Abnormalities of Striatal Projection Neurons and N -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 4.123 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… © 1990, Massachusetts Medical Society. All rights reserved.