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Brain Advance Access published online on May 13, 2008
Brain, doi:10.1093/brain/awn088
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© The Author (2008). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Book Review

‘Come sleep, O sleep, the certain knot of peace’ (Sir Philip Sidney, 1554–86)

The first 150 words of the full text of this article appear below.

This book addresses many of the major questions concerning the nature of human prion diseases by combining a chronological approach to the identification, study and diagnosis of these diseases with an historical account of one of the rarest human prion diseases, fatal familial insomnia (FFI). The author describes the effects of FFI on a Venetian family of supposed noble ancestry and interweaves the effects of this strange illness across several generations with descriptions of different forms of prion diseases in animals and humans, beginning with scrapie, passing on to Creutzfeldt-Jakob disease (CJD) and kuru, and progressing to bovine spongiform encephalopathy (BSE) and variant CJD. It is an ambitious attempt to encapsulate ‘prionology’ with personal descriptions of a devastating familial disorder, many of which are historical fiction, albeit informed by a detailed knowledge of the clinical features of FFI and an ability to evoke the changing atmosphere of Venice over the . . . [Full Text of this Article]

James W. Ironside

National CJD Surveillance Unit
University of Edinburgh
United Kingdom


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