title: Hypothalamic hamartomas: seven cases and review of the literature.
abstract: Hypothalamic hamartomas constitute rare developmental lesions associated with gelastic epilepsy and/or precocious puberty (PP). We elected to review cases encountered at our center (7 patients) and the existing literature (277 patients) to obtain a better understanding of the clinical aspects, pathogenesis, and treatment of this entity. Evidence suggests that gelastic seizures are due to intrinsic epileptogenicity. The cause of the subsequent development of other seizure types, cognitive decline, and diffuse spike-and-wave pattern remains unresolved and is addressed. Anticonvulsants often fail to control seizures and different surgical options are available. Available evidence suggests that a resection through a subtemporal approach is best for lesions that are pedunculated or with a significant prepontine component, while a transcallosal approach is more appropriate for sessile lesions with an intraventricular component. Gamma knife surgery may be especially useful for small sessile lesions, failed partial resections, or patients not appropriate or refusing open surgery.
authors.affiliation: Department of Neurology, Yale University School of Medicine, New Haven, CT 06520-8018, USA.
authors.forename: Dang
authors.initials: D
authors.lastname: Nguyen
authors.forename: Sanjay
authors.initials: S
authors.lastname: Singh
authors.forename: Megdad
authors.initials: M
authors.lastname: Zaatreh
authors.forename: Edward
authors.initials: E
authors.lastname: Novotny
authors.forename: Susan
authors.initials: S
authors.lastname: Levy
authors.forename: Francine
authors.initials: F
authors.lastname: Testa
authors.forename: Susan S
authors.initials: SS
authors.lastname: Spencer
mesh_terms: D000293:Adolescent; D000328:Adult; D000927:Anticonvulsants / Q000627:therapeutic use; D001927:Brain Diseases / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology; D004569:Electroencephalography; D004828:Epilepsies, Partial / Q000188:drug therapy / Q000209:etiology; D005260:Female; D013007:Growth Hormone-Releasing Hormone / Q000627:therapeutic use; D006222:Hamartoma / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D006801:Humans; D007031:Hypothalamus / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008297:Male; D019635:Neurosurgical Procedures; D011629:Puberty, Precocious / Q000188:drug therapy / Q000209:etiology; D016634:Radiosurgery / Q000295:instrumentation; D014055:Tomography, Emission-Computed
chemical_list: D000927:Anticonvulsants; D013007:Growth Hormone-Releasing Hormone