Using fiber tractography and diffusion kurtosis imaging to evaluate neuroimaging changes in patients with cerebrotendinous xanthomatosis after stopping chenodeoxycholic acid treatment for three years
Background: The aim of this study was to use tractography and diffusion kurtosis imaging (DKI) to evaluate cerebral white matter (WM) changes in patients with cerebrotendinous xanthomatosis (CTX) after stopping chenodeoxycholic acid (CDCA) treatment. Methods: Two siblings with CTX aged 40 and 38 yea...
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| Format: | Article |
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Elsevier
2022-10-01
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| Series: | Biomedical Journal |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2319417021001153 |
| _version_ | 1818467866698055680 |
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| author | Jun-Jun Lee Chiung-Chih Chang Wen-Neng Chang |
| author_facet | Jun-Jun Lee Chiung-Chih Chang Wen-Neng Chang |
| author_sort | Jun-Jun Lee |
| collection | DOAJ |
| description | Background: The aim of this study was to use tractography and diffusion kurtosis imaging (DKI) to evaluate cerebral white matter (WM) changes in patients with cerebrotendinous xanthomatosis (CTX) after stopping chenodeoxycholic acid (CDCA) treatment. Methods: Two siblings with CTX aged 40 and 38 years, respectively, who had been diagnosed with CTX for 16 years were enrolled. They had received CDCA treatment from 2005 until 2015, after which CDCA was no longer available in Taiwan. Serial brain magnetic resonance imaging (MRI) studies were used to record brain changes, and a seres of neuropsychiatric tests were used to evaluate cognitive changes 3 years after stopping CDCA treatment. Results: The conventional MRI studies revealed progressive changes in dentate nuclei and surrounding cerebellar hemispheres, but no obvious changes in cerebral white matter (WM). Tractography captured in 2018 showed a general reduction in fiber density, especially involving frontal lobe fibers, compared to 2015. In addition, the DKI studies performed in 2018 showed a decreased axonal water fraction in diffuse WM structures and increased RadEAD in frontal WM. Comparisons of the neuropsychiatric test results between 2015 and 2018 showed a marked decline in executive function including design fluency, digit backward span and digit forward span, and this cognitive impairment highly suggested frontal lobe dysfunction. Conclusions: This study may suggest that cerebral tractography and DKI study results can identify changes in cerebral WM in CTX patients shortly after stopping CDCA treatment, and that they may have a better correlation with the results of neuropsychiatric tests. |
| first_indexed | 2024-04-13T21:05:50Z |
| format | Article |
| id | doaj.art-b71135d114624a0681aad6ef8ea437ce |
| institution | Directory Open Access Journal |
| issn | 2319-4170 |
| language | English |
| last_indexed | 2024-04-13T21:05:50Z |
| publishDate | 2022-10-01 |
| publisher | Elsevier |
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| series | Biomedical Journal |
| spelling | doaj.art-b71135d114624a0681aad6ef8ea437ce2022-12-22T02:29:59ZengElsevierBiomedical Journal2319-41702022-10-01455814820Using fiber tractography and diffusion kurtosis imaging to evaluate neuroimaging changes in patients with cerebrotendinous xanthomatosis after stopping chenodeoxycholic acid treatment for three yearsJun-Jun Lee0Chiung-Chih Chang1Wen-Neng Chang2Department of Neurology, Chang Gung Memorial Hospital at Kaohsiung, Kaohsiung, Taiwan; Department of Information Management, National Sun Yat-sen University, Kaohsiung, Taiwan; College of Medicine, Chang Gung University, Taoyuan, TaiwanDepartment of Neurology, Chang Gung Memorial Hospital at Kaohsiung, Kaohsiung, Taiwan; College of Medicine, Chang Gung University, Taoyuan, TaiwanDepartment of Neurology, Chang Gung Memorial Hospital at Kaohsiung, Kaohsiung, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan; Corresponding author. Department of Neurology, Chang Gung Memorial Hospital at Kaohsiung, 123, Ta-Pei Rd., Niaosung, Kaohsiung 833, Taiwan. Tel. +886 7 7319831.Background: The aim of this study was to use tractography and diffusion kurtosis imaging (DKI) to evaluate cerebral white matter (WM) changes in patients with cerebrotendinous xanthomatosis (CTX) after stopping chenodeoxycholic acid (CDCA) treatment. Methods: Two siblings with CTX aged 40 and 38 years, respectively, who had been diagnosed with CTX for 16 years were enrolled. They had received CDCA treatment from 2005 until 2015, after which CDCA was no longer available in Taiwan. Serial brain magnetic resonance imaging (MRI) studies were used to record brain changes, and a seres of neuropsychiatric tests were used to evaluate cognitive changes 3 years after stopping CDCA treatment. Results: The conventional MRI studies revealed progressive changes in dentate nuclei and surrounding cerebellar hemispheres, but no obvious changes in cerebral white matter (WM). Tractography captured in 2018 showed a general reduction in fiber density, especially involving frontal lobe fibers, compared to 2015. In addition, the DKI studies performed in 2018 showed a decreased axonal water fraction in diffuse WM structures and increased RadEAD in frontal WM. Comparisons of the neuropsychiatric test results between 2015 and 2018 showed a marked decline in executive function including design fluency, digit backward span and digit forward span, and this cognitive impairment highly suggested frontal lobe dysfunction. Conclusions: This study may suggest that cerebral tractography and DKI study results can identify changes in cerebral WM in CTX patients shortly after stopping CDCA treatment, and that they may have a better correlation with the results of neuropsychiatric tests.http://www.sciencedirect.com/science/article/pii/S2319417021001153Cerebrotendinous xanthomatosisChenodeoxycholic acidMagnetic resonance imagingTractographyDiffusion kurtosis imaging |
| spellingShingle | Jun-Jun Lee Chiung-Chih Chang Wen-Neng Chang Using fiber tractography and diffusion kurtosis imaging to evaluate neuroimaging changes in patients with cerebrotendinous xanthomatosis after stopping chenodeoxycholic acid treatment for three years Biomedical Journal Cerebrotendinous xanthomatosis Chenodeoxycholic acid Magnetic resonance imaging Tractography Diffusion kurtosis imaging |
| title | Using fiber tractography and diffusion kurtosis imaging to evaluate neuroimaging changes in patients with cerebrotendinous xanthomatosis after stopping chenodeoxycholic acid treatment for three years |
| title_full | Using fiber tractography and diffusion kurtosis imaging to evaluate neuroimaging changes in patients with cerebrotendinous xanthomatosis after stopping chenodeoxycholic acid treatment for three years |
| title_fullStr | Using fiber tractography and diffusion kurtosis imaging to evaluate neuroimaging changes in patients with cerebrotendinous xanthomatosis after stopping chenodeoxycholic acid treatment for three years |
| title_full_unstemmed | Using fiber tractography and diffusion kurtosis imaging to evaluate neuroimaging changes in patients with cerebrotendinous xanthomatosis after stopping chenodeoxycholic acid treatment for three years |
| title_short | Using fiber tractography and diffusion kurtosis imaging to evaluate neuroimaging changes in patients with cerebrotendinous xanthomatosis after stopping chenodeoxycholic acid treatment for three years |
| title_sort | using fiber tractography and diffusion kurtosis imaging to evaluate neuroimaging changes in patients with cerebrotendinous xanthomatosis after stopping chenodeoxycholic acid treatment for three years |
| topic | Cerebrotendinous xanthomatosis Chenodeoxycholic acid Magnetic resonance imaging Tractography Diffusion kurtosis imaging |
| url | http://www.sciencedirect.com/science/article/pii/S2319417021001153 |
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