2023, Number 3
Arch Neurocien 2023; 28 (3)
George Huntington’s “on chorea” after 150 years: how a “few words” changed the history of a disease
Castillo-Torres SA
Full text
Language: English
References: 13
Page: 49-51
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ABSTRACT
“In the whole range of descriptive nosology there is not, to my knowledge, an instance in which a disease has been so accurately and fully delineated in so few words were given.”Sir William Osler, On Chorea and Choreiform Affections (1894)
Huntington’s disease (HD, OMIM #143100) is an autosomal dominant neurodegenerative disorder caused by a repeat expansion of the CAG trinucleotide in the Huntingtin gene (Htt), located on chromosome 4 (4p16.3). Clinically, it is widely known for chorea: an involuntary hyperkinetic movement disorder characterized by movements apparently flowing from one body part to another in a random, non-rhythmic, and non-patterned fashion.
The term “chorea” comes from the Greek χορεία (khoreía), meaning “a round dance”. It was first proposed by Paracelsus in the XVI century to describe the main symptom of St. Vitus’ dance, also known as the dancing disease; which he suggested to rename chorea lasciva (unrestrained dance), attributing it to the sufferer’s imagination, since neither “God or the Saint inflict it”. In contrast, chorea naturalis, or of natural (“organic”) origin, was considered to require medicines to be treated.
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