Lee Jae-Hyeok, Lee Myung-Sik
Department of Neurology, Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Pusan National University School of Medicine, Yangsan, South Korea.
Department of Neurology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea.
Front Neurol. 2019 Feb 12;10:74. doi: 10.3389/fneur.2019.00074. eCollection 2019.
Recent data suggest mechanistic links among perturbed iron homeostasis, oxidative stress, and misfolded protein aggregation in neurodegenerative diseases. Iron overload and toxicity toward dopaminergic neurons have been established as playing a role in the pathogenesis of Parkinson's disease (PD). Brain iron accumulation has also been documented in atypical parkinsonian syndromes (APS), mainly comprising multiple system atrophy (MSA), and progressive supranuclear palsy (PSP). Iron-sensitive magnetic resonance imaging (MRI) has been applied to identify iron-related signal changes for the diagnosis and differentiation of these disorders. Topographic patterns of widespread iron deposition in deep brain nuclei have been described as differing between patients with MSA and PSP and those with PD. A disease-specific increase of iron occurs in the brain regions mainly affected by underlying disease pathologies. However, whether iron changes are a primary pathogenic factor or an epiphenomenon of neuronal degeneration has not been fully elucidated. Moreover, the clinical implications of iron-related pathology in APS remain unclear. In this review study, we collected data from qualitative and quantitative MRI studies on brain iron accumulation in APS to identify disease-related patterns and the potential role of iron-sensitive MRI.
近期数据表明，神经退行性疾病中，铁稳态失衡、氧化应激和错误折叠蛋白聚集之间存在机制联系。铁过载以及对多巴胺能神经元的毒性作用已被证实与帕金森病（PD）的发病机制有关。脑铁蓄积在非典型帕金森综合征（APS）中也有记录，主要包括多系统萎缩（MSA）和进行性核上性麻痹（PSP）。铁敏感磁共振成像（MRI）已被用于识别与铁相关的信号变化，以诊断和区分这些疾病。深部脑核中广泛铁沉积的地形模式在MSA和PSP患者与PD患者之间存在差异。在主要受潜在疾病病理影响的脑区会出现特定疾病的铁含量增加。然而，铁变化是主要致病因素还是神经元变性的附带现象尚未完全阐明。此外，APS中铁相关病理的临床意义仍不明确。在这项综述研究中，我们收集了关于APS脑铁蓄积的定性和定量MRI研究数据，以确定与疾病相关的模式以及铁敏感MRI的潜在作用。
