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Attention deficits in tuberous sclerosis complex (TSC): rethinking the pathways to the endstate.

Accepted version
Peer-reviewed

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Type

Article

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Authors

de Vries, PJ 

Abstract

BACKGROUND: Tuberous sclerosis complex (TSC) is a genetic disorder associated with a range of neurocognitive manifestations, including neuropsychological attention deficits most notably in dual tasking/divided attention. These dual-task deficits have so far been interpreted as evidence of a vulnerable 'cognitive module' in TSC. Here, we suggest that this interpretation represents an 'adult neuropsychological' perspective, and argue that a developmental approach would be more appropriate to examine attention deficits in TSC. METHOD: We examined the pathway to 'endstate' dual-task deficits in twenty 6-16 year olds with TSC utilising the Test of Everyday Attention for Children (TEA-Ch). We predicted that the pattern of attentional deficits in TSC would support a 'conditional' model where the establishment of a later-maturing skill was dependent on the functional maturation of an earlier expected skill. RESULTS: Attentional profiles showed statistical support for a conditional model. Only one child showed a deterministic pattern while one showed a hybrid pattern, attributed to the admixture of a surgically acquired lesion and a neurodevelopmental disorder. CONCLUSION: This preliminary study suggests that the developmental cascade in TSC may be arrested at various stages of neuropsychological development, thus leading to different developmental trajectories towards similar 'endstate' profiles.

Description

Keywords

Adolescent, Attention Deficit Disorder with Hyperactivity, Child, Comorbidity, Female, Humans, Intelligence Tests, Male, Neuropsychological Tests, Task Performance and Analysis, Tuberous Sclerosis, United Kingdom

Journal Title

J Intellect Disabil Res

Conference Name

Journal ISSN

0964-2633
1365-2788

Volume Title

52

Publisher

Wiley

Rights

All rights reserved