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Attention deficits in Tuberous Sclerosis Complex (TSC) - rethinking the pathways to the endstate
de Vries, P., WATSON, P.
Journal of Intellectual Disability Research, 52(4), 348-357
Year of publication:
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 to 16 year olds with TSC utilizing 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-expected 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.