title: Hypothalamic hamartomas. Part 2. Surgical considerations and outcome.
abstract: Hypothalamic hamartomas are uncommon developmental heterotopic masses composed of an intermixed array of neurons, glia, and myelinated fibers closely associated with the mammillary bodies. Gelastic seizures, the hallmark feature of hypothalamic hamartomas, commonly present in early childhood. However, patients usually also display a disabling clinical syndrome, which may include various other types of refractory seizures with secondary generalization together with progressive cognitive, behavioral, and psychiatric dysfunction. The hamartoma itself has been unequivocally shown to be intrinsically epileptogenic. Over the past 2 decades there has been considerable effort to develop neurosurgical techniques to treat the epileptic syndrome effectively as well as to improve the neurocognitive and behavioral outcome.
authors.affiliation: Department of Neurosurgery, Comprehensive Epilepsy Center, Wayne State University, Detroit Medical Center, Detroit, MI 48201, USA. smittal@med.wayne.edu
authors.forename: Sandeep
authors.initials: S
authors.lastname: Mittal
authors.forename: Monika
authors.initials: M
authors.lastname: Mittal
authors.forename: José Luis
authors.initials: JL
authors.lastname: Montes
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Farmer
authors.forename: Frederick
authors.initials: F
authors.lastname: Andermann
mesh_terms: D002675:Child, Preschool; D046690:Deep Brain Stimulation; D019723:Endoscopes; D006222:Hamartoma / Q000150:complications* / Q000628:therapy*; D006801:Humans; D007027:Hypothalamic Diseases / Q000150:complications* / Q000628:therapy*; D008279:Magnetic Resonance Imaging; D008297:Male; D019635:Neurosurgical Procedures / Q000379:methods*; D016634:Radiosurgery; D012640:Seizures / Q000209:etiology*; D016896:Treatment Outcome; D055536:Vagus Nerve Stimulation