title: Coexistent ganglioglioma, focal cortical dysplasia, and hippocampal sclerosis (triple pathology) in chronic epilepsy.
abstract: 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 most 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 2 cases of triple pathology including hippocampal sclerosis, ganglioglioma, and focal cortical dysplasia. Cases of pathologically confirmed hippocampal sclerosis diagnosed between January 2000 to December 2012 (n= 349) were reviewed, and only 2 cases (0.6%) with triple pathology were identified. The histopathologic and clinical features of these 2 cases are reviewed. The patients included a 6-year-old girl and 10-year-old boy. The former patient 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 latter patient presented with an 8-year history of epilepsy, attention deficient hyperactivity disease, and a pervasive developmental disorder. Imaging identified a lesion in the left posterior temporal and occipital region. Resected tissues in both patients showed a ganglioglioma (World Health Organization grade I) with accompanying focal cortical dysplasia and hippocampal sclerosis. Both patients were seizure free on antiepileptic medication at last follow-up at 20 and 38 months, respectively. The prevalence of triple pathology including hippocampal sclerosis is low (<1% in the current study). Surgical intervention for triple pathology cases anecdotally appears effective in achieving seizure control.
authors.affiliation: Cleveland Clinic Department of Anatomic Pathology and Cleveland Clinic Lerner College of Medicine, Cleveland, OH. Electronic address: praysor@ccf.org.
authors.forename: Richard A
authors.initials: RA
authors.lastname: Prayson
authors.affiliation: Cleveland Clinic Department of Anatomic Pathology and Cleveland Clinic Lerner College of Medicine, Cleveland, OH.
authors.forename: Jordan M
authors.initials: JM
authors.lastname: Gales
mesh_terms: D002540:Cerebral Cortex / Q000473:pathology / Q000601:surgery; D002648:Child; D004827:Epilepsy / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D005260:Female; D018303:Ganglioglioma / Q000473:pathology* / Q000601:surgery; D006624:Hippocampus / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008297:Male; D054220:Malformations of Cortical Development / Q000473:pathology / Q000601:surgery; D065703:Malformations of Cortical Development, Group I / Q000473:pathology* / Q000601:surgery; D012598:Sclerosis / Q000473:pathology / Q000601:surgery; D016896:Treatment Outcome
keywords: Epilepsy; Focal cortical dysplasia; Ganglioglioma; Hippocampal sclerosis; Triple pathology