Mechanisms of intrinsic epileptogenesis in human gelastic seizures with hypothalamic hamartoma

Jie Wu, Ming Gao, Jian Xin Shen, Shen Feng Qiu, John F. Kerrigan

Research output: Contribution to journalReview article

9 Scopus citations

Abstract

Human hypothalamic hamartoma (HH) is a rare developmental malformation often characterized by gelastic seizures, which are refractory to medical therapy. Ictal EEG recordings from the HH have demonstrated that the epileptic source of gelastic seizures lies within the HH lesion itself. Recent advances in surgical techniques targeting HH have led to dramatic improvements in seizure control, which further supports the hypothesis that gelastic seizures originate within the HH. However, the basic cellular and molecular mechanisms of epileptogenesis in this subcortical lesion are poorly understood. Since 2003, Barrow Neurological Institute has maintained a multidisciplinary clinical program to evaluate and treat patients with HH. This program has provided a unique opportunity to investigate the basic mechanisms of epileptogenesis using surgically resected HH tissue. The first report on the electrophysiological properties of HH neurons was published in 2005. Since then, ongoing research has provided additional insights into the mechanisms by which HH generate seizure activity. In this review, we summarize this progress and propose a cellular model that suggests that GABA-mediated excitation contributes to epileptogenesis in HH lesions.

Original languageEnglish (US)
Pages (from-to)104-111
Number of pages8
JournalCNS Neuroscience and Therapeutics
Volume21
Issue number2
DOIs
StatePublished - Feb 1 2015

Keywords

  • Epileptogenesis
  • Gelastic seizures
  • Human hypothalamic hamartoma

ASJC Scopus subject areas

  • Pharmacology
  • Psychiatry and Mental health
  • Physiology (medical)
  • Pharmacology (medical)

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