Belgian Journal of Paediatrics
Surgical treatment for infantile spasms (West syndrome): a case report
Vol. 23 No. 1 (2021), Case reports
Vol. 23 No. 1 (2021)

Surgical treatment for infantile spasms (West syndrome): a case report

Case reports
Published 2021-04-12 — Updated on 2023-01-18
Justine Van Gaver
Department of Pediatrics, Hôpital Universitaire des Enfants Reine Fabiola, Université Libre de Bruxelles, Brussels, Belgium
Claudine Sculier
Department of Neurology, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium
Laetitia Lebrun
Department of Pathology, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium
Marie Lucie Racu
Department of Pathology, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium
Isabelle Salmon
Department of Pathology, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium
Olivier Dewitte
Department of Neurosurgery, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium
Grammatina Boitsios
Department of Radiology and medical imaging, Hôpital Universitaire des Enfants Reine Fabiola, Université Libre de Bruxelles, Brussels, Belgium
Patrick Van Bogaert
Department of Pediatric Neurology, CHU d'Angers and Laboratoire Angevin de Recherche en Ingénierie des Systèmes (LARIS), Université d'Angers, France
Alec Aeby
Department of Pediatric Neurology, Hôpital Universitaire des Enfants Reine Fabiola, Université Libre de Bruxelles, Brussels, Belgium

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Keywords

infantile spasms
West syndrome
pediatric brain tumor
BRAFV600E

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How to Cite

Van Gaver, J., Sculier, C., Lebrun, L., Racu, M. L., Salmon, I., Dewitte, O., Boitsios, G., Van Bogaert, P., & Aeby, A. (2023). Surgical treatment for infantile spasms (West syndrome): a case report. Belgian Journal of Paediatrics, 23(1), 76–78. Retrieved from http://belgjpaediatrics.com/index.php/bjp/article/view/183 (Original work published April 12, 2021)
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Abstract

We report the case of a 10-month-old boy with developmental regression, symmetrical flexion spasms and hypsarrhythmia, characteristic of West syndrome. Brain magnetic resonance imaging showed an expansive entorhinal and right parahippocampal lesion. A right temporal lobectomy was performed. Pathological examination showed the presence of a low-grade oligodendrocyte-like glioneuronal tumor with a BRAF V600E genetic variant.

Our case highlights the role of the BRAF V600E genetic variant in the development of this refractory epileptic syndrome. In addition, it shows that surgery offers a potentially curative treatment for epilepsy in the subgroup of children with a focal brain lesion.

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