Current Review in Basic Science: Animal Models of Focal Cortical Dysplasia and Epilepsy
- PMID: 36187145
- PMCID: PMC9483763
- DOI: 10.1177/15357597221098230
Current Review in Basic Science: Animal Models of Focal Cortical Dysplasia and Epilepsy
Abstract
Focal cortical dysplasia (FCD) is a malformation of cortical development that is a prevalent cause of intractable epilepsy in children. Of the three FCD subtypes, understanding the etiology and pathogenesis of FCD type II has seen the most progress owing to the recent advances in identifying gene mutations along the mTOR signaling pathway as a frequent cause of this disorder. Accordingly, numerous animal models of FCD type II based on genetic manipulation of the mTOR signaling pathway have emerged to investigate the mechanisms of epileptogenesis and novel therapeutics for epilepsy. These include transgenic and in utero electroporation-based animal models. Here, we review the histopathological and electroclinical features of existing FCD type II animal models and discuss the scientific and technical considerations, clinical applications, and limitations of current models. We also highlight other models of FCD based on early life acquired factors.
Keywords: CRISPR/Cas9; astrocytes; cortical development; cortical lesion; in utero electroporation; malformation of cortical development; pyramidal neurons; seizure; transgenic mice.
© The Author(s) 2022.
Conflict of interest statement
Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
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