SEARCH

SEARCH BY CITATION

Keywords:

  • benign hereditary chorea;
  • pathology;
  • striatal interneurons;
  • projection neurons;
  • TITF-1;
  • migration

Abstract

Benign hereditary chorea (BHC) recently has been associated with mutations in TITF-1 gene, although a pathological study of an individual with BHC and a TITF-1 mutation revealed no significant gross or microscopic abnormalities using standard methods. Immunohistochemical staining of striatal tissue from a BHC-affected postmortem brain was performed using antibodies against neurotransmitters of interneurons whose tangential migration is mediated by TITF-1. There was a loss of most TITF-1–mediated striatal interneurons in the BHC specimen compared to four matched control brains. © 2005 Movement Disorder Society