Elevated arteriolar cerebral blood volume in prodromal Huntington's disease

Jun Hua; Paul G. Unschuld; Russell L. Margolis; Peter C.M. Van Zijl; Christopher A. Ross

doi:10.1002/mds.25591

Elevated arteriolar cerebral blood volume in prodromal Huntington's disease

Jun Hua, Paul G. Unschuld, Russell L. Margolis, Peter C.M. Van Zijl, Christopher A. Ross

Research output: Contribution to journal › Article › peer-review

27 Scopus citations

Abstract

Background: 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 (CBV_a) is an important indicator of cerebrovascular regulation. The objective of this pilot study was to investigate potential neurovascular (CBV_a) abnormality in prodromal-HD patients and compare it with the widely used imaging marker: brain atrophy. Methods: CBV_a 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. Results: Cortical CBV_a was elevated significantly in prodromal-HD patients compared with controls (relative difference, 38.5%; effect size, 1.48). Significant correlations were found between CBV_a in the frontal cortex and genetic measures. By contrast, no significant brain atrophy was detected in the prodromal-HD patients. Conclusions: CBV_a 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 CBV_a could be used as a potential biomarker for clinical trials.

Original language	English (US)
Pages (from-to)	396-401
Number of pages	6
Journal	Movement Disorders
Volume	29
Issue number	3
DOIs	https://doi.org/10.1002/mds.25591
State	Published - Mar 2014

Keywords

Atrophy
Biomarker
Imaging
Neurodegeneration
Neurovascular

ASJC Scopus subject areas

Neurology
Clinical Neurology

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10.1002/mds.25591

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title = "Elevated arteriolar cerebral blood volume in prodromal Huntington's disease",

abstract = "Background: 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. Methods: 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. Results: 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. Conclusions: 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.",

keywords = "Atrophy, Biomarker, Imaging, Neurodegeneration, Neurovascular",

author = "Jun Hua and Unschuld, {Paul G.} and Margolis, {Russell L.} and {Van Zijl}, {Peter C.M.} and Ross, {Christopher A.}",

year = "2014",

month = mar,

doi = "10.1002/mds.25591",

language = "English (US)",

volume = "29",

pages = "396--401",

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T1 - Elevated arteriolar cerebral blood volume in prodromal Huntington's disease

AU - Hua, Jun

AU - Unschuld, Paul G.

AU - Margolis, Russell L.

AU - Van Zijl, Peter C.M.

AU - Ross, Christopher A.

PY - 2014/3

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N2 - Background: 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. Methods: 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. Results: 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. Conclusions: 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.

AB - Background: 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. Methods: 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. Results: 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. Conclusions: 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.

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KW - Biomarker

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KW - Neurodegeneration

KW - Neurovascular

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Elevated arteriolar cerebral blood volume in prodromal Huntington's disease

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Keywords

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