Caso Francesca, Agosta Federica, Volonté Maria Antonietta, Ferraro Pilar M, Tiraboschi Pietro, Copetti Massimiliano, Valsasina Paola, Falautano Monica, Comi Giancarlo, Falini Andrea, Filippi Massimo
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.
Parkinsonism Relat Disord. 2016 Oct;31:65-71. doi: 10.1016/j.parkreldis.2016.07.007. Epub 2016 Jul 18.
Beside motor symptoms, patients with progressive supranuclear palsy syndrome (PSPs) commonly present cognitive and behavioral disorders. In this study we aimed to assess the structural brain correlates of cognitive impairment in PSPs.
We enrolled 23 patients with probable PSP Richardson's syndrome and 15 matched healthy controls. Patients underwent an extensive clinical and neuropsychological evaluation. Cortical thickness measures and diffusion tensor metrics of white matter tracts were obtained. Random forest analysis was used to identify the strongest MRI predictors of cognitive impairment in PSPs at an individual patient level.
PSPs patients were in a moderate stage of the disease showing mild cognitive deficits with prominent executive dysfunction. Relative to controls, PSPs patients had a focal, bilateral cortical thinning mainly located in the prefrontal/precentral cortex and temporal pole. PSPs patients also showed a distributed white matter damage involving the main tracts including the superior cerebellar peduncle, corpus callosum, corticospinal tract, and extramotor tracts, such as the inferior fronto-occipital, superior longitudinal and uncinate fasciculi, and cingulum, bilaterally. Regional cortical thinning measures did not relate with cognitive features, while white matter damage showed a significant impact on cognitive impairment (r values ranging from -0.80 to 0.74).
PSPs patients show both focal cortical thinning in dorsolateral anterior regions and a distributed white matter damage involving the main motor and extramotor tracts. White matter measures are highly associated with cognitive deficits. Diffusion tensor MRI metrics are likely to be the most sensitive markers of extramotor deficits in PSPs.
除运动症状外，进行性核上性麻痹综合征（PSP）患者通常还存在认知和行为障碍。在本研究中，我们旨在评估PSP患者认知障碍的脑结构相关性。
我们纳入了23例可能患有PSP理查森综合征的患者和15名匹配的健康对照。患者接受了全面的临床和神经心理学评估。获取了皮质厚度测量值和白质束的扩散张量指标。采用随机森林分析在个体患者水平上确定PSP患者认知障碍最强的MRI预测指标。
PSP患者处于疾病的中度阶段，表现出轻度认知缺陷，伴有明显的执行功能障碍。与对照组相比，PSP患者有局灶性双侧皮质变薄，主要位于前额叶/中央前皮质和颞极。PSP患者还表现出弥漫性白质损伤，累及主要神经束，包括双侧上小脑脚、胼胝体、皮质脊髓束和运动外神经束，如下额枕束、上纵束、钩束和扣带束。区域皮质变薄测量值与认知特征无关，而白质损伤对认知障碍有显著影响（r值范围为-0.80至0.74）。
PSP患者表现出背外侧前部区域的局灶性皮质变薄以及累及主要运动和运动外神经束的弥漫性白质损伤。白质测量值与认知缺陷高度相关。扩散张量MRI指标可能是PSP患者运动外缺陷最敏感的标志物。
