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The Tuberous Sclerosis is a neurocutaneous autosomal dominant syndrome with varied clinical expressions. Most patients present alterations in brain function, which highlights the cognitive assessment as an important tool for evaluating the mentioned alterations. The present work aims to relate the general intellectual performance to the performance of working memory in patients with Tuberous Sclerosis. 12 patients were evaluated; both sexes aged between 6 years and 0 months and 29 years and 11 months. For cognitive assessment, were used the Wechsler Intelligence Scales Third Edition, for children and adults. From this assessment the general intellectual level and performance on Digits subtest of each participant were obtained. Regarding the general intellectual level the results show that 33.3% of participants are in a lower average rating, 16.7% are at average, 41.7% borderline performance and 8.3% are at extremely below expectations. Regarding the performance in working memory assessed by Digits subtest, 100% of participants showed lower performance, that is, below the expected average for the corresponding age group. In view of this, it is concluded that the patients evaluated had low performance in working memory, even patients with general intellectual performance within or close to the expected average. The influence of low working memory performance is observed in academic and social spheres, being this difficulty one of the factors for high rates of school failure, learning difficulties and daily difficulties lives, such as frequent forgetfulness, among others.

Tuberous Sclerosis; Memory; Cognitive Assessment.