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by Dr George El-Nimr and
Dr Darren Carr
Last updated: February 2016
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
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
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.
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