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Few isolated neurons in hypothalamic hamartomas may cause gelastic seizures.

Publication ,  Journal Article
Waldau, B; McLendon, RE; Fuchs, HE; George, TM; Grant, GA
Published in: Pediatr Neurosurg
2009

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.

Duke Scholars

Published In

Pediatr Neurosurg

DOI

EISSN

1423-0305

Publication Date

2009

Volume

45

Issue

3

Start / End Page

225 / 229

Location

Switzerland

Related Subject Headings

  • Neurons
  • Neurology & Neurosurgery
  • Male
  • Magnetic Resonance Imaging
  • Hypothalamus
  • Hypothalamic Diseases
  • Humans
  • Hamartoma
  • Epilepsies, Partial
  • Child, Preschool
 

Citation

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Waldau, B., McLendon, R. E., Fuchs, H. E., George, T. M., & Grant, G. A. (2009). Few isolated neurons in hypothalamic hamartomas may cause gelastic seizures. Pediatr Neurosurg, 45(3), 225–229. https://doi.org/10.1159/000224620
Waldau, Ben, Roger E. McLendon, Herbert E. Fuchs, Timothy M. George, and Gerald A. Grant. “Few isolated neurons in hypothalamic hamartomas may cause gelastic seizures.Pediatr Neurosurg 45, no. 3 (2009): 225–29. https://doi.org/10.1159/000224620.
Waldau B, McLendon RE, Fuchs HE, George TM, Grant GA. Few isolated neurons in hypothalamic hamartomas may cause gelastic seizures. Pediatr Neurosurg. 2009;45(3):225–9.
Waldau, Ben, et al. “Few isolated neurons in hypothalamic hamartomas may cause gelastic seizures.Pediatr Neurosurg, vol. 45, no. 3, 2009, pp. 225–29. Pubmed, doi:10.1159/000224620.
Waldau B, McLendon RE, Fuchs HE, George TM, Grant GA. Few isolated neurons in hypothalamic hamartomas may cause gelastic seizures. Pediatr Neurosurg. 2009;45(3):225–229.
Journal cover image

Published In

Pediatr Neurosurg

DOI

EISSN

1423-0305

Publication Date

2009

Volume

45

Issue

3

Start / End Page

225 / 229

Location

Switzerland

Related Subject Headings

  • Neurons
  • Neurology & Neurosurgery
  • Male
  • Magnetic Resonance Imaging
  • Hypothalamus
  • Hypothalamic Diseases
  • Humans
  • Hamartoma
  • Epilepsies, Partial
  • Child, Preschool