title: Hypothalamic hamartoma, gelastic epilepsy, precocious puberty--a diffuse cerebral dysgenesis.
abstract: Childhood epileptic syndrome characterized by early onset gelastic seizures, hypothalamic hamartoma and precocious puberty is well recognized though rare. We report association of agenesis of corpus callosum, Dandy-Walker complex and heterotopic gray matter with this childhood epileptic syndrome which is hitherto an unreported association. The child showed a satisfactory response to gonadotropin releasing hormone agonist.
authors.affiliation: Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Sheffali
authors.initials: S
authors.lastname: Gulati
authors.forename: Sameer
authors.initials: S
authors.lastname: Gera
authors.forename: P S N
authors.initials: PS
authors.lastname: Menon
authors.forename: Madhulika
authors.initials: M
authors.lastname: Kabra
authors.forename: Veena
authors.initials: V
authors.lastname: Kalra
mesh_terms: D061085:Agenesis of Corpus Callosum; D002540:Cerebral Cortex / Q000002:abnormalities* / Q000473:pathology / Q000503:physiopathology; D003337:Corpus Callosum / Q000473:pathology / Q000503:physiopathology; D003616:Dandy-Walker Syndrome / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D004828:Epilepsies, Partial / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D007987:Gonadotropin-Releasing Hormone / Q000037:antagonists & inhibitors / Q000378:metabolism; D006222:Hamartoma / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D007029:Hypothalamic Neoplasms / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D007223:Infant; D008186:Luteolytic Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D009421:Nervous System Malformations / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D011629:Puberty, Precocious / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D016896:Treatment Outcome; D017329:Triptorelin Pamoate / Q000627:therapeutic use
chemical_list: D008186:Luteolytic Agents; D017329:Triptorelin Pamoate; D007987:Gonadotropin-Releasing Hormone