Diffusion Tensor Magnetic Resonance Imaging for Differentiating Multiple System Atrophy Cerebellar Type and Spinocerebellar Ataxia Type 3

Jao, Chi Wen; Soong, Bing Wen; Huang, Chao; Duan, Chien An; Wu, Chao‐Hsiung; Wu, Yu Te; Wang, Po Shan

doi:10.3390/brainsci9120354

Cited by 13 publications

(15 citation statements)

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“…What is more, MD increases showed a similar pattern as FA decreases (Kang et al, 2014;Wu et al, 2017;Jao et al, 2019), and these findings were in line with the results from the multi-atlas segmented findings (Rezende et al, 2018). WM abnormalities were also identified in some fiber pathways that were mostly in the cerebellar connecting tracts, including the pyramidal tract, thalamic radiations, medial lemnisci, corticospinal tract, corticobulbar tract, and corticopontocerebellar tract in patients with SCA3/MJD (Rezende et al, 2018;Jao et al, 2019). WM tract impairments are common features among SCA patients.…”

Section: Diffusion Tensor Imagingsupporting

confidence: 85%

“…Decreased FA and Increased AD, RD, and MD in SCA3/MJD Patients DTI studies with TBSS have revealed a widespread decrease in FA accompanied by increased AD, RD, and MD in the WM across the whole brain, especially the cerebellum and the brainstem, as well as some supratentorial areas, such as the bilateral frontal, parietal, temporal, and occipital lobes and thalamus, in patients with SCA3/MJD compared with those in controls, as shown in Figure 3 (Guimarães et al, 2013;Nunes et al, 2015;Jao et al, 2019). What is more, MD increases showed a similar pattern as FA decreases (Kang et al, 2014;Wu et al, 2017;Jao et al, 2019), and these findings were in line with the results from the multi-atlas segmented findings (Rezende et al, 2018). WM abnormalities were also identified in some fiber pathways that were mostly in the cerebellar connecting tracts, including the pyramidal tract, thalamic radiations, medial lemnisci, corticospinal tract, corticobulbar tract, and corticopontocerebellar tract in patients with SCA3/MJD (Rezende et al, 2018;Jao et al, 2019).…”

Section: Diffusion Tensor Imagingmentioning

confidence: 97%

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MR Imaging of SCA3/MJD

et al. 2020

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Spinocerebellar ataxia type 3/Machado-Joseph disease (SCA3/MJD) is a progressive autosomal dominantly inherited cerebellar ataxia characterized by the aggregation of polyglutamine-expanded protein within neuronal nuclei in the brain, which can lead to brain damage that precedes the onset of clinical manifestations. Magnetic resonance imaging (MRI) techniques such as morphometric MRI, diffusion tensor imaging (DTI), functional magnetic resonance imaging (fMRI), and magnetic resonance spectroscopy (MRS) have gained increasing attention as non-invasive and quantitative methods for the assessment of structural and functional alterations in clinical SCA3/MJD patients as well as preclinical carriers. Morphometric MRI has demonstrated typical patterns of atrophy or volume loss in the cerebellum and brainstem with extensive lesions in some supratentorial areas. DTI has detected widespread microstructural alterations in brain white matter, which indicate disrupted brain anatomical connectivity. Taskrelated fMRI has presented unusual brain activation patterns within the cerebellum and some extracerebellar tissue, reflecting the decreased functional connectivity of these brain regions in SCA3/MJD subjects. MRS has revealed abnormal neurochemical profiles, such as the levels or ratios of N-acetyl aspartate, choline, and creatine, in both clinical cases and preclinical cases before the alterations in brain anatomical structure. Moreover, a number of studies have reported correlations of MR imaging alterations with clinical and genetic features. The utility of these MR imaging techniques can help to identify preclinical SCA3/MJD carriers, monitor disease progression, evaluate response to therapeutic interventions, and illustrate the pathophysiological mechanisms underlying the occurrence, development, and prognosis of SCA3/MJD.

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Section: Diffusion Tensor Imagingsupporting

confidence: 85%

Section: Diffusion Tensor Imagingmentioning

confidence: 97%

MR Imaging of SCA3/MJD

et al. 2020

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“…Finally, we included a total of 29 articles in this review. Twenty papers examined structural MRI [volumetric: n = 12 (Bürk et al, 1996;Etchebehere et al, 2001;Yoshizawa et al, 2003;Liang et al, 2009;Schulz et al, 2010;Camargos et al, 2011;D'Abreu et al, 2011;Ogawa et al, 2012;De Rezende et al, 2015;Nunes et al, 2015;Rezende et al, 2018;Jao et al, 2019b), voxel-based morphometry (VBM): n = 8 (Schulz et al, 2010;D'Abreu et al, 2012;Guimarães et al, 2013;Lopes et al, 2013;Kang et al, 2014;Hernandez-Castillo et al, 2017;Peng et al, 2019;Guo et al, 2020), and surface analysis: n = 4 (De Nunes et al, 2015;Rezende et al, 2018;Arruda et al, 2020)], seven papers examined MRS (Lei et al, 2011;Lirng et al, 2012;Wang et al, 2012;Lopes et al, 2013;Chen et al, 2014;Adanyeguh et al, 2015;Peng et al, 2019), and ten papers examined DTI (Guimarães et al, 2013;Lopes et al, 2013;Kang et al, 2014;Nunes et al, 2015;Wu et al, 2017;Rezende et al, 2018;Jao et al, 2019a;Peng et al, 2019;Meira et al, ...…”

Section: Data Extraction and Analysismentioning

confidence: 99%

Magnetic Resonance Imaging and Its Clinical Correlation in Spinocerebellar Ataxia Type 3: A Systematic Review

et al. 2022

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BackgroundSpinocerebellar ataxia type 3 (SCA3) is a complex cerebrocerebellar disease primarily characterized by ataxia symptoms alongside motor and cognitive impairments. The heterogeneous clinical presentation of SCA3 necessitates correlations between magnetic resonance imaging (MRI) and clinical findings in reflecting progressive disease changes. At present, an attempt to systematically examine the brain-behavior relationship in SCA3, specifically, the correlation between MRI and clinical findings, is lacking.ObjectiveWe investigated the association strength between MRI abnormality and each clinical symptom to understand the brain-behavior relationship in SCA3.MethodsWe conducted a systematic review on Medline and Scopus to review studies evaluating the brain MRI profile of SCA3 using structural MRI (volumetric, voxel-based morphometry, surface analysis), magnetic resonance spectroscopy, and diffusion tensor imaging, including their correlations with clinical outcomes.ResultsOf 1,767 articles identified, 29 articles met the eligibility criteria. According to the National Institutes of Health quality assessment tool for case-control studies, all articles were of excellent quality. This systematic review found that SCA3 neuropathology contributes to widespread brain degeneration, affecting the cerebellum and brainstem. The disease gradually impedes the cerebral cortex and basal ganglia in the late stages of SCA3. Most findings reported moderate correlations (r = 0.30–0.49) between MRI features in several regions and clinical findings. Regardless of the MRI techniques, most studies focused on the brainstem and cerebellum.ConclusionsClinical findings suggest that rather than individual brain regions, the connectivity between different brain regions in distributed networks (i.e., cerebellar-cerebral network) may be responsible for motor and neurocognitive function in SCA3. This review highlights the importance of evaluating the progressive changes of the cerebellar-cerebral networks in SCA3 patients, specifically the functional connectivity. Given the relative lack of knowledge about functional connectivity on SCA3, future studies should investigate possible functional connectivity abnormalities in SCA3 using fMRI.

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“…Recently, we used diffusion tensor imaging (DTI) to measure water molecular diffusion of white matter (WM) alteration in SCA3 (15). We found SCA3 revealed widespread white matter lesions in the bilateral cerebral-frontal, -parietal, -temporal, and -occipital WM; cerebellar WM; the thalamus, and the brainstem.…”

Section: Discussionmentioning

confidence: 99%

“…Diffusion tensor imaging (DTI) can facilitate the visualization and characterization of white matter (WM), and DTI is also an efficient method for study of SCA3 (15). SCA3 had been verified as a WM dominant atrophy neurodegenerative disease (16).…”

Section: Introductionmentioning

confidence: 99%

Supratentorial and Infratentorial Lesions in Spinocerebellar Ataxia Type 3

Wang¹,

Wu²,

Wang

et al. 2020

Front. Neurol.

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Background: Spinocerebellar ataxia type 3 (SCA) is a cerebellum-dominant degenerative disorder that is characterized primarily by infratentorial damage, although less severe supratentorial involvement may contribute to the clinical manifestation. These impairments may result from the efferent loss of the cerebellar cortex and degeneration of the cerebral cortex. Method:We used the three-dimensional fractal dimension (3D-FD) method to quantify the morphological changes in the supratentorial regions and assessed atrophy in the relatively focal regions in patients with SCA3. A total of 48 patients with SCA3 and 50 sex-and age-matched healthy individuals, as the control group, participated in this study. The 3D-FD method was proposed to distinguish 97 automatic anatomical label regions of gray matter (left cerebrum: 45, right cerebrum: 45, cerebellum: 7) between healthy individuals and patients with SCA3.Results: Patients with SCA3 exhibited reduced brain complexity within both the traditional olivopontocerebellar atrophy (OPCA) pattern and specific supratentorial regions. The study results confirmed the extensive involvement of extracerebellar regions in SCA3. The atrophied regions of SCA3 in infratentorial and supratentorial cortex showed a wide range of overlapped areas as in two functional cortexes, namely cerebellum-related cortex and basal ganglia-related cortex.Conclusions: Our results found that the atrophy of the SCA3 are not only limited in the infratentorial regions. Both cerebellar related cortex and basal ganglia related cortex were affected in the disease process of SCA3. Our findings might correlate to the common symptoms of SCA3, such as ataxia, Parkinsonism, dysarthria, and dysmetria. SCA3 should no longer be considered a disease limited to the cerebellum and its connections; rather, it should be considered a pathology affecting the whole brain.

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Diffusion Tensor Magnetic Resonance Imaging for Differentiating Multiple System Atrophy Cerebellar Type and Spinocerebellar Ataxia Type 3

Cited by 13 publications

References 54 publications

MR Imaging of SCA3/MJD

MR Imaging of SCA3/MJD

Magnetic Resonance Imaging and Its Clinical Correlation in Spinocerebellar Ataxia Type 3: A Systematic Review

Supratentorial and Infratentorial Lesions in Spinocerebellar Ataxia Type 3

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