Quantitative susceptibility mapping in spinocerebellar ataxia type 3/Machado–Joseph disease (SCA3/MJD)

Background The deep nuclei, brainstem, and anterior central gyrus are important sites of spinocerebellar ataxia type3/Machado–Joseph Disease (SCA3/MJD) involvement. These locations were the common iron deposition areas. We hypothesized that iron deposition changes occur in SCA3/MJD patients and are...

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Bibliographic Details
Published in:Acta radiologica (1987) 2020-04, Vol.61 (4), p.520-527
Main Authors: Xie, Fangfang, Weihua, Liao, Lirong, Ouyang, Wang, Xiaoyi, Xing, Wu
Format: Article
Language:English
Subjects:
Adult
Brain - diagnostic imaging
Brain - metabolism
Brain Mapping - methods
Evaluation Studies as Topic
Female
Humans
Iron - metabolism
Machado-Joseph Disease - diagnostic imaging
Machado-Joseph Disease - metabolism
Magnetic Resonance Imaging - methods
Male
Middle Aged
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Summary:Background The deep nuclei, brainstem, and anterior central gyrus are important sites of spinocerebellar ataxia type3/Machado–Joseph Disease (SCA3/MJD) involvement. These locations were the common iron deposition areas. We hypothesized that iron deposition changes occur in SCA3/MJD patients and are associated with disease progression. Purpose Quantitative susceptibility mapping was used to quantitatively analyze changes in iron levels in SCA3/MJD patients. Material and Methods Eighteen symptomatic SCA3/MJD patients and 18 age-matched normal controls (group 1; NC1), and 12 asymptomatic mutation carriers (pre-SCA3/MJD) and 16 age-matched normal controls (group 2; NC2) were examined by enhanced gradient echo T2*-weighted angiography. Data were processed to obtain the quantitative susceptibility mapping values. Independent sample t-tests were conducted to compare the differences in the quantitative susceptibility mapping values. Results In the red nuclei and substantia nigra, the quantitative susceptibility mapping values of the symptomatic SCA3/MJD group were significantly higher than those of NC1 (P 
ISSN:0284-1851
1600-0455
DOI:10.1177/0284185119868906