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Presurgical Evaluation of Children with Tuberous Sclerosis Complex and Epilepsy

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Neurocutaneous Disorders

Abstract

Between 80% and 90% of patients with tuberous sclerosis complex (TSC) will suffer from various types of epileptic seizures early in life, which is one of the most frequent clinical manifestations of this congenital neurocutaneous multiorgan disease. Cortical tubers are the hallmark for TSC-associated epilepsy, present in almost 90% of the affected population. These tubers were traditionally considered inoperable, but advanced imaging techniques, the use of invasive EEG monitoring, and encouraging published data in infants and children with TSC over the last two decades have made perspectives on epilepsy surgery more optimistic. This chapter describes the different aspects of intractable epilepsy in the context of TSC, the selection process of identifying eligible candidates for a curative surgery, and the methods to localize the epileptogenic zone, which is crucial in obtaining a favorable postoperative outcome.

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Authors and Affiliations

  1. Division of Pediatric Neurosurgery, Department of Neurosciences, Rothschild Foundation Hospital, Paris, France

    Georg Dorfmüller & Martine Fohlen

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  1. Georg Dorfmüller
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  2. Martine Fohlen
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Correspondence to Georg Dorfmüller .

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Editors and Affiliations

  1. Department of Paediatric Neurology and Developmental Neurology, Aristotle University of Thessaloniki, Thessaloniki, Greece

    Christos P. Panteliadis

  2. Department of Neurology, University of Washington, Seattle, WA, USA

    Ramsis Benjamin

  3. Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany

    Christian Hagel

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Dorfmüller, G., Fohlen, M. (2022). Presurgical Evaluation of Children with Tuberous Sclerosis Complex and Epilepsy. In: Panteliadis, C.P., Benjamin, R., Hagel, C. (eds) Neurocutaneous Disorders. Springer, Cham. https://doi.org/10.1007/978-3-030-87893-1_49

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  • DOI: https://doi.org/10.1007/978-3-030-87893-1_49

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-87892-4

  • Online ISBN: 978-3-030-87893-1

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