Chronic progressive chorea
Huntington disease (HD) is a dominant hereditary neurodegenerative disease with involuntary movements, mental disorders and progressive dementia as its main clinical features. It belongs to the category of dynamic gene mutation disease or polyglutamine repeat disease. Because Huntington disease has chorea as its prominent clinical symptom, the disease has been named as grand chorea, Huntington's disease, chronic progressive chorea or hereditary chorea. The clinical symptoms of this disease were first described by American physician Huntington in 1872. Alzheimer observed the pathological changes in 1911. In 1993, it was determined that its pathogenic gene was located at locus 63 of the short arm of the fourth pair of autosomes. The protein encoded by this gene was named Huntingtin. The pathological changes are characterized by the loss of neurons in the striatum and cerebral cortex. Recently, ubiquitin-positive neuronal nuclear inclusions and dystrophic neurites have been found in the cerebral cortex.