Evaluation of longitudinal 12 and 24 month cognitive outcomes in premanifest and early Huntington's disease

Julie C Stout; Rebecca Jones; Izelle Labuschagne; Alison M O'Regan; Miranda J Say; Eve M Dumas; Sarah Queller; Damian Justo; Rachelle Dar Santos; Allison Coleman; Ellen P Hart; Alexandra Dürr; Blair R Leavitt; Raymund A Roos; Doug R Langbehn; Sarah J Tabrizi; Chris Frost

doi:10.1136/jnnp-2011-301940

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Evaluation of longitudinal 12 and 24 month cognitive outcomes in premanifest and early Huntington's disease

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Evaluation of longitudinal 12 and 24 month cognitive outcomes in premanifest and early Huntington's disease

Julie C Stout, Rebecca Jones, Izelle Labuschagne, Alison M O'Regan, Miranda J Say, Eve M Dumas, Sarah Queller, Damian Justo, Rachelle Dar Santos, Allison Coleman, …

Journal of neurology, neurosurgery and psychiatry, Vol.83(7), pp.687-694

07/2012

DOI: 10.1136/jnnp-2011-301940

PMCID: PMC3368487

PMID: 22566599

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Abstract

Background Deterioration of cognitive functioning is a debilitating symptom in many neurodegenerative diseases, such as Huntington's disease (HD). To date, there are no effective treatments for the cognitive problems associated with HD. Cognitive assessment outcomes will have a central role in the efforts to develop treatments to delay onset or slow the progression of the disease. The TRACK-HD study was designed to build a rational basis for the selection of cognitive outcomes for HD clinical trials. Methods There were a total of 349 participants, including controls (n=116), premanifest HD (n=117) and early HD (n=116). A standardised cognitive assessment battery (including nine cognitive tests comprising 12 outcome measures) was administered at baseline, and at 12 and 24 months, and consisted of a combination of paper and pencil and computerised tasks selected to be sensitive to cortical-striatal damage or HD. Each cognitive outcome was analysed separately using a generalised least squares regression model. Results are expressed as effect sizes to permit comparisons between tasks. Results 10 of the 12 cognitive outcomes showed evidence of deterioration in the early HD group, relative to controls, over 24 months, with greatest sensitivity in Symbol Digit, Circle Tracing direct and indirect, and Stroop word reading. In contrast, there was very little evidence of deterioration in the premanifest HD group relative to controls. Conclusions The findings describe tests that are sensitive to longitudinal cognitive change in HD and elucidate important considerations for selecting cognitive outcomes for clinical trials of compounds aimed at ameliorating cognitive decline in HD.

Details

Title: Subtitle: Evaluation of longitudinal 12 and 24 month cognitive outcomes in premanifest and early Huntington's disease
Creators: Julie C Stout - School of Psychology and Psychiatry, Monash University, Melbourne, Victoria, Australia
Rebecca Jones - Department of Medical Statistics, London School of Hygiene and Tropical Medicine, London, UK
Izelle Labuschagne - School of Psychology and Psychiatry, Monash University, Melbourne, Victoria, Australia
Alison M O'Regan - School of Psychology and Psychiatry, Monash University, Melbourne, Victoria, Australia
Miranda J Say - UCL Institute of Neurology, University College London, London, UK
Eve M Dumas - Department of Neurology, Leiden University Medical Centre, The Netherlands
Sarah Queller - Queller Consulting, Dunedin, Florida, USA
Damian Justo - Department of Genetics and Cytogenetics, and INSERM UMR S, APHP Hôpital de la Salpêtrière, Paris, France
Rachelle Dar Santos - Department of Medical Genetics, University of British Columbia, Vancouver, British Columbia, Canada
Allison Coleman - Department of Medical Genetics, University of British Columbia, Vancouver, British Columbia, Canada
Ellen P Hart - Department of Neurology, Leiden University Medical Centre, The Netherlands
Alexandra Dürr - Department of Genetics and Cytogenetics, and INSERM UMR S, APHP Hôpital de la Salpêtrière, Paris, France
Blair R Leavitt - Department of Medical Genetics, University of British Columbia, Vancouver, British Columbia, Canada
Raymund A Roos - Department of Neurology, Leiden University Medical Centre, The Netherlands
Doug R Langbehn - Departments of Psychiatry and Biostatistics (Secondary), University of Iowa, Iowa City, Iowa, USA
Sarah J Tabrizi - UCL Institute of Neurology, University College London, London, UK
Chris Frost - Department of Medical Statistics, London School of Hygiene and Tropical Medicine, London, UK
Resource Type: Journal article
Publication Details: Journal of neurology, neurosurgery and psychiatry, Vol.83(7), pp.687-694
DOI: 10.1136/jnnp-2011-301940
PMID: 22566599
PMCID: PMC3368487
NLM abbreviation: J Neurol Neurosurg Psychiatry
ISSN: 0022-3050
eISSN: 1468-330X
Language: English
Date published: 07/2012
Academic Unit: Psychiatry; Iowa Neuroscience Institute
Record Identifier: 9984004197002771

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