title: [Gelastic seizures, precocious puberty and hypothalamic hamartomas. A case report and the contributions of Single Photon Emission Computed Tomography (SPECT)].
abstract: We present a patient with gelastic seizures, precocious puberty and a hypothalamic hamartoma. The diagnostic method of choice for hypothalamic hamartoma is new generation MRI. The characteristic MRI images along with lack of growth during the course of disease indicates a diagnosis of hamartoma firmly with no need for pathological studies. Although the physical nature of gelastic seizures in this syndrome is a subject of dispute, SPECT findings point to activity at a distance from nerve routes connecting the hypothalamus to the cortical regions (the temporal region in this case). Prognosis improves if the various components of the syndrome are treated early and when dysgenesis is less extensive.
authors.affiliation: Servico de Neurología, Hospital Universitario Virgen del Rocío, Sevilla.
authors.forename: V
authors.initials: V
authors.lastname: Acilona Echeverría
authors.forename: J L
authors.initials: JL
authors.lastname: Casado Chocán
authors.forename: J M
authors.initials: JM
authors.lastname: López Domínguez
authors.forename: J M
authors.initials: JM
authors.lastname: Aguilera Navarro
authors.forename: E
authors.initials: E
authors.lastname: Marqués Martín
authors.forename: C
authors.initials: C
authors.lastname: Muñoz Villa
mesh_terms: D000293:Adolescent; D004827:Epilepsy / Q000150:complications* / Q000188:drug therapy; D005260:Female; D006222:Hamartoma / Q000150:complications* / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D007031:Hypothalamus / Q000473:pathology*; D007845:Laughter; D008279:Magnetic Resonance Imaging; D010634:Phenobarbital / Q000627:therapeutic use; D010672:Phenytoin / Q000627:therapeutic use; D011629:Puberty, Precocious / Q000150:complications* / Q000175:diagnosis; D013702:Temporal Lobe / Q000000981:diagnostic imaging / Q000473:pathology; D015899:Tomography, Emission-Computed, Single-Photon
chemical_list: D010672:Phenytoin; D010634:Phenobarbital
vernacular_title: Síndrome de crisis gelásticas, pubertad precoz y hamartoma hipotalámico. Presentación de un caso y aportaciones del SPECT.