title: Hypothalamic hamartomas. Part 1. Clinical, neuroimaging, and neurophysiological characteristics.
abstract: Hypothalamic hamartomas are uncommon but well-recognized developmental malformations that are classically associated with gelastic seizures and other refractory seizure types. The clinical course is often progressive and, in addition to the catastrophic epileptic syndrome, patients commonly exhibit debilitating cognitive, behavioral, and psychiatric disturbances. Over the past decade, investigators have gained considerable knowledge into the pathobiological and neurophysiological properties of these rare lesions. In this review, the authors examine the causes and molecular biology of hypothalamic hamartomas as well as the principal clinical features, neuroimaging findings, and electrophysiological characteristics. The diverse surgical modalities and strategies used to manage these difficult lesions are outlined in the second article of this 2-part review.
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: D058256:Brain Waves / Q000502:physiology*; D004569:Electroencephalography; D004827:Epilepsy / Q000209:etiology*; D006222:Hamartoma / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D007027:Hypothalamic Diseases / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D015225:Magnetoencephalography; D009419:Nerve Tissue Proteins / Q000378:metabolism; D059906:Neuroimaging
chemical_list: D009419:Nerve Tissue Proteins