Coexistent Ganglioglioma, Focal Cortical Dysplasia, and Hippocampal Sclerosis (Triple Pathology) in Chronic Epilepsy

Introduction: The most commonly identified pathologies in patients with medically intractable epilepsy include focal cortical dysplasia, hippocampal sclerosis, tumors and remote ischemic damage. Surgery has proven to be an effective therapeutic modality in the majority of such patients. The coexistence of multiple pathologies in resected tissues is well documented, particularly ganglioglioma and focal cortical dysplasia. Cases of triple pathology are, however, extraordinarily unusual. We report on two cases of triple pathology involving hippocampal sclerosis, ganglioglioma and focal cortical dysplasia. Methods: Cases of pathologically confirmed hippocampal sclerosis diagnosed between January 2000 and December 2012 (n = 349) were reviewed, and only two cases (0.6%) were identified with triple pathology. We are reviewing the histopathologic and clinical features of these two cases. Results: The patients were a 6-year-old girl and a 10-year-old boy. The girl presented with a 4-year history of epilepsy and oppositional defiant disorder. Imaging identified a lesion in the left parahippocampal gyrus and posterior hippocampus. The boy presented with an 8-year history of epilepsy, attention deficit hyperactivity disorder and a pervasive developmental disorder. Imaging identified a lesion in the left posterior temporal and occipital region. Both patients had had normal deliveries and no history of head trauma, infection or febrile seizures. Resected tissues in both patients showed a ganglioglioma (World Health Organization [WHO] grade I) with accompanying focal cortical dysplasia. The girl also had classic hippocampal sclerosis, and the boy had severe hippocampal sclerosis, according to International League Against Epilepsy (ILAE) classification. Both patients were seizure-free on antiepileptic medication at last follow-up at 20 and 38 months, respectively. Conclusion: The prevalence of tumor and hippocampal sclerosis is low (<1% in the current study). Surgical intervention for triple pathology cases anecdotally appears effective in attaining seizure control in pharmacoresistant patients.