Marxreiter Franz, Lambrecht Vera, Mennecke Angelika, Hanspach Jannis, Jukic Jelena, Regensburger Martin, Herrler Juergen, German Alexander, Kassubek Jan, Grön Georg, Müller Hans-Peter, Laun Frederik B, Dörfler Arnd, Winkler Juergen, Schmidt Manuel A
Department of Molecular Neurology, University Hospital Erlangen, Schwabachanlage 6, 91054 Erlangen, Germany.
Center for Rare Diseases, University Hospital Erlangen, Erlangen, Germany.
Ther Adv Neurol Disord. 2023 Feb 22;16:17562864221143834. doi: 10.1177/17562864221143834. eCollection 2023.
Due to the absence of robust biomarkers, and the low sensitivity and specificity of routine imaging techniques, the differential diagnosis between Parkinson's disease (PD) and multiple system atrophy (MSA) is challenging. High-field magnetic resonance imaging (MRI) opened up new possibilities regarding the analysis of pathological alterations associated with neurodegenerative processes. Recently, we have shown that quantitative susceptibility mapping (QSM) enables visualization and quantification of two major histopathologic hallmarks observed in MSA: reduced myelin density and iron accumulation in the basal ganglia of a transgenic murine model of MSA. It is therefore emerging as a promising imaging modality on the differential diagnosis of Parkinsonian syndromes.
To assess QSM on high-field MRI for the differential diagnosis of PD and MSA.
We assessed 23 patients (nine PDs and 14 MSAs) and nine controls using QSM on 3T and 7T MRI scanners at two academic centers.
We observed increased susceptibility in MSA at 3T in prototypical subcortical and brainstem regions. Susceptibility measures of putamen, pallidum, and substantia nigra reached excellent diagnostic accuracy to separate both synucleinopathies. Increase toward 100% sensitivity and specificity was achieved using 7T MRI in a subset of patients. Magnetic susceptibility correlated with age in all groups, but not with disease duration in MSA. Sensitivity and specificity were particularly high for possible MSA, and reached 100% in the putamen.
Putaminal susceptibility measures, in particular on ultra-high-field MRI, may distinguish MSA patients from both, PD and controls, allowing an early and sensitive diagnosis of MSA.
由于缺乏可靠的生物标志物，且常规成像技术的敏感性和特异性较低，帕金森病（PD）与多系统萎缩（MSA）的鉴别诊断具有挑战性。高场磁共振成像（MRI）为分析与神经退行性过程相关的病理改变开辟了新的可能性。最近，我们已经表明，定量磁化率成像（QSM）能够可视化和量化在MSA中观察到的两个主要组织病理学特征：髓磷脂密度降低和MSA转基因小鼠模型基底神经节中的铁积累。因此，它正在成为一种有前途的成像方式，用于帕金森综合征的鉴别诊断。
评估高场MRI上的QSM对PD和MSA的鉴别诊断价值。
我们在两个学术中心使用3T和7T MRI扫描仪上的QSM对23例患者（9例PD患者和14例MSA患者）和9例对照进行了评估。
我们观察到在3T时，MSA患者典型的皮质下和脑干区域的磁化率增加。壳核、苍白球和黑质的磁化率测量在区分这两种突触核蛋白病方面达到了极佳的诊断准确性。在一部分患者中使用7T MRI可使敏感性和特异性提高至100%。所有组的磁化率均与年龄相关，但在MSA中与病程无关。对于可能的MSA，敏感性和特异性特别高，壳核的敏感性和特异性达到100%。
特别是在超高场MRI上，壳核磁化率测量可能有助于将MSA患者与PD患者及对照区分开来，从而实现MSA的早期和敏感诊断。
