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Elevated arteriolar cerebral blood volume in prodromal Huntington's disease.

ABSTRACT: Neurovascular alterations have been implicated in the pathophysiology of Huntington's disease (HD). Because arterioles are most responsive to metabolic alterations, arteriolar cerebral blood volume (CBVa) is an important indicator of cerebrovascular regulation. The objective of this pilot study was to investigate potential neurovascular (CBVa ) abnormality in prodromal-HD patients and compare it with the widely used imaging marker: brain atrophy.CBVa and brain volumes were measured with ultra-high-field (7.0-Telsa) magnetic resonance imaging in seven prodromal-HD patients and nine age-matched controls.Cortical CBVa was elevated significantly in prodromal-HD patients compared with controls (relative difference, 38.5%; effect size, 1.48). Significant correlations were found between CBVa in the frontal cortex and genetic measures. By contrast, no significant brain atrophy was detected in the prodromal-HD patients.CBVa may be abnormal in prodromal-HD, even before substantial brain atrophy occurs. Further investigation with a larger cohort and longitudinal follow-up is merited to determine whether CBVa could be used as a potential biomarker for clinical trials.

SUBMITTER: Hua J

PROVIDER: S-EPMC3834086 | biostudies-other | 2014 Mar

REPOSITORIES: biostudies-other

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Elevated arteriolar cerebral blood volume in prodromal Huntington's disease.

Hua Jun J Unschuld Paul G PG Margolis Russell L RL van Zijl Peter C M PC Ross Christopher A CA

Movement disorders : official journal of the Movement Disorder Society 20130711 3

<h4>Background</h4>Neurovascular alterations have been implicated in the pathophysiology of Huntington's disease (HD). Because arterioles are most responsive to metabolic alterations, arteriolar cerebral blood volume (CBVa) is an important indicator of cerebrovascular regulation. The objective of this pilot study was to investigate potential neurovascular (CBVa ) abnormality in prodromal-HD patients and compare it with the widely used imaging marker: brain atrophy.<h4>Methods</h4>CBVa and brain ...[more]

PMID: 23847161

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Project description:ObjectivePsychiatric symptoms are a significant aspect of Huntington's disease, an inherited neurodegenerative illness. The presentation of these symptoms is highly variable, and their course does not fully correlate with motor or cognitive disease progression. The authors sought to better understand the development and longitudinal course of psychiatric manifestations in individuals who carry the Huntington's disease mutation, starting from the prodromal period prior to motor diagnosis.MethodLongitudinal measures for up to 10 years of psychiatric symptoms from the Symptom Checklist-90-Revised were obtained from 1,305 participants (1,007 carrying the Huntington's disease mutation and 298 without [classified as controls]) and 1,235 companions enrolled in the Neurobiological Predictors of Huntington's Disease (PREDICT-HD) study. Participants with the mutation were stratified into three groups according to probability of motor diagnosis within 5 years. Using linear mixed-effects regression models, differences in psychiatric symptoms at baseline and over time between the mutation-positive groups and the controls were compared, as well as between ratings by mutation-positive participants and their companions.ResultsNineteen of 24 psychiatric measures (12 participant ratings and 12 companion ratings) were significantly higher at baseline and showed significant increases longitudinally in the individuals with the Huntington's disease mutation compared with controls. The differences were greatest in comparisons of symptom reports from companions compared with self-reports, especially in participants who were closest to motor diagnosis.ConclusionsThe results indicate that psychiatric manifestations develop more often than previously thought in the Huntington's disease prodrome. Symptoms also increase with progression of disease severity. Greater symptom ratings by companions than by mutation-positive participants suggest decreasing awareness in those affected.

Dataset Information

Elevated arteriolar cerebral blood volume in prodromal Huntington's disease.

Publications

Elevated arteriolar cerebral blood volume in prodromal Huntington's disease.

Similar Datasets

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