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CC BY 4.0 · Arq Neuropsiquiatr 2023; 81(S 01): S1-S96
DOI: 10.1055/s-0043-1774434
SCIENTIFIC WORK
Epilepsias
Code: PE047

Tuberous sclerosis complex and west syndrome: an assessment of cognitive aspects

Patricia do Rocio Litça
1   Universidade Federal do Paraná, Curitiba PR, Brazil
,
Luísa Teixeira dos Santos
1   Universidade Federal do Paraná, Curitiba PR, Brazil
,
Angel Miríade
1   Universidade Federal do Paraná, Curitiba PR, Brazil
,
Laís Faria Masulk Cardozo
2   Universidade Federal do Paraná, Complexo Hospital de Clínicas, Curitiba PR, Brazil
,
Sérgio Antonio Antoniuk
2   Universidade Federal do Paraná, Complexo Hospital de Clínicas, Curitiba PR, Brazil
,
Ana Paula Almeida de Pereira
2   Universidade Federal do Paraná, Complexo Hospital de Clínicas, Curitiba PR, Brazil
,
Ana Chrystina de Souza Crippa
2   Universidade Federal do Paraná, Complexo Hospital de Clínicas, Curitiba PR, Brazil
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    Background: 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).

    Objective: 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.

    Methods: A sample consisting of 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.

    Results: 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.

    Conclusions: 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.


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    No conflict of interest has been declared by the author(s).

    Publication History

    Article published online:
    18 September 2023

    © 2023. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/)

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