Hypothalamic hamartoma

Jeffrey V. Rosenfeld, A. Simon Harvey

Research output: Chapter in Book/Report/Conference proceedingChapter (Book)Researchpeer-review

Abstract

Hypothalamic hamartoma (HH) is a rare developmental tumor which has a benign histology and does not progressively enlarge. Pedunculated HH attached to the tuber cinereum may cause precocious puberty whereas sessile HH which have intraventricular and intrahypothalamic components cause gelastic epilepsy and behavioral disorders. Large HH with involvement of both mammillary and tuberal regions tend to manifest with neurologic and endocrine problems [6,7,9]. Gelastic epilepsy is generally resistant to antiepileptic drugs (AED), ketogenic diet, and vagal nerve stimulation. The epilepsy syndrome that results may be progressive and devastating for the patient and their family. The localization of seizures can be deceptive, with some patients having had temporal lobectomy or frontal corticectomy without any effect on the seizures [3,6,17]. There is now good evidence that the epilepsy in these patients emanates from the HH [3].

Original languageEnglish
Title of host publicationNeuro-Oncology of CNS Tumors
Place of PublicationGermany
PublisherSpringer
Chapter33
Pages443-452
Number of pages10
ISBN (Electronic)9783540312604
ISBN (Print)3540258337, 9783540258339
DOIs
Publication statusPublished - 2006
Externally publishedYes
  • Hypothalamic Hamartoma

    Rosenfeld, J. V. & Harvey, A. S., 2010, Oncology of CNS Tumors. Tonn, J-C., Westphal, M. & Rutka, J. T. (eds.). 2nd ed. Germany: Springer, p. 491-502 12 p.

    Research output: Chapter in Book/Report/Conference proceedingChapter (Book)Other

    2 Citations (Scopus)

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