Literature DB >> 34783886

Brain structural abnormalities in the preclinical stage of Machado-Joseph disease/spinocerebellar ataxia type 3 (MJD/SCA3): evaluation by MRI morphometry, diffusion tensor imaging and neurite orientation dispersion and density imaging.

Mengcheng Li1, Xinyuan Chen2, Hao-Ling Xu3, Ziqiang Huang1, Naping Chen1, Yuqing Tu1, Shirui Gan4,5, Jianping Hu6.   

Abstract

OBJECTIVE: To investigate whether neurite orientation dispersion and density imaging (NODDI) could provide the added value for detecting brain microstructural alterations in the preclinical stage of Machado-Joseph disease/spinocerebellar ataxia type 3 (MJD/SCA3) compared with MRI morphometry and diffusion tensor imaging (DTI).
METHODS: Twenty preclinical MJD/SCA3 patients and 21 healthy controls were enrolled. Three b values DWI and 3D T1-weighted images were acquired at 3.0 T. Tract-based spatial statistics (TBSS) approach was used to investigate the white matter (WM) alterations in the DTI metrics and NODDI metrics. Gray matter-based spatial statistics (GBSS) approach was used to investigate the grey matter (GM) alterations in the NODDI metrics. Voxel-based morphometry (VBM) approach was performed on the 3D T1-weighted images. The relationship between the cytosine-adenine-guanine (CAG) repeat length and brain microstructural alterations of preclinical MJD/SCA3 was identified.
RESULTS: Compared with healthy controls, the preclinical MJD/SCA3 patients showed decreased FA and NDI as well as increased MD, AD, and RD in the WM of cerebellum and brainstem (corrected P < 0.05), and decreased NDI in the GM of cerebellar vermis (corrected P < 0.05). The CAG repeat length in preclinical MJD/SCA3 patients was negatively correlated with the reduced FA and NDI of the infratentorial WM and the reduced NDI of the cerebellum, and positively with the increased MD and RD of the infratentorial WM.
CONCLUSIONS: NOODI can provide novel quantitative microstructural changes in MJD/SCA3 carriers, expanding our understanding of the gray and white matter (axons and dendrites) degeneration in this frequent ataxia syndrome.
© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.

Entities:  

Keywords:  DTI; MJD; NODDI; Preclinical; SCA3; VBM

Mesh:

Year:  2021        PMID: 34783886     DOI: 10.1007/s00415-021-10890-2

Source DB:  PubMed          Journal:  J Neurol        ISSN: 0340-5354            Impact factor:   6.682


  42 in total

Review 1.  The preclinical stage of spinocerebellar ataxias.

Authors:  Roderick P P W M Maas; Judith van Gaalen; Thomas Klockgether; Bart P C van de Warrenburg
Journal:  Neurology       Date:  2015-06-10       Impact factor: 9.910

Review 2.  Spinocerebellar ataxia.

Authors:  Thomas Klockgether; Caterina Mariotti; Henry L Paulson
Journal:  Nat Rev Dis Primers       Date:  2019-04-11       Impact factor: 52.329

Review 3.  The Neuropathology of Spinocerebellar Ataxia Type 3/Machado-Joseph Disease.

Authors:  Arnulf H Koeppen
Journal:  Adv Exp Med Biol       Date:  2018       Impact factor: 2.622

4.  Biological and clinical characteristics of individuals at risk for spinocerebellar ataxia types 1, 2, 3, and 6 in the longitudinal RISCA study: analysis of baseline data.

Authors:  Heike Jacobi; Kathrin Reetz; Sophie Tezenas du Montcel; Peter Bauer; Caterina Mariotti; Lorenzo Nanetti; Maria Rakowicz; Anna Sulek; Alexandra Durr; Perrine Charles; Alessandro Filla; Antonella Antenora; Ludger Schöls; Julia Schicks; Jon Infante; Jun-Suk Kang; Dagmar Timmann; Roberto Di Fabio; Marcella Masciullo; Laszlo Baliko; Bela Melegh; Sylvia Boesch; Katrin Bürk; Annkathrin Peltz; Jörg B Schulz; Isabelle Dufaure-Garé; Thomas Klockgether
Journal:  Lancet Neurol       Date:  2013-05-22       Impact factor: 44.182

5.  Gray matter atrophy patterns within the cerebellum-neostriatum-cortical network in SCA3.

Authors:  Jing Guo; Hui Chen; Bharat B Biswal; Xiaonan Guo; Huangbin Zhang; Limeng Dai; Yuhan Zhang; Liang Li; Yunshuang Fan; Shaoqiang Han; Juan Liu; Liu Feng; Qiannan Wang; Jian Wang; Chen Liu; Huafu Chen
Journal:  Neurology       Date:  2020-10-06       Impact factor: 9.910

6.  Structural signature of SCA3: From presymptomatic to late disease stages.

Authors:  Thiago Junqueira Ribeiro Rezende; Jean Levi Ribeiro de Paiva; Alberto Rolim Muro Martinez; Iscia Lopes-Cendes; José Luiz Pedroso; Orlando Graziani Povoas Barsottini; Fernando Cendes; Marcondes C França
Journal:  Ann Neurol       Date:  2018-09-04       Impact factor: 10.422

Review 7.  Rating scales and biomarkers for CAG-repeat spinocerebellar ataxias: Implications for therapy development.

Authors:  Meng-Ling Chen; Chih-Chun Lin; Liana S Rosenthal; Puneet Opal; Sheng-Han Kuo
Journal:  J Neurol Sci       Date:  2021-04-01       Impact factor: 3.181

8.  Microstructural Alterations in Asymptomatic and Symptomatic Patients with Spinocerebellar Ataxia Type 3: A Tract-Based Spatial Statistics Study.

Authors:  Xinwei Wu; Xinxin Liao; Yafeng Zhan; Cheng Cheng; Wei Shen; Mufang Huang; Zhifan Zhou; Zheng Wang; Zilong Qiu; Wu Xing; Weihua Liao; Beisha Tang; Lu Shen
Journal:  Front Neurol       Date:  2017-12-22       Impact factor: 4.003

Review 9.  Clinical, Imaging, and Laboratory Markers of Premanifest Spinocerebellar Ataxia 1, 2, 3, and 6: A Systematic Review.

Authors:  Dong Hoi Kim; Ryul Kim; Jee Young Lee; Kyoung Min Lee
Journal:  J Clin Neurol       Date:  2021-04       Impact factor: 3.077

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