Huntington's disease (HD) is a rare genetic disorder affecting the central nervous system. It was described by Dr George Huntington in a paper submitted to the Medical and Surgical Reporter in 1872:
'the hereditary chorea, as I shall call it, is confined to certain and fortunately few families, and has been transmitted to them, an heirloom from generation away back in the dim past. It is spoken of by those in whose veins the seed of the disease are known to exist, with a kind of horror, and not at all alluded to except through dire necessity, when it is mentioned as "that disorder". It is attended generally by all the symptoms of common chorea, only in an aggravated degree hardly ever manifesting itself until adult or middle life, and then coming on gradually but surely, by increasing degrees, and often occupying years in its development, until the hapless sufferer is but a quivering wreck of his former self'.
While this disease is named after Dr George Huntington, it is important to remember that it was actually described by previous authors; though not as concisely or eloquently!
This module will cover the basic neuroscience in relation to this disorder together with the main clinical aspects. Different management strategies and input from different disciplines will also be discussed.