Large Epileptogenic Type IIIb Dysplasia: A Radiological and Anatomopathological Challenge.

摘要: Background Type IIIb dysplasia is a subtype of focal cortical associated with tumor, most frequently gangliogliomas then dysembryoplastic neuroepithelial tumors (DNETs). Their preoperative diagnosis often remains equivocal since specific features are missing. The functional results (i.e., seizure free) good 81%–87% Engel Ia at 5-year follow-up. Case Description A 4-year-old boy presented 1-year history severe, invalidating, drug-resistant epilepsy. Imaging workup demonstrated huge left limbic lesion, which remained speculative. Because worsening neurological status, resective surgery was recommended after multidisciplinary discussion. resection performed through transtemporal approach under neuronavigation (C.R.). Postoperative magnetic resonance imaging assessed uncomplicated near-total resection. Histopathological analysis showed combined DNET nonspecific type and dysplasia. Conclusion We describe rare condition combining DNET. Preoperative the lesion utmost difficultly, thereby rendering mandatory thorough histopathological examination resected specimen in vast majority cases. Increased recognition brings up hypothesis genetic continuum or linkage between 2 conditions. Functional on activity ablative maximal safe should be goal.