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Cerebral cavernous malformations in the setting of focal epilepsies: pathological findings, clinical characteristics, and surgical treatment principles

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Abstract

Cavernous cerebral malformations (CCMs) are a well-defined epilepsy-associated pathology. They represent lesions/conglomerates of abnormally configured vessels leading to seizures either as a result of physiological changes affecting the cerebral cortex immediately surrounding the CCM (an epileptogenic mechanism that is relevant for both temporal and extratemporal lesions), or as a result of promoting epileptogenicity in remote but anatomo-functionally connected brain regions (a mechanism that is particularly relevant for temporal lobe lesions). This review details the pathological findings in CCMs and discusses the mechanisms of epileptogenicity in this context. The bulk of the review will focus on therapeutic strategies. Medical therapy using antiepileptic drugs is recommended as a first-line therapy, but surgical removal of the CCM with the surrounding cortex should be pursued if seizures prove to be drug resistant. Early timing of the resection and complete removal of any associated epileptic pathology are critical for best outcomes. In addition to reviewing the available data from prior series, we present original research from two specialized epilepsy centers targeted at answering particularly pressing clinical questions mainly related to the ideal timing and extent of surgery. Further research is needed to define the best surgical strategies in patients with temporal lobe CCMs and structurally normal hippocampi.

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Authors and Affiliations

  1. Epilepsy Center, Cleveland Clinic Neurological Institute, Cleveland, OH, 44195, USA

    Lara E. Jehi & Usha Aryal

  2. Faculty of Medicine, Pontificia Universidade Catolica Do Rio Grande do Sul (PUCRS), Porto Alegre Epilepsy Surgery Program, Hospital Sao Lucas (PUCRS), Porto Alegre, Brazil

    Andre Palmini & Eliseu Paglioli

  3. Department of Neuropathology, University Hospital Erlangen, 91054, Erlangen, Germany

    Roland Coras

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  1. Lara E. Jehi
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  2. Andre Palmini
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Correspondence to Lara E. Jehi.

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Jehi, L.E., Palmini, A., Aryal, U. et al. Cerebral cavernous malformations in the setting of focal epilepsies: pathological findings, clinical characteristics, and surgical treatment principles. Acta Neuropathol 128, 55–65 (2014). https://doi.org/10.1007/s00401-014-1294-y

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  • DOI: https://doi.org/10.1007/s00401-014-1294-y

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