Hypothalamic hamartoma, gelastic epilepsy, precocious puberty--a diffuse cerebral dysgenesis

Brain Dev. 2002 Dec;24(8):784-6. doi: 10.1016/s0387-7604(02)00096-7.

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.

Publication types

  • Case Reports

MeSH terms

  • Agenesis of Corpus Callosum
  • Cerebral Cortex / abnormalities*
  • Cerebral Cortex / pathology
  • Cerebral Cortex / physiopathology
  • Corpus Callosum / pathology
  • Corpus Callosum / physiopathology
  • Dandy-Walker Syndrome / etiology
  • Dandy-Walker Syndrome / pathology
  • Dandy-Walker Syndrome / physiopathology
  • Epilepsies, Partial / etiology*
  • Epilepsies, Partial / pathology
  • Epilepsies, Partial / physiopathology
  • Gonadotropin-Releasing Hormone / antagonists & inhibitors
  • Gonadotropin-Releasing Hormone / metabolism
  • Hamartoma / complications*
  • Hamartoma / pathology
  • Hamartoma / physiopathology
  • Humans
  • Hypothalamic Neoplasms / complications*
  • Hypothalamic Neoplasms / pathology
  • Hypothalamic Neoplasms / physiopathology
  • Infant
  • Luteolytic Agents / therapeutic use
  • Magnetic Resonance Imaging
  • Male
  • Nervous System Malformations / complications*
  • Nervous System Malformations / pathology
  • Nervous System Malformations / physiopathology
  • Puberty, Precocious / etiology*
  • Puberty, Precocious / pathology
  • Puberty, Precocious / physiopathology
  • Treatment Outcome
  • Triptorelin Pamoate / therapeutic use

Substances

  • Luteolytic Agents
  • Triptorelin Pamoate
  • Gonadotropin-Releasing Hormone