Dados do Trabalho

Título

TUBEROUS SCLEROSIS COMPLEX AND WEST SYNDROME: AN ASSESSMENT OF COGNITIVE ASPECTS

Introdução

Tuberous sclerosis complex (TSC) is a genetic disorder characterized by the development of benign tumors in multiple organs and tissues, especially in the brain, kidneys, heart, lungs, and skin. Brain lesions are frequently associated with cognitive deficits, neuropsychiatric disorders, learning disabilities, and seizures. Seizures occur in approximately 80% of patients and 30% to 60% of them had West Syndrome (WS).

Objetivo

The aim of the present study was to explore and describe cognitive development differences between patients with and without West Syndrome diagnosis. All of them had clinical or genetic diagnosis of TSC.

Métodos

A sample consisting 39 patients, from 6 to 27 years of age, answered the neuropsychological assessment. Group A, 10 of them (25,6%) had a history of  WS and Group B, 29 of them without WS. Participants  were assessed by the Wechsler Intelligence Scale for Children (WISC-IV) or the Wechler Adult Intelligence Scale (WAIS-III). Data were analyzed using density graphs.

Resultados

There were not significant differences between groups regarding total IQ, verbal comprehension, perceptual organization, and working memory indexes. The processing speed index showed significant differences between groups, with an index of 60 to 80 on most of the participants with TSC and WS, and 80 to 100 on patients with TSC only.

Conclusões

The study showed a significant impact on processing speed index on patients with TSC associated with WS. Given that there weren’t significant differences between groups regarding the other indexes assessed. The impact on those may be caused by TSC in general, without specific influence by WS. A more in-depth study on processing speed in patients with TSC associated with WS is needed, with investigation of other variables, such as treatment modalities. Considering the developmental impact of WS, it is important to identify and control seizures and infantile spasms in early childhood, avoiding lifetime impacts on cognition as shown by these results.

Palavras chave

Tuberous sclerosis complex; West Syndrome; Cognitive Aspects.

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