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帕金森病伴 REM 睡眠行为障碍患者出现对称性和显著的单胺能神经退行性变。

Symmetric and Profound Monoaminergic Degeneration in Parkinson's Disease with Premotor REM Sleep Behavior Disorder.

机构信息

Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.

Department of Nuclear Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.

出版信息

J Parkinsons Dis. 2024;14(4):823-831. doi: 10.3233/JPD-230459.

DOI:10.3233/JPD-230459
PMID:38640171
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11191437/
Abstract

BACKGROUND

Rapid eye movement sleep behavior disorder (RBD) may precede or follow motor symptoms in Parkinson's disease (PD). While over 70% of idiopathic RBD cases phenoconvert within a decade, a small subset develops PD after a more extended period or remains nonconverted. These heterogeneous manifestations of RBD in PD prompt subtype investigations. Premotor RBD may signify "body-first" PD with bottom-up, symmetric synucleinopathy propagation.

OBJECTIVE

Explore brainstem and nigrostriatal monoaminergic degeneration pattern differences based on premotor RBD presence and duration in de novo PD patients.

METHODS

In a cross-sectional analysis of de novo PD patients (n = 150) undergoing FP-CIT PET and RBD Single-Question Screen, the cohort was categorized into groups with and without premotor RBD (PDRBD +/-), with further classification of PDRBD + based on a 10-year duration of premotor RBD. Analysis of FP-CIT binding in the striatum and pons, striatal asymmetry, and striatum-to-pons ratios compared patterns of nigrostriatal and brainstem monoaminergic degeneration.

RESULTS

PDRBD + exhibited more severe and symmetrical striatal dopaminergic denervation compared to PDRBD-, with the difference in severity accentuated in the least-affected hemisphere. The PDRBD +<10Y subgroup displayed the most prominent striatal symmetry, supporting a more homogeneous "body-first" subtype. Pontine uptakes remained lower in PDRBD + even after adjusting for striatal uptake, suggesting early degeneration of pontine monoaminergic nuclei.

CONCLUSIONS

Premotor RBD in PD is associated with severe, symmetrical nigrostriatal and brainstem monoaminergic degeneration, especially in cases with PD onset within 10 years of RBD. This supports the concept of a "widespread, bottom-up" pathophysiological mechanism associated with premotor RBD in PD.

摘要

背景

快速眼动睡眠行为障碍(RBD)可能先于或后于帕金森病(PD)的运动症状出现。虽然超过 70%的特发性 RBD 病例在十年内出现表型转化,但一小部分病例在更长时间后发展为 PD 或仍未转化。这些 PD 中 RBD 的异质表现促使进行亚型研究。前驱期 RBD 可能标志着“身体首先”的 PD,具有自下而上、对称的突触核蛋白病传播。

目的

根据新发 PD 患者中前驱期 RBD 的存在和持续时间,探索脑干和黑质纹状体单胺能退行性变模式的差异。

方法

在一项对接受 FP-CIT PET 和 RBD 单问题筛查的新发 PD 患者(n = 150)的横断面分析中,该队列根据是否存在前驱期 RBD(PDRBD +/ -)进行分组,进一步根据前驱期 RBD 的 10 年持续时间对 PDRBD + 进行分类。分析纹状体和脑桥的 FP-CIT 结合,纹状体不对称,以及纹状体-脑桥比值,比较黑质纹状体和脑干单胺能退行性变的模式。

结果

与 PDRBD-相比,PDRBD + 表现出更严重和更对称的纹状体多巴胺能脱失,并且在受影响较小的半球中,严重程度的差异更加明显。PDRBD +<10Y 亚组显示出最明显的纹状体对称性,支持更同质的“身体首先”亚型。即使在调整纹状体摄取后,PDRBD + 的脑桥摄取仍然较低,表明脑桥单胺能核早期退化。

结论

PD 中的前驱期 RBD 与严重、对称的黑质纹状体和脑干单胺能退行性变相关,尤其是在 RBD 出现后 10 年内 PD 发病的病例中。这支持了与 PD 中前驱期 RBD 相关的“广泛、自下而上”病理生理机制的概念。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c68c/11191437/8579622956ad/jpd-14-jpd230459-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c68c/11191437/8579622956ad/jpd-14-jpd230459-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c68c/11191437/8579622956ad/jpd-14-jpd230459-g001.jpg

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