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Research ArticleBrain
Open Access

Onset and Progression of Pathologic Atrophy in Huntington Disease: A Longitudinal MR Imaging Study

N.Z. Hobbs, J. Barnes, C. Frost, S.M.D. Henley, E.J. Wild, K. Macdonald, R.A. Barker, R.I. Scahill, N.C. Fox and S.J. Tabrizi
American Journal of Neuroradiology June 2010, 31 (6) 1036-1041; DOI: https://doi.org/10.3174/ajnr.A2018
N.Z. Hobbs
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J. Barnes
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C. Frost
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S.M.D. Henley
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E.J. Wild
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K. Macdonald
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R.A. Barker
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R.I. Scahill
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N.C. Fox
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S.J. Tabrizi
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Abstract

BACKGROUND AND PURPOSE: Longitudinal MR imaging measures provide an opportunity to track progression in HD before the emergence of clinical symptoms. This may prove useful in assessing disease-modifying treatments. We investigated how caudate and global volumes change as HD progresses from premanifest to early disease.

MATERIALS AND METHODS: Forty HD gene-positive individuals and 19 controls underwent serial volumetric MR imaging (baseline, 12 and 27 months; 2 or 3 scans per person). At baseline, 3 patients with HD were premanifest but developed overt motor features during the study, and 37 had early HD. All had dates of motor onset recorded. Caudates, lateral ventricles, and TIVs were measured using semiautomated procedures. Linear mixed models were used to investigate differences between HD and controls in relation to motor onset, controlling for TIV, sex, and age.

RESULTS: Extrapolating backwards in time, we found that differences in caudate and ventricular volumes between patients with HD and controls were evident 14 and 5 years, respectively, before motor onset (P < .05). At onset, caudate volume was 2.58 mL smaller than that in controls (P < .0001); ventricular volume was 9.27 mL larger (P < .0001). HD caudate atrophy rates were linear, showed low variability between subjects, and were approximately 10-fold higher than those in controls (P < .001). HD ventricular enlargement rates were variable between subjects, were approximately 4-fold higher than those in controls at onset (P < .001), and accelerated with disease duration (P = .02).

CONCLUSIONS: We provide evidence of acceleration of global atrophy in HD with disproportionate caudate involvement. Both caudate and global measures may be of use as early markers of HD pathology.

Abbreviations

CI
confidence interval
Dur
disease duration (time since motor onset)
HD
Huntington disease
ICC
intraclass correlation coefficient
MBI
mean brain intensity
NHNN
National Hospital for Neurology and Neurosurgery
NIHR
National Institute for Health Research
TIV
total intracranial volume
UCL
University College London
UHDRS
Unified Huntington's Disease Rating Scale
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American Journal of Neuroradiology: 31 (6)
American Journal of Neuroradiology
Vol. 31, Issue 6
1 Jun 2010
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Cite this article
N.Z. Hobbs, J. Barnes, C. Frost, S.M.D. Henley, E.J. Wild, K. Macdonald, R.A. Barker, R.I. Scahill, N.C. Fox, S.J. Tabrizi
Onset and Progression of Pathologic Atrophy in Huntington Disease: A Longitudinal MR Imaging Study
American Journal of Neuroradiology Jun 2010, 31 (6) 1036-1041; DOI: 10.3174/ajnr.A2018

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Onset and Progression of Pathologic Atrophy in Huntington Disease: A Longitudinal MR Imaging Study
N.Z. Hobbs, J. Barnes, C. Frost, S.M.D. Henley, E.J. Wild, K. Macdonald, R.A. Barker, R.I. Scahill, N.C. Fox, S.J. Tabrizi
American Journal of Neuroradiology Jun 2010, 31 (6) 1036-1041; DOI: 10.3174/ajnr.A2018
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