Alterations of striatal neurons in benign hereditary chorea

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