title: Hypothalamic hamartoma: is the epileptogenic zone always hypothalamic? Arguments for independent (third stage) secondary epileptogenesis.
abstract: Gelastic seizures associated with hypothalamic hamartomas (HHs) are a clinicoradiologic syndrome presenting with a variety of symptoms, including pharmacoresistant epilepsy with multiple seizure types, electroencephalography (EEG) abnormalities, precocious puberty, behavioral disturbances, and progressive cognitive deterioration. Surgery in adults provides seizure freedom in only one third of patients. The poor results of epilepsy surgery could be explained by an extrahypothalamic epileptogenic zone. The existence of an independent, secondary epileptogenic area with persistent seizures after resection of the presumably primary lesion supports the concept of a "hypothalamic plus" epilepsy. "Hypothalamic plus" epilepsy could be related to either an extrahypothalamic structural lesion (visible on magnetic resonance imaging [MRI] or on neuropathology) or if the former is absent, to a functional alteration with enhanced epileptogenic properties due to a process termed secondary epileptogenesis. We report two patients with gelastic seizures with HH (gelastic seizures isolated or associated with dyscognitive seizures of temporal origin). Both patients underwent two-step surgery: first an endoscopic resection of the HH, followed at a later time by temporal lobectomy. Both patients became seizure-free only after the temporal lobectomy. In both cases, neuropathology failed to demonstrate a significant structural lesion in the temporal lobe. To our knowledge, for the first time, these two cases suggest the existence of independent secondary epileptogenesis in humans.
authors: Medical and Surgical Epilepsy Unit, University of Strasbourg, Strasbourg, France; Kork, Epilepsy Center, Kehl-Kork, Germany.
authors: Julia
authors: J
authors: Scholly
authors: Maria-Paola
authors: MP
authors: Valenti
authors: Anke M
authors: AM
authors: Staack
authors: Karl
authors: K
authors: Strobl
authors: Thomas
authors: T
authors: Bast
authors: Pierre
authors: P
authors: Kehrli
authors: Bernhard J
authors: BJ
authors: Steinhoff
authors: Edouard
authors: E
authors: Hirsch
mesh_terms: D000328:Adult; D004569:Electroencephalography; D004828:Epilepsies, Partial / Q000150:complications* / Q000601:surgery; D019788:Fluorodeoxyglucose F18; D006222:Hamartoma / Q000150:complications*; D006801:Humans; D007027:Hypothalamic Diseases / Q000150:complications*; D007031:Hypothalamus / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011877:Radionuclide Imaging; D013702:Temporal Lobe / Q000000981:diagnostic imaging; D020542:Third Ventricle / Q000000981:diagnostic imaging
chemical_list: D019788:Fluorodeoxyglucose F18
keywords: Epilepsy; Hypothalamic hamartoma; Secondary epileptogenesis; Surgery