Hypothalamic hamartomas: With special reference to gelastic epilepsy and surgery

José Manuel Valdueza, Loris Cristante, Olaf Dammann, Karl Bentele, Alexander Vortmeyer, Wolfgang Saeger, Barbara Padberg, Jörg Freitag, Hans Dietrich Herrmann

Research output: Contribution to journalArticlepeer-review

219 Scopus citations


THIS STUDY PRESENTS six patients with hypothalamic hamartomas diagnosed on the basis of magnetic resonance imaging. Histological confirmation was performed in three patients who underwent surgery. Immunohistological assays were used to determine the neurosecretory pattern. Four patients presented with epilepsy, including gelastic seizures. Other symptoms included behavioral abnormalities in four patients and precocious puberty and visual impairment in two patients. One patient presented associated developmental defects. Good results without morbidity were achieved with surgical resectioning in two patients with large hamartomas associated with behavioral abnormalities and gelastic epilepsy that was unresponsive to conventional medical treatment and in one patient with visual impairment. We propose a classification of the hypothalamic hamartomas based on topographical and clinical data obtained from 36 selected cases in the literature and six of our own cases. This classification should help to classify the various treatment methods and the surgical risks into four subgroups (Types Ia, Ib, IIa, and IIb). We conclude that the surgical approach is a realistic alternative in certain cases, including large and broad-based Type IIb hamartomas associated with gelastic epilepsy and behavioral disorders.

Original languageEnglish (US)
Pages (from-to)949-958
Number of pages10
Issue number6
StatePublished - Jun 1994
Externally publishedYes


  • Behavior disorder
  • Gelastic epilepsy
  • Hypothalamic hamartoma
  • Immunohistochemistry
  • Magnetic resonance imaging
  • Precocious puberty

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

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