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The clinicopathologic spectrum of focal cortical dysplasias: a consensus classification proposed by an ad hoc Task Force of the ILAE Diagnostic Methods Commission.

Ingmar Blümcke 1 Maria Thom 2 Eleonora Aronica 3 Dawna D. Armstrong 4 Harry V. Vinters 5 Andre Palmini 6 Thomas S. Jacques 7 Giuliano Avanzini 8 James A. Barkovich 9 Giorgio Battaglia 10 Albert J. Becker 11 Carlos Cepeda 12 Fernando Cendes 13 Nadia Colombo 14 Crino Peter 15 Helen J. Cross 16 Olivier Delalande 17 François Dubeau 18 John Duncan 19 Renzo Guerrini 20 Philippe Kahane 21, 22 Gary Mathern 23 Imad Najm 24 Ciğdem Ozkara 25 Charles Raybaud 26 Alfonso Represa 27 Steven N. Roper 28 Noriko Salamon 29 Andreas Schulze-Bonhage 30 Laura Tassi 31 Annamaria Vezzani 32 Roberto Spreafico 8 
Abstract : PURPOSE:   Focal cortical dysplasias (FCD) are localized regions of malformed cerebral cortex and are very frequently associated with epilepsy in both children and adults. A broad spectrum of histopathology has been included in the diagnosis of FCD. An ILAE task force proposes an international consensus classification system to better characterize specific clinicopathological FCD entities. METHODS: Thirty-two Task Force members have reevaluated available data on electroclinical presentation, imaging, neuropathological examination of surgical specimens as well as postsurgical outcome. KEY FINDINGS: The ILAE Task Force proposes a three-tiered classification system. FCD Type I refers to isolated lesions, which present either as radial (FCD Type Ia) or tangential (FCD Type Ib) dyslamination of the neocortex, microscopically identified in one or multiple lobes. FCD Type II is an isolated lesion characterized by cortical dyslamination and dysmorphic neurons without (Type IIa) or with balloon cells (Type IIb). Hence, the major change since a prior classification represents the introduction of FCD Type III, which occurs in combination with hippocampal sclerosis (FCD Type IIIa), or with epilepsy-associated tumors (FCD Type IIIb). FCD Type IIIc is found adjacent to vascular malformations, whereas FCD Type IIId can be diagnosed in association with epileptogenic lesions acquired in early life (i.e., traumatic injury, ischemic injury or encephalitis). SIGNIFICANCE: This three-tiered classification system will be an important basis to evaluate imaging, electroclinical features, and postsurgical seizure control as well as to explore underlying molecular pathomechanisms in FCD.
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Submitted on : Wednesday, May 11, 2011 - 1:06:32 PM
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Ingmar Blümcke, Maria Thom, Eleonora Aronica, Dawna D. Armstrong, Harry V. Vinters, et al.. The clinicopathologic spectrum of focal cortical dysplasias: a consensus classification proposed by an ad hoc Task Force of the ILAE Diagnostic Methods Commission.. Epilepsia, Wiley, 2011, 52 (1), pp.158-74. ⟨10.1111/j.1528-1167.2010.02777.x⟩. ⟨inserm-00592131⟩



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