Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy.
Department of Neurology, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy.
J Neurol Neurosurg Psychiatry. 2018 Jul;89(7):696-701. doi: 10.1136/jnnp-2017-317443. Epub 2018 Jan 18.
In this prospective, longitudinal, multiparametric MRI study, we investigated clinical as well as brain grey matter and white matter (WM) regional changes in patients with progressive supranuclear palsy-Richardson's syndrome (PSP-RS).
Twenty-one patients with PSP-RS were evaluated at baseline relative to 36 healthy controls and after a mean follow-up of 1.4 years with clinical rating scales, neuropsychological tests and MRI scans.
Relative to controls, patients with PSP-RS showed at baseline a typical pattern of brain damage, including midbrain atrophy, frontal cortical thinning and widespread WM involvement of the main infratentorial and supratentorial tracts that exceeded cortical damage. Longitudinal study showed that PSP-RS exhibited no further changes in cortical thinning, which remained relatively focal, while midbrain atrophy and WM damage significantly progressed. Corpus callosum and frontal WM tract changes correlated with the progression of both disease severity and behavioural dysfunction.
This study demonstrated the feasibility of carrying out longitudinal diffusion tensor MRI in patients with PSP-RS and its sensitivity to identifying the progression of pathology. Longitudinal midbrain volume loss and WM changes are associated with PSP disease course.
在这项前瞻性、纵向、多参数 MRI 研究中，我们研究了进行性核上性麻痹 - 理查森综合征（PSP-RS）患者的临床以及大脑灰质和白质（WM）区域变化。
21 名 PSP-RS 患者在基线时与 36 名健康对照者进行了评估，并在平均 1.4 年的随访后进行了临床评分量表、神经心理学测试和 MRI 扫描。
与对照组相比，PSP-RS 患者在基线时表现出典型的脑损伤模式，包括中脑萎缩、额皮质变薄和广泛的 WM 累及主要幕下和幕上束，超过皮质损伤。纵向研究表明，PSP-RS 患者的皮质变薄没有进一步变化，仍然相对局限，而中脑萎缩和 WM 损伤则显著进展。胼胝体和额 WM 束的变化与疾病严重程度和行为功能障碍的进展相关。
这项研究证明了在 PSP-RS 患者中进行纵向弥散张量 MRI 的可行性及其对识别病理进展的敏感性。纵向的中脑体积损失和 WM 变化与 PSP 疾病过程相关。
