7T磁共振成像下帕金森病、特发性快速眼动睡眠行为障碍及帕金森叠加综合征患者黑质的背侧高信号和铁沉积模式：一项前瞻性诊断研究

Dorsal hyperintensity and iron deposition patterns in the substantia nigra of Parkinson's disease, idiopathic REM sleep behavior disorder, and Parkinson-plus syndromes at 7T MRI: a prospective diagnostic study.

作者信息

Su Dongning, Zhang Zhijin, Zhang Zhe, Yan Rui, Zhu Wanlin, Wei Ning, Suo Yue, Liu Xinyao, Jiang Ying, Ma Lingyan, Zhao Huiqing, Wang Zhan, Wang Xuemei, Ma Huizi, Liu Xin, Wang Chaodong, Wan Zhirong, Li Fangfei, Li Yuan, Lam Joyce S T, Zhou Junhong, Zhang Ning, Wu Tao, Jing Jing, Feng Tao

机构信息

Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.

China National Clinical Research Center for Neurological Diseases, Beijing, China.

出版信息

Transl Neurodegener. 2025 Jul 4;14(1):35. doi: 10.1186/s40035-025-00495-4.

DOI:10.1186/s40035-025-00495-4

PMID:40616129

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12231697/

Abstract

BACKGROUND

Dorsal nigral hyperintensity (DNH) abnormality associated with excessive iron deposition in the substantia nigra, is recognized as an imaging characteristic of Parkinson's disease (PD) and can be effectively visualized using 7T MRI. This study was aimed to develop and validate the optimal DNH assessment method as a biomarker for PD, idiopathic rapid eye movement sleep behavior disorder (iRBD), and Parkinson-plus syndromes, and to explore the nigral iron deposition patterns in these diseases.

METHODS

Three-dimensional gradient-echo T2*-weighted images were acquired by 7T MRI from a total of 402 patients and 100 healthy controls (HCs) in two independent cohorts (development and validation cohorts). Seven methods, including four dichotomous methods and three DNH rating scales, were used to assess DNH and evaluate their diagnostic performance. R2* mapping and principal component analysis were performed to assess nigral iron deposition patterns.

RESULTS

Bilateral DNH detection rates in the development cohort were 22.6% for early-stage PD, 3.7% for advanced PD, 93.5% for iRBD, 5.7% for MSA-parkinsonian type, 78.8% for MSA-cerebellar type, 11.8% for progressive supranuclear palsy (PSP), and 100% for HC, with similar rates in the validation cohort. A cut-off of 6 on the 6-point visibility scale demonstrated a 100% accuracy for diagnosing early-stage PD in both the development and the validation cohorts. This scale exhibited moderate differential diagnostic performance between early-stage PD and iRBD (area under the curve [AUC] = 0.940) or MSA-C (AUC = 0.892). Iron deposition was predominantly in the dorsal and posterior substantia nigra of PD and PSP, the intermediate and posterior substantia nigra of MSA-P, and the ventral substantia nigra of MSA-C.

CONCLUSION

DNH may be preserved in approximately one-quarter of early-stage PD and most MSA-C cases. The 6-point visibility scale on 7T effectively distinguished PD from HC, iRBD, and MSA-C. The nigral iron deposition pattern in PD may help distinguish PD from MSA-P and MSA-C, although it overlaps with that of PSP.

摘要

背景

黑质背侧高信号（DNH）异常与黑质中铁沉积过多相关，被认为是帕金森病（PD）的一种影像学特征，并且可以通过7T磁共振成像（MRI）有效显示。本研究旨在开发并验证作为PD、特发性快速眼动睡眠行为障碍（iRBD）和帕金森叠加综合征生物标志物的最佳DNH评估方法，并探索这些疾病中的黑质铁沉积模式。

方法

通过7T MRI在两个独立队列（开发队列和验证队列）中对总共402例患者和100名健康对照（HC）采集三维梯度回波T2加权图像。使用七种方法，包括四种二分法和三种DNH评分量表，来评估DNH并评估其诊断性能。进行R2映射和主成分分析以评估黑质铁沉积模式。

结果

在开发队列中，早期PD的双侧DNH检出率为22.6%，晚期PD为3.7%，iRBD为93.5%，多系统萎缩帕金森型（MSA-p）为5.7%，多系统萎缩小脑型（MSA-c）为78.8%，进行性核上性麻痹（PSP）为11.8%，HC为100%，验证队列中的比率相似。在6分可见度量表上，截断值为6时在开发队列和验证队列中诊断早期PD的准确率均为100%。该量表在早期PD与iRBD（曲线下面积[AUC]=0.940）或MSA-C（AUC=0.892）之间表现出中等的鉴别诊断性能。铁沉积主要在PD和PSP的黑质背侧和后部，MSA-P的黑质中部和后部，以及MSA-C的黑质腹侧。