Hypothalamic hamartomas: Seven cases and review of the literature

Dang Nguyen, Sanjay Singh, Megdad Zaatreh, Edward Novotny, Susan Levy, Francine Testa, Susan S. Spencer

Research output: Contribution to journalArticle

80 Scopus citations

Abstract

Hypothalamic hamartomas constitute rare developmental lesions associated with gelastic epilepsy and/or precocious puberty (PP). We elected to review cases encountered at our center (7 patients) and the existing literature (277 patients) to obtain a better understanding of the clinical aspects, pathogenesis, and treatment of this entity. Evidence suggests that gelastic seizures are due to intrinsic epileptogenicity. The cause of the subsequent development of other seizure types, cognitive decline, and diffuse spike-and-wave pattern remains unresolved and is addressed. Anticonvulsants often fail to control seizures and different surgical options are available. Available evidence suggests that a resection through a subtemporal approach is best for lesions that are pedunculated or with a significant prepontine component, while a transcallosal approach is more appropriate for sessile lesions with an intraventricular component. Gamma knife surgery may be especially useful for small sessile lesions, failed partial resections, or patients not appropriate or refusing open surgery.

Original languageEnglish (US)
Pages (from-to)246-258
Number of pages13
JournalEpilepsy and Behavior
Volume4
Issue number3
DOIs
StatePublished - Jun 2003
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Neurology
  • Clinical Neurology
  • Behavioral Neuroscience

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    Nguyen, D., Singh, S., Zaatreh, M., Novotny, E., Levy, S., Testa, F., & Spencer, S. S. (2003). Hypothalamic hamartomas: Seven cases and review of the literature. Epilepsy and Behavior, 4(3), 246-258. https://doi.org/10.1016/S1525-5050(03)00086-6