Focal cortical dysplasia: etiology, epileptogenesis, classification, clinical presentation, imaging, and management
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REVIEW ARTICLE
Focal cortical dysplasia: etiology, epileptogenesis, classification, clinical presentation, imaging, and management Alican Tahta 1 & Mehmet Turgut 2,3 Received: 5 April 2020 / Accepted: 30 July 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Background Focal cortical dysplasia (FCD) is the most prevalent cause of intractable epilepsy in children. It was first described by Taylor et al. in 1971. In 2011, the International League against Epilepsy described an international consensus of classification for FCD. However, the exact mechanism causing this pathology remains unclear. The diagnosis and recognition of FCD increase with the advances in neuroradiology and electrophysiology. Focus of review In this paper, we discuss the literature regarding management of FCD with a focus on etiology, pathophysiology, classification, clinical presentation, and imaging modalities. We will also discuss certain variables affecting surgical outcome of patients with FCD. Conclusion Based on our review findings, it is concluded that surgical management with complete resection of the lesion following preoperative localization of the epileptogenic zone in patients with FCD subtypes can provide a seizure-free outcome. Keywords Epilepsy . Focal cortical dysplasia. Outcome . Surgery
Introduction The most prevalent cause of anti-epileptic resistant focal epilepsy is focal cortical dysplasia (FCD) in children [7]. FCD, which was described by Taylor et al. in 1971, is characterized by deranged neurons in white matter, dyslamination, and abnormal balloon cells [68]. Approximately one-half (46.5%) of drug-resistant epileptic patients has some form of this pathology [10]. Complications such as deterioration, autism, and motor dysfunction can be seen secondary to epilepsy, and may be partially reversible with the remission of the seizures [40]. Thus, children with FCD need effective surgical treatment for better prognosis.
* Mehmet Turgut [email protected] Alican Tahta [email protected] 1
Department of Neurosurgery, Istanbul Medipol University School of Medicine, Istanbul, Turkey
2
Department of Neurosurgery, Aydın Adnan Menderes University School of Medicine, Efeler, Aydın, Turkey
3
Department of Histology and Embryology, Aydın Adnan Menderes University Health Sciences Institute, Efeler, Aydın, Turkey
In this study, we will review etiology, pathophysiology, classification, clinical presentation, imaging findings, and variables affecting surgical outcome of children with FCD.
Etiology Environmental factors, such as perinatal/postnatal injury, viral infections, and also genetic factors are believed to be the causes of the disruption of cortical formation in utero which result in FCD [5]. There are two new theories about the etiology of FCD. Some authors believe that FCD is a form of tuberous sclerosis without any extracranial manifestations and that the gene commonly found in tuberous sclerosis syndrome, TSC1, is responsible for the formation of focal dysplasia [28]. Another theory about the FC
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