Neuropathology of Huntington’s Disease

Abstract

Huntington’s disease (HD) is a devastating neurodegenerative disease that results in motor, cognitive, and psychiatric impairments. HD results from an autosomal dominant polyglutamine expansion in the huntingtin (HTT) gene that results in a misfolded and aggregated protein. The disease is uniformly fatal and demonstrates characteristic neuropathological changes. While the striatum is preferentially affected, the cortex and many other brain regions are involved in pathogenesis and show progressive changes throughout the disease.