Evidence map›Paper›PMID 40298952›Full record

ArticleNeuroradiology2025

MRI-based brain structural changes in adult-onset dentatorubral-pallidoluysian atrophy.

Mengcheng Li, Xinyuan Chen, Ruying Yuan, Shuping Fan, Ziqiang Huang, Zhenyi Liu, Jiaqi Weng, Qiaozhen Zheng, Shirui Gan, Jianping Hu

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Article in Neuroradiology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.

0numbers the graph read from it
0cells of the map it votes in
3citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

3 citing papers in PubMed.

  1. Article
  2. Atypical subacute sclerosing panencephalitis mimicking mitochondrial encephalopathy, lactic acidosis and stroke-like episodes.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2026
    Article
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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

10 authors.

Mengcheng LiDepartment of Radiology, The First Affiliated Hospital of Fujian Medical University, 350005, Fuzhou, China.
Xinyuan ChenDepartment of Rehabilitation Medicine, The First Affiliated Hospital of Fujian Medical University, 350005, Fuzhou, China.
Ruying YuanDepartment of Neurology, The First Affiliated Hospital, Institute of Neuroscience, Fujian Key Laboratory of Molecular Neurology, Fujian Medical University, 350005, Fuzhou, China.
Shuping FanDepartment of Radiology, The First Affiliated Hospital of Fujian Medical University, 350005, Fuzhou, China.
Ziqiang HuangDepartment of Radiology, The First Affiliated Hospital of Fujian Medical University, 350005, Fuzhou, China.
Zhenyi LiuDepartment of Radiology, The First Affiliated Hospital of Fujian Medical University, 350005, Fuzhou, China.
Jiaqi WengDepartment of Radiology, The First Affiliated Hospital of Fujian Medical University, 350005, Fuzhou, China.
Qiaozhen ZhengDepartment of Radiology, The First Affiliated Hospital of Fujian Medical University, 350005, Fuzhou, China.
Shirui GanDepartment of Neurology, The First Affiliated Hospital, Institute of Neuroscience, Fujian Key Laboratory of Molecular Neurology, Fujian Medical University, 350005, Fuzhou, China. ganshirui@fjmu.edu.cn.
Jianping HuDepartment of Radiology, The First Affiliated Hospital of Fujian Medical University, 350005, Fuzhou, China. fmrihjp@163.com.

Funding

Joint Funds for the Innovation of Science and Technology, Fujian Province 2024Y9202
6 · The paper itself

Abstract

background and purposeAdult-onset dentatorubral-pallidoluysian atrophy (DRPLA) is a rare autosomal dominant spinocerebellar ataxia subtype, characterized by widespread brain neurodegeneration. However, comprehensive studies on whole-brain structural changes remain limited. This study aimed to quantify brain structural damage in adult-onset DRPLA and correlate these findings with clinical data.

methodsTwenty-four adult-onset DRPLA patients were recruited and underwent structural and diffusion MRI at 3.0 Tesla. Multimodal analyses were conducted to assess cortical thickness, the volumes of subcortical structures, the brainstem, cerebellum, and cerebral white matter (WM), as well as the microstructural integrity of WM. Results were compared with those from 30 age- and gender-matched healthy controls. The relationships between neuroimaging abnormalities and clinical data, including ataxia severity, cognitive performance, CAG repeat length, and disease duration, were explored.

resultsIn adult-onset DRPLA patients, reduced cortical thickness was observed in the bilateral frontal, parietal, temporal, and occipital lobes. Volumetric analysis revealed significant reductions in all subcortical structures, as well as in the brainstem, cerebellum, and cerebral WM substructures. Tract-based spatial statistics (TBSS) analysis of diffusion tensor imaging (DTI) and neurite orientation dispersion and density imaging (NODDI) metrics demonstrated widespread WM abnormalities throughout the brain. Furthermore, several imaging measures demonstrated significant correlations with ataxia severity, cognitive function scores, CAG repeat length, and disease duration.

conclusionDRPLA should be considered a multisystem neurodegenerative disease, characterized by widespread and severe gray matter and WM atrophy throughout the brain. The advanced MRI techniques employed in this study expand our understanding of the underlying pathophysiological mechanisms.

Indexed as

BrainMagnetic Resonance ImagingMyoclonic Epilepsies, ProgressiveAdultCase-Control StudiesDiffusion Tensor ImagingFemaleHumansMaleMiddle AgedDRPLADTINODDISpinocerebellar ataxiaStructural MRI

Identifiers

PMID40298952

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