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- James L Frazier, C Rory Goodwin, Edward S Ahn, and George I Jallo.
- Department of Neurosurgery, Meyer 8-161, Johns Hopkins Hospital, 600 North Wolfe Street, Baltimore, MD 21287, USA. jfrazier@jhmi.edu
- Childs Nerv Syst. 2009 Apr 1;25(4):423-32.
IntroductionHypothalamic hamartomas are rare congenital malformations located in the region of the tuber cinereum and third ventricle. Patients may be asymptomatic, but the usual presentation is gelastic seizures, precocious puberty, and/or developmental delay.Clinical PresentationWithout surgical intervention, the gelastic seizures, which are typically present in childhood, may progress to other seizure types, including generalized epilepsy, and are generally refractory to antiepileptic drugs.SummaryThis review will discuss the clinical and electrophysiologic aspects of these lesions, as well as treatment options, including surgery, endoscopy, and radiosurgery.
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