Role of the hypothalamic hamartoma in the genesis of gelastic fits (a video-stereo-EEG study)

C. Munari, P. Kahane, S. Francione, D. Hoffmann, L. Tassi, R. Cusmai, F. Vigevano, B. Pasquier, O. O. Betti

Research output: Contribution to journalArticlepeer-review

Abstract

Patients having a hypothalamic hamartoma frequently present epileptic attacks of laughter, and they later experience multiple additional seizure types, which invariably lead to a severe drug-resistant epilepsy. If this association is now well-known, relationships between the hypothalamic mass and the different types of seizures remain still mysterious. We report the case of a 16-year-old girl suffering from this peculiar epileptic picture, in whom a stereo-EEG study was performed, allowing us to record both the hamartoma, the neighboring hypothalamic structures, and other bilateral cortical areas. It showed that gelastic fits were strictly linked to ictal discharges which began and remained well localized in the hamartoma. Conversely, atonic seizures, which might result from a secondary epileptogenesis, admitted a widely extended bilateral frontal cortical origin, sparing the lesion, and slightly involving the posterior hypothalamus. Stereotactic radiosurgery of the hamartoma proved to be ineffective on both types of seizures, probably because of the too low dose of X-rays delivered (18 grays), as suggested by the absence of hypothalamic mass changes on MRI. Such data, never reported to our knowledge, seem able to contribute to a better understanding of this very peculiar epileptic syndrome, and perhaps to a better adapted therapeutic management.

Original languageEnglish
Pages (from-to)154-160
Number of pages7
JournalElectroencephalography and Clinical Neurophysiology
Volume95
Issue number3
DOIs
Publication statusPublished - 1995

Keywords

  • Atonic seizures
  • Depth electrodes
  • Epilepsy surgery
  • Gelastic seizures
  • Hypothalamic hamartoma
  • Stereotactic biopsy

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

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