Hypothalamic hamartoma with neurofibrillary tangles

Neuropathology. 2016 Oct;36(5):480-484. doi: 10.1111/neup.12296. Epub 2016 Apr 18.

Abstract

Hypothalamic hamartomas are rare tumors that typically present in childhood, often with gelastic seizures, precocious puberty, or as a manifestation of Pallister-Hall syndrome. Neurofibrillary tangles are cytoplasmic aggregates of hyperphosphorylated tau that are best recognized in Alzheimer disease, other tau-associated neurodegenerative diseases, or as part of aging, but occasionally may be seen in low-grade neoplasms with a ganglion cell component as gangliocytoma or ganglioglioma. Herein, we report a case of hypothalamic hamartoma with neurofibrillary tangles.

Keywords: gangliocytoma; ganglioglioma; hypothalamic hamartoma; neurofibrillary tangle; tau.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Brain / diagnostic imaging
  • Brain / metabolism
  • Brain / pathology
  • Hamartoma / complications*
  • Hamartoma / diagnostic imaging
  • Hamartoma / metabolism
  • Hamartoma / pathology*
  • Humans
  • Hypothalamic Diseases / complications*
  • Hypothalamic Diseases / diagnostic imaging
  • Hypothalamic Diseases / metabolism
  • Hypothalamic Diseases / pathology*
  • Male
  • Neurofibrillary Tangles / metabolism
  • Neurofibrillary Tangles / pathology*
  • Neurons / metabolism
  • Neurons / pathology
  • tau Proteins / metabolism

Substances

  • MAPT protein, human
  • tau Proteins

Supplementary concepts

  • Hypothalamic hamartomas