Jost Wolfgang H, Rau Alexander, Brumberg Joachim, Urbach Horst, Meyer Philipp T, Schröter Nils
Parkinson-Klinik Ortenau, Kreuzbergstraße 12-16, 77709, Wolfach, Germany.
Department of Neurology, Saarland University Medical Center, Homburg, Germany.
J Neural Transm (Vienna). 2025 Apr 11. doi: 10.1007/s00702-025-02921-3.
Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by cerebellar dysfunction, a Parkinsonian syndrome with poor response to levodopa and autonomic failure. The diagnosis of MSA is particularly challenging in its early stages due to symptom overlap with other neurodegenerative Parkinson syndromes. Recent advances in neuroimaging have greatly improved the accuracy of the diagnosis in clinical routine and provided valuable insights into the pathophysiology and progression of MSA. Macrostructural MRI shows atrophy in regions such as the putamen and pontocerebellar regions, distinguishing MSA from other Parkinson syndromes. Advanced imaging techniques, including diffusion tensor imaging (DTI), free water imaging and quantitative susceptibility mapping, add further value in assessing disease progression. While dopamine transporter (DAT) imaging is the mainstay for confirmation of nigrostriatal degeneration in suspected neurodegenerative Parkinson syndromes and may enable to identify prodromal cases, cardiac sympathetic imaging with [123I]MIBG scintigraphy may be used for delineation of MSA from Parkinson's disease (PD). Positron emission tomography (PET) with the glucose analogue [F]FDG depicts disease-specific metabolic patterns in MSA and various neurodegenerative diseases, which do not only enable a highly accurate differential diagnosis of MSA (e.g., from PD and other atypical Parkinson syndromes) but also carry important prognostic and pathophysiological information. Various other PET radiopharmaceuticals currently under investigation in MSA provide novel insights into neurotransmitter system changes, glial pathology and, most recently, α-synuclein pathology. These imaging modalities considerably expand the diagnostic and prognostic capabilities in MSA and may provide important biomarkers for tracking disease development, progression and treatment.
多系统萎缩（MSA）是一种神经退行性疾病，其特征为小脑功能障碍、对左旋多巴反应不佳的帕金森综合征以及自主神经功能衰竭。由于MSA在早期阶段的症状与其他神经退行性帕金森综合征重叠，因此其诊断极具挑战性。神经影像学的最新进展极大地提高了临床常规诊断的准确性，并为MSA的病理生理学和病程发展提供了有价值的见解。宏观结构MRI显示壳核和脑桥小脑区域等部位萎缩，这有助于将MSA与其他帕金森综合征区分开来。包括扩散张量成像（DTI）、自由水成像和定量磁化率映射在内的先进成像技术在评估疾病进展方面具有更大价值。虽然多巴胺转运体（DAT）成像在疑似神经退行性帕金森综合征中是确认黑质纹状体变性的主要手段，并且可能有助于识别前驱病例，但[123I]间碘苄胍闪烁显像的心脏交感神经成像可用于区分MSA和帕金森病（PD）。使用葡萄糖类似物[F]氟代脱氧葡萄糖的正电子发射断层扫描（PET）描绘了MSA和各种神经退行性疾病中特定疾病的代谢模式，这不仅有助于对MSA进行高度准确的鉴别诊断（例如与PD和其他非典型帕金森综合征相鉴别），还携带重要的预后和病理生理信息。目前正在MSA研究中的各种其他PET放射性药物为神经递质系统变化、胶质细胞病理学以及最近的α-突触核蛋白病理学提供了新的见解。这些成像方式极大地扩展了MSA的诊断和预后能力，并可能为追踪疾病发展、进展和治疗提供重要的生物标志物。
