Few isolated neurons in hypothalamic hamartomas may cause gelastic seizures

Ben Waldau, Roger E. McLendon, Herbert E. Fuchs, Timothy M. George, Gerald A. Grant

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

Hypothalamic hamartomas (HHs) are congenital, benign masses in the hypothalamus and tuber cinereum that may cause central precocious puberty and gelastic seizures. Nodules of small neurons are thought to be a universal feature of the microarchitecture of HH lesions associated with epilepsy. Here we describe the case of a 5-year-old boy with gelastic seizures who underwent resection of a HH that contained nodules of glial cells, but only few, randomly distributed neurons. HHs that contain few or no neurons have only been reported thus far in cases associated with precocious puberty. This case demonstrates that few solitary neurons in HHs can drive the development of gelastic seizures, and nodules of small neurons may not be a universal feature of HHs associated with epilepsy. This finding is clinically important since hypothalamic hamartomas with rare neurons can easily be misdiagnosed as pilocytic astrocytomas or subependymomas if their presence is overlooked. A neuronal stain is helpful in making the correct diagnosis in these cases.

Original languageEnglish (US)
Pages (from-to)225-229
Number of pages5
JournalPediatric Neurosurgery
Volume45
Issue number3
DOIs
StatePublished - Jul 2009
Externally publishedYes

Fingerprint

Laughter
Seizures
Neurons
Tuber Cinereum
Epilepsy
Subependymal Glioma
Precocious Puberty
Astrocytoma
Hypothalamic hamartomas
Diagnostic Errors
Neuroglia
Hypothalamus
Coloring Agents

Keywords

  • Epilepsy
  • Gelastic seizure
  • Hypothalamic hamartoma

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology
  • Surgery

Cite this

Few isolated neurons in hypothalamic hamartomas may cause gelastic seizures. / Waldau, Ben; McLendon, Roger E.; Fuchs, Herbert E.; George, Timothy M.; Grant, Gerald A.

In: Pediatric Neurosurgery, Vol. 45, No. 3, 07.2009, p. 225-229.

Research output: Contribution to journalArticle

Waldau, Ben ; McLendon, Roger E. ; Fuchs, Herbert E. ; George, Timothy M. ; Grant, Gerald A. / Few isolated neurons in hypothalamic hamartomas may cause gelastic seizures. In: Pediatric Neurosurgery. 2009 ; Vol. 45, No. 3. pp. 225-229.
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