E08 Cerebral blood flow is associated with disease severity and cognitive defecits in pre/early huntington’s disease

Author(s):  
Hannah Furby ◽  
James Ralph ◽  
Anne E Rosser ◽  
Peter O’Callaghan ◽  
Kevin Murphy ◽  
...  
1992 ◽  
Vol 55 (11) ◽  
pp. 1018-1023 ◽  
Author(s):  
S G Hasselbalch ◽  
G Oberg ◽  
S A Sorensen ◽  
A R Andersen ◽  
G Waldemar ◽  
...  

1989 ◽  
Vol 2 (1-2) ◽  
pp. 136
Author(s):  
TerryE. Goldberg ◽  
KarenFaith Berman ◽  
Erich Mohr ◽  
DanielR. Weinberger

1985 ◽  
Vol 42 (12) ◽  
pp. 1169-1175 ◽  
Author(s):  
N. Tanahashi ◽  
J. S. Meyer ◽  
Y. Ishikawa ◽  
P. Kandula ◽  
K. F. Mortel ◽  
...  

2011 ◽  
Vol 31 (9) ◽  
pp. 1908-1918 ◽  
Author(s):  
Robert C Wolf ◽  
Georg Grön ◽  
Fabio Sambataro ◽  
Nenad Vasic ◽  
Nadine D Wolf ◽  
...  

Magnetic resonance imaging (MRI) of the brain could be a powerful tool for discovering early biomarkers in clinically presymptomatic carriers of the Huntington's disease gene mutation (preHD). The aim of this study was to investigate the sensitivity of resting-state perfusion MRI in preHD and to identify neural changes, which could serve as biomarkers for future clinical trials. Differences in regional cerebral blood flow (rCBF) in 18 preHD and 18 controls were assessed with a novel MRI method based on perfusion images obtained with continuous arterial spin labeling. High-resolution structural data were collected to test for changes of brain volume. Compared with controls, preHD individuals showed decreased rCBF in medial and lateral prefrontal regions and increased rCBF in the precuneus. PreHD near to symptom onset additionally showed decreased rCBF in the putamen and increased rCBF in the hippocampus. Network analyses revealed an abnormal lateral prefrontal pattern in preHD far and near to motor onset. These data suggest early changes of frontostriatal baseline perfusion in preHD independent of substantial reductions of gray matter volume. This study also shows the feasibility of detecting neural changes in preHD with a robust MRI technique that would be suitable for longitudinal multisite application.


Author(s):  
Samuel Woodgate ◽  
Philippa Morgan-Jones ◽  
Susanne Clinch ◽  
Cheney Drew ◽  
Rebecca Playle ◽  
...  

Abstract Background The Clinch Token Transfer Test (C3t) is a bi-manual coin transfer task that incorporates cognitive tasks to add complexity. This study explored the concurrent and convergent validity of the C3t as a simple, objective assessment of impairment that is reflective of disease severity in Huntington’s, that is not reliant on clinical expertise for administration. Methods One-hundred-and-five participants presenting with pre-manifest (n = 16) or manifest (TFC-Stage-1 n = 39; TFC-Stage-2 n = 43; TFC-Stage-3 n = 7) Huntington’s disease completed the Unified Huntington’s Disease Rating Scale and the C3t at baseline. Of these, thirty-three were followed up after 12 months. Regression was used to estimate baseline individual and composite clinical scores (including cognitive, motor, and functional ability) using baseline C3t scores. Correlations between C3t and clinical scores were assessed using Spearman’s R and visually inspected in relation to disease severity using scatterplots. Effect size over 12 months provided an indication of longitudinal behaviour of the C3t in relation to clinical measures. Results Baseline C3t scores predicted baseline clinical scores to within 9–13% accuracy, being associated with individual and composite clinical scores. Changes in C3t scores over 12 months were small ($$\Omega$$ Ω ≤ 0.15) and mirrored the change in clinical scores. Conclusion The C3t demonstrates promise as a simple, easy to administer, objective outcome measure capable of predicting impairment that is reflective of Huntington’s disease severity and offers a viable solution to support remote clinical monitoring. It may also offer utility as a screening tool for recruitment to clinical trials given preliminary indications of association with the prognostic index normed for Huntington’s disease.


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