Challenges in assessment

Helen M. Brewer, Roger A. Barker, and Oliver W. J. Quarrell

in Juvenile Huntington's Disease

Published on behalf of Oxford University Press

Published in print January 2009 | ISBN: 9780199236121
Published online November 2012 | e-ISBN: 9780191753213 | DOI:
Challenges in assessment

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Reliable and valid clinical rating scales that monitor disease progression are important—firstly, to monitor disease progression in an individual patient in the clinic and, secondly, to assess the effectiveness of any potential therapeutic intervention. Huntington-specific scales have been developed and used widely both in the clinic and for research. Whilst having some limitations, existing scales offer a useful tool to guide assessment and produce a uniformity of examination, with obvious advantages for the longitudinal assessment of patients and their response to treatment. However, given the varied clinical presentation of Huntington’s disease (HD), and in particular the very different clinical presentation seen in juvenile Huntington’s disease (JHD) the scales that have been used to monitor disease progression are less suitable for use in this context. This chapter will outline some of the main difficulties with the existing clinical rating scales for HD, and attempts to modify these for patients with JHD. It must be realized that rating scales of any sort have limitations given that they will only ever capture some aspects of the disease and convert it into a unitary value, typically using non-linear scales.

Chapter.  2585 words. 

Subjects: Neurology

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