Review: The international consensus classification of Focal Cortical Dysplasia - a critical update 2018

Najm IM, Sarnat HB, Blümcke I (2018)

Publication Type: Journal article, Review article

Publication year: 2018

Journal

Neuropathology and Applied Neurobiology Wiley-Blackwell

Book Volume: 44

Pages Range: 18-31

Journal Issue: 1

DOI: 10.1111/nan.12462

Abstract

The Diagnostic Methods commission of the International League against Epilepsy (ILAE) released a first international consensus classification of Focal Cortical Dysplasia (FCD) in 2011. Since that time, this FCD classification has been widely used in clinical diagnosis and research (more than 740 papers cited in Pubmed between 1/1/2012 and 7/1/2017). Herein, we review the new data that will inform and revise the FCD classification. Many recent papers described molecular-genetic characteristics in FCD type II including multiple mutations in the mTOR pathway. In addition, the electro-clinico-imaging phenotype and surgical outcomes of FCD type II (in particular type IIb) were further defined and validated. These results pave the way for the design of an integrated clinico-pathological and genetic classification system, as recently recommended by the WHO for the classification of malignant brain tumours. On the other hand, little new information was acquired on FCD types I and III. Focal cortical dysplasia type I subtypes are still lacking a comprehensive description of clinical phenotypes, reproducible imaging characteristics, and specific molecular/genetic biomarkers. Associated FCD III subtypes also became rare in published literature. Despite temporal lobe epilepsy being the most common focal epilepsy in adults, we have not identified neurophysiological, imaging, histopathological and/or genetic biomarkers to reliably classify FCD III with or without hippocampal sclerosis. In respect of pathogenesis, FCD adjacent to a non-developmental, postnatally acquired lesion is difficult to explain and perhaps does not exist. This update may help foster shared efforts towards a better understanding of FCD, potential future updates of classification and novel targeted treatments.

Authors with CRIS profile

Ingmar Blümcke Lehrstuhl für Neuropathologie

Involved external institutions

University of Calgary CA Canada (CA) Cleveland Clinic US United States (USA) (US)

How to cite

APA:

Najm, I.M., Sarnat, H.B., & Blümcke, I. (2018). Review: The international consensus classification of Focal Cortical Dysplasia - a critical update 2018. Neuropathology and Applied Neurobiology, 44(1), 18-31. https://dx.doi.org/10.1111/nan.12462

MLA:

Najm, I. M., H. B. Sarnat, and Ingmar Blümcke. "Review: The international consensus classification of Focal Cortical Dysplasia - a critical update 2018." Neuropathology and Applied Neurobiology 44.1 (2018): 18-31.

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