title: Neuropsychological attention skills and related behaviours in adults with tuberous sclerosis complex.
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.
authors.affiliation: Neurodevelopmental Service (NDS), Cambridgeshire and Peterborough NHS Foundation Trust, Peterborough, UK.
authors.forename: Kevin M
authors.initials: KM
authors.lastname: Tierney
authors.forename: Deborah L
authors.initials: DL
authors.lastname: McCartney
authors.forename: Jaco R
authors.initials: JR
authors.lastname: Serfontein
authors.forename: Petrus J
authors.initials: PJ
authors.lastname: de Vries
mesh_terms: D000328:Adult; D001288:Attention; D001289:Attention Deficit Disorder with Hyperactivity / Q000175:diagnosis / Q000235:genetics*; D004827:Epilepsy / Q000235:genetics; D005260:Female; D006801:Humans; D007360:Intelligence / Q000235:genetics; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests / Q000706:statistics & numerical data*; D010641:Phenotype; D011594:Psychometrics; D058570:TOR Serine-Threonine Kinases / Q000235:genetics; D014402:Tuberous Sclerosis / Q000175:diagnosis / Q000235:genetics*
chemical_list: C546842:MTOR protein, human; D058570:TOR Serine-Threonine Kinases