Is there really evidence for neurodegeneration in Dravet syndrome? Commentary on the publication by Selvarajah et al

Sanjay M Sisodiya¹, Simona Balestrini^{1

2

3}, Samuel F Berkovic^{4

5}, Kailash P Bhatia⁶, Lisa M Clayton¹, J Helen Cross⁷, Claire Eldred⁸, Antonio Gambardella⁹, Renzo Guerrini^{2

3}, Rima Nabbout¹⁰, Adam Strzelczyk¹¹, Galia Wilson⁸, Ingrid E Scheffer^{12

13}

Affiliations

¹ Department of Clinical and Experimental Epilepsy, UCL Queen Square Institute of Neurology, London, UK.
² Neuroscience and Medical Genetics Department, Meyer Children's Hospital IRCCS, Florence, Italy.
³ Department of Neuroscience, Pharmacology and Child Health, University of Florence, Florence, Italy.
⁴ Epilepsy Research Centre, Department of Medicine (Austin Health), The University of Melbourne, Heidelberg, Victoria, Australia.
⁵ Bladin-Berkovic Comprehensive Epilepsy Program, Department of Neurology, Austin Health, Heidelberg, Victoria, Australia.
⁶ Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, London, UK.
⁷ UCL NIHR BRC Great Ormond Street Institute of Child Health, London, UK.
⁸ Dravet Syndrome UK, Chesterfield, UK.
⁹ Institute of Neurology, Department of Medical Sciences, University Magna Græcia, Catanzaro, Italy.
¹⁰ Reference Centre for Rare Epilepsies, Department of Pediatric Neurology, Hopital Necker Enfants Malades, Member of European Network EPICARE, INSERM, UMR 1163, Translational Research for Neurological Disorders, Insititut Imagine, Université Paris cité, Paris, France.
¹¹ Epilepsiezentrum Frankfurt Rhein-Main, Zentrum der Neurologie Und Neurochirurgie, Universitätsmedizin Frankfurt, Goethe-Universität Frankfurt, Frankfurt am Main, Germany.
¹² University of Melbourne, Austin Health and Royal Children's Hospital, Melbourne, Victoria, Australia.
¹³ Florey and Murdoch Children's Research Institutes, Melbourne, Victoria, Australia.

PMID: 40762979
DOI: 10.1111/epi.18582

Is there really evidence for neurodegeneration in Dravet syndrome? Commentary on the publication by Selvarajah et al

Sanjay M Sisodiya et al. Epilepsia. 2025 Oct.

. 2025 Oct;66(10):3676-3680.

doi: 10.1111/epi.18582. Epub 2025 Aug 5.

Authors

Affiliations

¹ Department of Clinical and Experimental Epilepsy, UCL Queen Square Institute of Neurology, London, UK.
² Neuroscience and Medical Genetics Department, Meyer Children's Hospital IRCCS, Florence, Italy.
³ Department of Neuroscience, Pharmacology and Child Health, University of Florence, Florence, Italy.
⁴ Epilepsy Research Centre, Department of Medicine (Austin Health), The University of Melbourne, Heidelberg, Victoria, Australia.
⁵ Bladin-Berkovic Comprehensive Epilepsy Program, Department of Neurology, Austin Health, Heidelberg, Victoria, Australia.
⁶ Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, London, UK.
⁷ UCL NIHR BRC Great Ormond Street Institute of Child Health, London, UK.
⁸ Dravet Syndrome UK, Chesterfield, UK.
⁹ Institute of Neurology, Department of Medical Sciences, University Magna Græcia, Catanzaro, Italy.
¹⁰ Reference Centre for Rare Epilepsies, Department of Pediatric Neurology, Hopital Necker Enfants Malades, Member of European Network EPICARE, INSERM, UMR 1163, Translational Research for Neurological Disorders, Insititut Imagine, Université Paris cité, Paris, France.
¹¹ Epilepsiezentrum Frankfurt Rhein-Main, Zentrum der Neurologie Und Neurochirurgie, Universitätsmedizin Frankfurt, Goethe-Universität Frankfurt, Frankfurt am Main, Germany.
¹² University of Melbourne, Austin Health and Royal Children's Hospital, Melbourne, Victoria, Australia.
¹³ Florey and Murdoch Children's Research Institutes, Melbourne, Victoria, Australia.

PMID: 40762979
DOI: 10.1111/epi.18582

No abstract available

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References

REFERENCES

1. Selvarajah A, Sabo A, Gorodetsky C, Marques PT, Chandran I, Thompson M, et al. Dravet syndrome: from neurodevelopmental to neurodegenerative disease? Epilepsia. 2025;66:1975–1987. https://doi.org/10.1111/epi.18329
1. Wilson DM 3rd, Cookson MR, van den Bosch L, Zetterberg H, Holtzman DM, Dewachter I. Hallmarks of neurodegenerative diseases. Cell. 2023;186(4):693–714.
1. Catarino CB, Liu JY, Liagkouras I, Gibbons VS, Labrum RW, Ellis R, et al. Dravet syndrome as epileptic encephalopathy: evidence from long‐term course and neuropathology. Brain. 2011;134(Pt 10):2982–3010.
1. Jansen FE, Sadleir LG, Harkin LA, Vadlamudi L, McMahon JM, Mulley JC, et al. Severe myoclonic epilepsy of infancy (Dravet syndrome): recognition and diagnosis in adults. Neurology. 2006;67(12):2224–2226.
1. Rodda JM, Scheffer IE, McMahon JM, Berkovic SF, Graham HK. Progressive gait deterioration in adolescents with Dravet syndrome. Arch Neurol. 2012;69(7):873–878.

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Is there really evidence for neurodegeneration in Dravet syndrome? Commentary on the publication by Selvarajah et al

Affiliations

Is there really evidence for neurodegeneration in Dravet syndrome? Commentary on the publication by Selvarajah et al

Authors

Affiliations

References

REFERENCES

LinkOut - more resources

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