear impact of dual task deficits on attention-related behaviours in daily life. The presence or absence of epilepsy did not influence dual task performance or attention-deficits in daily life. Taken together with similar findings in children, results suggest that dual task difficulties are a core feature of the neuropsychological phenotype of TSC.

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Year:  2010        PMID: 21191642     DOI: 10.1007/s10519-010-9423-4

Source DB:  PubMed          Journal:  Behav Genet        ISSN: 0001-8244            Impact factor:   2.805


  5 in total

Review 1.  A clinical update on tuberous sclerosis complex-associated neuropsychiatric disorders (TAND).

Authors:  Petrus J de Vries; Lucy Wilde; Magdalena C de Vries; Romina Moavero; Deborah A Pearson; Paolo Curatolo
Journal:  Am J Med Genet C Semin Med Genet       Date:  2018-08-16       Impact factor: 3.908

2.  Tuberous sclerosis associated neuropsychiatric disorders (TAND) and the TAND Checklist.

Authors:  Petrus J de Vries; Vicky H Whittemore; Loren Leclezio; Anna W Byars; David Dunn; Kevin C Ess; Dena Hook; Bryan H King; Mustafa Sahin; Anna Jansen
Journal:  Pediatr Neurol       Date:  2014-10-16       Impact factor: 3.372

3.  Graded loss of tuberin in an allelic series of brain models of TSC correlates with survival, and biochemical, histological and behavioral features.

Authors:  Elizabeth Yuan; Peter T Tsai; Emily Greene-Colozzi; Mustafa Sahin; David J Kwiatkowski; Izabela A Malinowska
Journal:  Hum Mol Genet       Date:  2012-06-29       Impact factor: 6.150

4.  Multivariate data analysis identifies natural clusters of Tuberous Sclerosis Complex Associated Neuropsychiatric Disorders (TAND).

Authors:  Petrus J de Vries; Loren Leclezio; Sugnet Gardner-Lubbe; Darcy Krueger; Mustafa Sahin; Steven Sparagana; Liesbeth De Waele; Anna Jansen
Journal:  Orphanet J Rare Dis       Date:  2021-10-24       Impact factor: 4.123

Review 5.  Healthcare transition from childhood to adulthood in Tuberous Sclerosis Complex.

Authors:  Angela Peron; Maria Paola Canevini; Filippo Ghelma; Fabiano Di Marco; Aglaia Vignoli
Journal:  Am J Med Genet C Semin Med Genet       Date:  2018-09-25       Impact factor: 3.908

  5 in total

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