Analysis of diffusion tensor parameters in spinocerebellar ataxia type 3 and type 10 patients

Meira, Alex Tiburtino; Arruda, Walter Oleschko; Ono, Sergio Eiji; Franklin, Gustavo Leite; Neto, Arnolfo de Carvalho; Raskin, Salmo; Ashizawa, Tetsuo; Camargo, Carlos Henrique Ferreira; Teive, Hélio A.G.

doi:10.1016/j.parkreldis.2020.06.460

Cited by 8 publications

(11 citation statements)

References 32 publications

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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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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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“…Structural abnormalities are accompanied by disturbances in white matter tracts as demonstrated by diffusion tensor imaging (DTI), with abnormalities discernible not just in the cerebellum and cerebellar peduncles but also in the brainstem and cerebral white matter. 3,96,97 These imaging abnormalities align 91 inferior, 91 and superior 91 frontal lobe, 91 lateral temporal lobe, 91 parietal lobe, and vermis 91 [ 99 mTc]ECD SPECT:↓perfusion in bilateral cerebellum 92 and vermis 92 [ 123 I]iomazenil SPECT:↓binding in cerebellum, 93 cerebral cortex, 93 thalamus, 93 41,98,100 Furthermore, the glial marker myoinositol has been shown to increase with gliosis. 100 Other metabolites that can be monitored include the metabolic marker creatine/phosphocreatine and the membrane marker choline.…”

Section: Imaging Biomarkersmentioning

confidence: 99%

Spinocerebellar ataxia clinical trials: opportunities and challenges

Brooker

Edamakanti

Akasha

et al. 2021

Ann Clin Transl Neurol

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The spinocerebellar ataxias (SCAs) are a group of dominantly inherited diseases that share the defining feature of progressive cerebellar ataxia. The disease process, however, is not confined to the cerebellum; other areas of the brain, in particular, the brainstem, are also affected, resulting in a high burden of morbidity and mortality. Currently, there are no disease-modifying treatments for the SCAs, but preclinical research has led to the development of therapeutic agents ripe for testing in patients. Unfortunately, due to the rarity of these diseases and their slow and variable progression, there are substantial hurdles to overcome in conducting clinical trials. While the epidemiological features of the SCAs are immutable, the feasibility of conducting clinical trials is being addressed through a combination of strategies. These include improvements in clinical outcome measures, the identification of imaging and fluid biomarkers, and innovations in clinical trial design. In this review, we highlight current challenges in initiating clinical trials for the SCAs and also discuss pathways for researchers and clinicians to mitigate these challenges and harness opportunities for clinical trial development.

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“…However, many therapeutic approaches to target and reduce this abnormal polyQ ATXN3 protein have been developed [2]. In order to assess therapeutic efficacy in clinical trials, reliable neuroimaging [4] and disease biomarkers are important. Recently, we demonstrated that polyQ ATXN3 proteins can be efficiently detected in human biological fluids [plasma and cerebrospinal fluid (CSF)], and may serve as a pharmacodynamic marker for mutant ATXN3 targeting therapies [1].…”

Section: Introductionmentioning

confidence: 99%

Urine levels of the polyglutamine ataxin-3 protein are elevated in patients with spinocerebellar ataxia type 3

Koike

Jansen-West

Al-Shaikh

et al. 2021

Parkinsonism & Related Disorders

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Analysis of diffusion tensor parameters in spinocerebellar ataxia type 3 and type 10 patients

Cited by 8 publications

References 32 publications

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

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

Spinocerebellar ataxia clinical trials: opportunities and challenges

Urine levels of the polyglutamine ataxin-3 protein are elevated in patients with spinocerebellar ataxia type 3

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