Department of Neurology, University of Ulm, Ulm, Germany.
Hum Brain Mapp. 2010 Nov;31(11):1727-40. doi: 10.1002/hbm.20971.
Different motor neuron disorders (MNDs) are mainly defined by the clinical presentation based on the predominance of upper or lower motor neuron impairment and the course of the disease. Magnetic resonance imaging (MRI) mostly serves as a tool to exclude other pathologies, but novel approaches such as diffusion tensor imaging (DTI) have begun to add information on the underlying pathophysiological processes of these disorders in vivo. The present study was designed to investigate three different rare MNDs, i.e., primary lateral sclerosis (PLS, N = 25), hereditary spastic paraparesis (HSP, N = 24), and X-linked spinobulbar muscular atrophy (X-SBMA, N = 20), by use of whole-brain-based DTI analysis in comparison with matched controls. This analysis of white matter (WM) impairment revealed widespread and characteristic patterns of alterations within the motor system with a predominant deterioration of the corticospinal tract (CST) in HSP and PLS patients according to the clinical presentation and also in patients with X-SBMA to a lesser degree, but also WM changes in projections to the limbic system and within distinct areas of the corpus callosum (CC), the latter both for HSP and PLS. In summary, DTI was able to define a characteristic WM pathoanatomy in motor and extra-motor brain areas, such as the CC and the limbic projectional system, for different MNDs via whole brain-based FA assessment and quantitative fiber tracking. Future advanced MRI-based investigations might help to provide a fingerprint-identification of MNDs.
不同的运动神经元疾病（MNDs）主要根据临床表现定义，这些表现基于上运动神经元或下运动神经元损伤的优势以及疾病的进程。磁共振成像（MRI）主要用作排除其他病理学的工具，但像弥散张量成像（DTI）这样的新方法已开始提供这些疾病在体内潜在病理生理过程的信息。本研究旨在通过全脑弥散张量成像（DTI）分析，比较三种不同的罕见 MND，即原发性侧索硬化症（PLS，N=25）、遗传性痉挛性截瘫（HSP，N=24）和 X 连锁脊髓延髓肌萎缩症（X-SBMA，N=20），与匹配的对照组进行比较。这种对脑白质（WM）损伤的分析揭示了运动系统内广泛存在的特征性改变模式，根据临床表现，HSP 和 PLS 患者的皮质脊髓束（CST）明显恶化，X-SBMA 患者也有一定程度的恶化，但也有 WM 改变位于边缘系统的投射区和胼胝体（CC）内的特定区域，这两种情况在 HSP 和 PLS 患者中都存在。总之，DTI 通过全脑 FA 评估和定量纤维追踪，能够在不同的 MND 中定义运动和运动外脑区（如 CC 和边缘投射系统）的特征性 WM 病理解剖结构。未来基于先进 MRI 的研究可能有助于提供 MND 的指纹识别。
