Abnormalities of Striatal Projection Neurons and<i>N</i>-Methyl-D-Aspartate Receptors in Presymptomatic Huntington's Disease

Abstract

HUNTINGTON'S disease is an autosomal dominant disease characterized by slowly progressive personality changes, dementia, and movement disorders.1 , 2 The average age at onset is 30 to 40 years, and the disease lasts for an average of 15 to 20 years. Analysis with restriction-fragmentlength polymorphisms (RFLPs) has localized the Huntington's disease gene close to the telomere of the short arm of chromosome 4.1 2 3 Prominent striatal atrophy with loss of striatal neurons and relative sparing of fibers of passage and afferent axons is the pathological hallmark of Huntington's disease.4 Recent studies have demonstrated a distinct pattern of vulnerability of striatal neurons in Huntington's . . .