Journal article
Subcortical T1-Rho MRI Abnormalities in Juvenile-Onset Huntington’s Disease
Brain sciences, Vol.10(8), p.533
08/01/2020
DOI: 10.3390/brainsci10080533
PMID: 32784364
Abstract
Huntington’s disease (HD) is a fatal neurodegenerative disease caused by the expansion of cytosine-adenine-guanine (CAG) repeats in the
huntingtin
gene. An increased CAG repeat length is associated with an earlier disease onset. About 5% of HD cases occur under the age of 21 years, which are classified as juvenile-onset Huntington’s disease (JOHD). Our study aims to measure subcortical metabolic abnormalities in JOHD participants. T1-Rho (T
1ρ
) MRI was used to compare brain regions of 13 JOHD participants and 39 controls. Region-of-interest analyses were used to assess differences in quantitative T
1ρ
relaxation times. We found that the mean relaxation times in the caudate (
p
< 0.001), putamen (
p
< 0.001), globus pallidus (
p
< 0.001), and thalamus (
p
< 0.001) were increased in JOHD participants compared to controls. Furthermore, increased T
1ρ
relaxation times in these areas were significantly associated with lower volumes amongst participants in the JOHD group. These findings suggest metabolic abnormalities in brain regions previously shown to degenerate in JOHD. We also analyzed the relationships between mean regional T
1ρ
relaxation times and Universal Huntington’s Disease Rating Scale (UHDRS) scores. UHDRS was used to evaluate participants’ motor function, cognitive function, behavior, and functional capacity. Mean T
1ρ
relaxation times in the caudate (
p
= 0.003), putamen (
p
= 0.005), globus pallidus (
p
= 0.009), and thalamus (
p
= 0.015) were directly proportional to the UHDRS score. This suggests that the T
1ρ
relaxation time may also predict HD-related motor deficits. Our findings suggest that subcortical metabolic abnormalities drive the unique hypokinetic symptoms in JOHD.
Details
- Title: Subtitle
- Subcortical T1-Rho MRI Abnormalities in Juvenile-Onset Huntington’s Disease
- Creators
- Alexander V Tereshchenko - Department of Psychiatry, Carver College of Medicine at the University of Iowa, Iowa City, IA 52242, USAJordan L Schultz - Department of Psychiatry, Carver College of Medicine at the University of Iowa, Iowa City, IA 52242, USAAnsley J Kunnath - Department of Cell Biology and Neuroscience, Rutgers University, New Brunswick, NJ 08854, USAJoel E Bruss - Department of Psychiatry, Carver College of Medicine at the University of Iowa, Iowa City, IA 52242, USAEric A Epping - Department of Psychiatry, Carver College of Medicine at the University of Iowa, Iowa City, IA 52242, USAVincent A Magnotta - Department of Psychiatry, Carver College of Medicine at the University of Iowa, Iowa City, IA 52242, USAPeg C Nopoulos - Department of Psychiatry, Carver College of Medicine at the University of Iowa, Iowa City, IA 52242, USA
- Resource Type
- Journal article
- Publication Details
- Brain sciences, Vol.10(8), p.533
- DOI
- 10.3390/brainsci10080533
- PMID
- 32784364
- NLM abbreviation
- Brain Sci
- ISSN
- 2076-3425
- eISSN
- 2076-3425
- Publisher
- MDPI
- Grant note
- DOI: 10.13039/100000065, name: National Institute of Neurological Disorders and Stroke, award: R01NS055903
- Language
- English
- Date published
- 08/01/2020
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; Neurology; Radiology; Psychiatry; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Pharmacy Practice and Science
- Record Identifier
- 9984066382302771
Metrics
13 Record Views