Hypothalamic hamartoma, gelastic epilepsy, precocious puberty--a diffuse cerebral dysgenesis.
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.
Gulati
Sheffali
S
Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
Gera
Sameer
S
Menon
P S N
PS
Kabra
Madhulika
M
Kalra
Veena
V
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
D008186:Luteolytic Agents; D017329:Triptorelin Pamoate; D007987:Gonadotropin-Releasing Hormone