An inherited adult-onset disorder of the nervous system due to a dominant gene mutation on chromosome 4 causing neurological degeneration, especially to GABA-ergic neurons in the caudate nucleus and putamen of the basal ganglia. Signs include uncoordinated movements, irritability, and attention span deficits. Leads to death with 15-20 years of onset. The first thorough description of chorea was made by Thomas Sydenham (1624-1689), now known as Sydenham’s chorea (a childhood chorea, distinct from Huntington’s disease, it is associated with rheumatic fever, and is also known as St. Vitus’s dance). Huntington’s disease was concisely described as a specific disease entity in 1872 by the physician George Huntington (1850-1916).
See Athetoid (or athetotiform) movements, Alzheimer’s disease, Autosomal dominant condition/disease, Basal ganglia (anatomy), Basal ganglia (disorders), Choreiform movements, Conditional knockouts, Gamma aminobutyric acid (GABA), Mutation (biology), Prefrontal-frontal-striatal loops, Protein-folding problem