Brain Advance Access originally published online on July 13, 2009
Brain 2009 132(8):2289-2295; doi:10.1093/brain/awp149
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© The Author (2009). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
The conquest of Wilson's disease
Formerly Dept of Neurology, The Middlesex Hospital, London (Retired)
Correspondence to: J. M. Walshe, Huntingdon, UK E-mail: penicillamine@waitrose.com
Received December 1, 2008. Revised February 16, 2009. Accepted May 7, 2009.
| The first 150 words of the full text of this article appear below. |
When (Samuel Alexander) Kinnier Wilson described a new disease involving the liver and the lenticular nucleus of the brain in 1912, he was unable to recommend any form of treatment, although that remained his life long ambition (Wilson, 1912
; Fig. 1). Until the underlying cause of the disease was understood, this inevitably remained a somewhat forlorn hope. A role for copper, as a possible pathogenic agent, was suggested the year after Wilson's original publication when Rumpel (1913) reported finding excess copper in the liver of a patient who had died of this newly described disease. But this observation was not followed until, in 1948, (John) Cumings (1948) demonstrated that copper is present in excess both in the brain and liver of patients with Wilson's disease. This observation led Cumings to suggest that treatment with the newly developed chelating agent British antilewisite (Dimercaprol) might arrest the progress