Focal cortical dysplasia: Molecular disturbances and clinicopathological classification (Review)

  • Authors:
    • Monika Siedlecka
    • Wiesława Grajkowska
    • Ryszard Galus
    • Bożenna Dembowska-Bagińska
    • Jarosław Jóźwiak
  • View Affiliations

  • Published online on: September 29, 2016     https://doi.org/10.3892/ijmm.2016.2760
  • Pages: 1327-1337
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Abstract

Focal cortical dysplasia (FCD) is one of the most important causes of drug-resistant epilepsy in paediatric patients, particularly in those below the age of 3. Even though over 40 years have passed since the first description of the entity by Taylor, the exact mechanisms causing these cortical abnormalities remain unelucidated. In this review, we summarise the current knowledge on clinical and histopathological aspects, taking into account the new classification system proposed by the International League Against Epilepsy. We focus on the clinicopathological associations and differences in post-surgical outcome among FCD subtypes, in particular isolated FCD vs. FCD associated with principal lesions, which have not been summarised to date. We also recapitulate genetic studies, pointing to the possible mechanisms of the cortical dysregulation and drug resistance, and summarise novel factors which may contribute to epileptogenesis in FCD. Furthermore, we compare FCD type IIB (FCDIIB) with brain tumours found in a neurocutaneous disorder, tuberous sclerosis, as we evaluate the hypothesis that FCD IIB may be a local form of this disease.
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November-2016
Volume 38 Issue 5

Print ISSN: 1107-3756
Online ISSN:1791-244X

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Spandidos Publications style
Siedlecka M, Grajkowska W, Galus R, Dembowska-Bagińska B and Jóźwiak J: Focal cortical dysplasia: Molecular disturbances and clinicopathological classification (Review). Int J Mol Med 38: 1327-1337, 2016
APA
Siedlecka, M., Grajkowska, W., Galus, R., Dembowska-Bagińska, B., & Jóźwiak, J. (2016). Focal cortical dysplasia: Molecular disturbances and clinicopathological classification (Review). International Journal of Molecular Medicine, 38, 1327-1337. https://doi.org/10.3892/ijmm.2016.2760
MLA
Siedlecka, M., Grajkowska, W., Galus, R., Dembowska-Bagińska, B., Jóźwiak, J."Focal cortical dysplasia: Molecular disturbances and clinicopathological classification (Review)". International Journal of Molecular Medicine 38.5 (2016): 1327-1337.
Chicago
Siedlecka, M., Grajkowska, W., Galus, R., Dembowska-Bagińska, B., Jóźwiak, J."Focal cortical dysplasia: Molecular disturbances and clinicopathological classification (Review)". International Journal of Molecular Medicine 38, no. 5 (2016): 1327-1337. https://doi.org/10.3892/ijmm.2016.2760