A review on the management of epilepsy associated with hypothalamic hamartomas

Childs Nerv Syst. 2009 Apr;25(4):423-32. doi: 10.1007/s00381-008-0798-y. Epub 2009 Jan 20.

Abstract

Introduction: Hypothalamic hamartomas are rare congenital malformations located in the region of the tuber cinereum and third ventricle. Patients may be asymptomatic, but the usual presentation is gelastic seizures, precocious puberty, and/or developmental delay.

Clinical presentation: Without surgical intervention, the gelastic seizures, which are typically present in childhood, may progress to other seizure types, including generalized epilepsy, and are generally refractory to antiepileptic drugs.

Summary: This review will discuss the clinical and electrophysiologic aspects of these lesions, as well as treatment options, including surgery, endoscopy, and radiosurgery.

Publication types

  • Review

MeSH terms

  • Behavior
  • Brachytherapy
  • Catheter Ablation
  • Cognition Disorders / complications
  • Endoscopy
  • Epilepsy / complications*
  • Epilepsy / pathology
  • Epilepsy / physiopathology
  • Epilepsy / therapy*
  • Hamartoma / complications*
  • Hamartoma / physiopathology
  • Hamartoma / therapy*
  • Humans
  • Hypothalamic Diseases / complications*
  • Hypothalamic Diseases / physiopathology
  • Hypothalamic Diseases / therapy
  • Hypothalamus / pathology
  • Hypothalamus / physiopathology*
  • Neurosurgery / methods
  • Radiosurgery
  • Seizures / complications
  • Seizures / pathology
  • Seizures / physiopathology
  • Vagus Nerve Stimulation