Dataset Information

Progression of structural neuropathology in preclinical Huntington's disease: a tensor based morphometry study.

ABSTRACT:

Background and objectives

Regional cerebral atrophy occurs in carriers of the Huntington's disease (HD) gene mutation before clinical diagnosis is possible. The current inability to reliably measure progression of pathology in this preclinical phase impedes development of therapies to delay clinical onset. We hypothesised that longitudinal statistical imaging would detect progression of structural pathology in preclinical carriers of the HD gene mutation, in the absence of measurable clinical change.

Methods

Thirty subjects (17 preclinical mutation positive, 13 mutation negative) underwent serial clinical and magnetic resonance imaging (MRI) assessments over an interval of 2 years. Statistically significant changes in regional grey and white matter volume on MRI were analysed using tensor based morphometry (TBM). This technique derives a voxel-wise estimation of regional tissue volume change from the deformation field required to warp a subject's early to late T1 images.

Results

Over 2 years, there was progressive regional grey matter atrophy in mutation-positive relative to negative subjects, without significant clinical progression of disease. Significant grey matter volume loss was limited to bilateral putamen and globus pallidus externa (GPe), left caudate nucleus, and left ventral midbrain in the region of the substantia nigra.

Conclusions

While these results are consistent with previous cross sectional pathologic and morphometric studies, significant progression of atrophy in HD before the onset of significant clinical decline is now demonstrable with longitudinal statistical imaging. Such measures could be used to assess the efficacy of potential disease modifying drugs in slowing the progression of pathology before confirmed clinical onset of HD.

SUBMITTER: Kipps CM

PROVIDER: S-EPMC1739615 | biostudies-literature | 2005 May

REPOSITORIES: biostudies-literature

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Publications

Progression of structural neuropathology in preclinical Huntington's disease: a tensor based morphometry study.

Kipps C M CM Duggins A J AJ Mahant N N Gomes L L Ashburner J J McCusker E A EA

Journal of neurology, neurosurgery, and psychiatry 20050501 5

<h4>Background and objectives</h4>Regional cerebral atrophy occurs in carriers of the Huntington's disease (HD) gene mutation before clinical diagnosis is possible. The current inability to reliably measure progression of pathology in this preclinical phase impedes development of therapies to delay clinical onset. We hypothesised that longitudinal statistical imaging would detect progression of structural pathology in preclinical carriers of the HD gene mutation, in the absence of measurable cli ...[more]

PMID: 15834021

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Dataset Information

Progression of structural neuropathology in preclinical Huntington's disease: a tensor based morphometry study.

Background and objectives

Methods

Results

Conclusions

Publications

Progression of structural neuropathology in preclinical Huntington's disease: a tensor based morphometry study.

Similar Datasets

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