About Huntington's Disease
Brief History of HD
Huntington's disease was first described in medical literature by Dr. George Huntington in 1872 in a paper titled On Chorea. The word chorea used in reference to HD dates as far back as the 16th century and is derived from the Greek word for "dancing", referring to the specific dance-like movements associated with the disease. Dr Huntington lived in Long Island among a population with a high prevalence of HD and was also the first person to discover the potential genetic link and describe the onset and symptoms of HD.
Over a century later, Dr Nancy Wexler began her pioneering twenty-year-long field study with communities in Venezuela that had an unusually high prevalence of HD. Dr Wexler led a group of scientists who first discovered that the HD gene marker was located on the short arm of chromosome 4. Ten years later, the group located the Huntington gene itself which was instrumental to the development of the predictive gene testing procedure.
The prevalence rates of HD in Australia mirror those in other countries influenced by Western European migration. Tasmania, with much higher numbers of people with HD, is an exception to this. It is believed that HD was brought to Tasmania by a woman from South West England, who most likely inherited the disease from her father. The woman arrived in Launceston in 1842 with her husband and seven children and then had a further seven children. Nine of her fourteen children developed HD and today, seven of the nine branches of her family still have living descendants.
Developed using information from HOPES and a 1990 article by SA Pridmore ‘The large Huntington’s disease family in Tasmania’.