Dravet syndrome: From neurodevelopmental to neurodegenerative disease?

This is the largest group of adults with DS to be systematically evaluated. They had severe nonseizure symptoms. Older age and longer use of SCBs were associated with worse adaptive skills, gait, and parkinsonism. Some older adults screened positive for depression and dementia. Caregivers identified functional decline in activities of daily living (ADLs). Taken together, the risk of dementia, parkinsonian gait, and decline in ability to perform previously mastered ADLs support that some adults with DS may be developing a neurodegenerative disorder.

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Institutions

Publication Details

Journal
Epilepsia
Published
2025-03-04
DOI
https://doi.org/10.1111/epi.18329
Citations
11
Primary Topic
Epilepsy research and treatment
Type
article
Field-Weighted Citation Impact
16.18

Funders

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article

Dravet syndrome: From neurodevelopmental to neurodegenerative disease?

Carolina Gorodetsky, Orrin Devinsky, Kette D. R. Valente, Nicole Villas et al.
11 citations
Epilepsia
Epilepsy research and treatment
16.18
article

Dravet syndrome: From neurodevelopmental to neurodegenerative disease?

Carolina Gorodetsky, Orrin Devinsky, Kette D. R. Valente, Nicole Villas, Paula Marques, Babak Taati, Linda Huh, Maria Carmela Tartaglia, Tadeu A. Fantaneanu, Andrea Sabo, Ryan K. C. Yuen, Danielle Molinari Andrade, Anne T. Berg, Mary Connolly, Mary Anne Meskis, Alfonso A. Fasano, Mary Pat McAndrews, Victor Lira, Luciana Midori Inuzuka, Elena Cardenal‐Muñoz, Karen Barboza, Veronica L. Hood, Quratulain Zulfiqar Ali, José Ángel Aibar, Miles D. Thompson, Stéphane Auvin, Eliana Garzón, Christine Linehan, Lysa Boissé Lomax, M. E. Crespo Espindola, Gianluca Sorrento, Farah Qaiser, Sophie Höhn, Arunan Selvarajah, Arezoo Rezazadeh, Marta González, Ilakkiah Chandran, Lori Ann McKenna, Monica Duong, Jack Arbinuch
article en
11 citations

Abstract

This is the largest group of adults with DS to be systematically evaluated. They had severe nonseizure symptoms. Older age and longer use of SCBs were associated with worse adaptive skills, gait, and parkinsonism. Some older adults screened positive for depression and dementia. Caregivers identified functional decline in activities of daily living (ADLs). Taken together, the risk of dementia, parkinsonian gait, and decline in ability to perform previously mastered ADLs support that some adults with DS may be developing a neurodegenerative disorder.

EpilepsiaVol. 66(6)
University College Dublin (IE), Lurie Children's Hospital (US), University Health Network (CA), University of British Columbia (CA), University of Ottawa (CA), University of Toronto (CA), Université Paris Cité (FR), Toronto Western Hospital (CA), Dravet Syndrome Foundation (US), Ottawa Hospital (CA), Kingston General Hospital (CA), Hospital for Sick Children (CA), Institut des Neurosciences Paris-Saclay (FR), NYU Langone Health (US), Hôpital Robert-Debré (FR), SickKids Foundation (CA), Fundación Botín (ES), Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (BR), BC Children's Hospital (CA), Neurological Surgery (US), Krembil Research Institute, University of Oklahoma (US)
Dravet Syndrome Foundation
Quality Education
Openalex Percentile: Top 1%
Epilepsy research and treatment
16.18
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