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.
Eur J Neurol. 2019 Sep;26(9):1143-1152. doi: 10.1111/ene.13957. Epub 2019 Apr 20.
This study aimed to assess the predictive value of multimodal brain magnetic resonance imaging (MRI) on survival in a large cohort of patients with motor neuron disease (MND), in combination with clinical and cognitive features.
Two hundred MND patients were followed up prospectively for a median of 4.13 years. At baseline, subjects underwent neurological examination, cognitive assessment and brain MRI. Grey matter volumes of cortical and subcortical structures and diffusion tensor MRI metrics of white matter tracts were obtained. A multivariable Royston-Parmar survival model was created using clinical and cognitive variables. The increase of survival prediction accuracy provided by MRI variables was assessed.
The multivariable clinical model included predominant upper or lower motor neuron presentations and diagnostic delay as significant prognostic predictors, reaching an area under the receiver operating characteristic curve (AUC) of a 4-year survival prediction of 0.79. The combined clinical and MRI model including selected grey matter fronto-temporal volumes and diffusion tensor MRI metrics of the corticospinal and extra-motor tracts reached an AUC of 0.89. Considering amyotrophic lateral sclerosis patients only, the clinical model including diagnostic delay and semantic fluency scores provided an AUC of 0.62, whereas the combined clinical and MRI model reached an AUC of 0.77.
Our study demonstrated that brain MRI measures of motor and extra-motor structural damage, when combined with clinical and cognitive features, are useful predictors of survival in patients with MND, particularly when a diagnosis of amyotrophic lateral sclerosis is made.
本研究旨在评估多模态脑磁共振成像（MRI）结合临床和认知特征对大量运动神经元病（MND）患者生存的预测价值。
200 例 MND 患者前瞻性随访，中位随访时间为 4.13 年。基线时，患者接受神经学检查、认知评估和脑 MRI。获得皮质和皮质下结构的灰质体积以及白质束的弥散张量 MRI 指标。使用临床和认知变量创建多变量 Royston-Parmar 生存模型。评估 MRI 变量对生存预测准确性的提高。
多变量临床模型包括主要的上运动神经元或下运动神经元表现和诊断延迟作为重要的预后预测因子，达到 4 年生存率预测的 0.79 的受试者工作特征曲线（ROC）曲线下面积。包括选定的额颞叶灰质体积和皮质脊髓束及运动外束的弥散张量 MRI 指标的临床和 MRI 综合模型达到 0.89 的 AUC。仅考虑肌萎缩侧索硬化症患者，包括诊断延迟和语义流畅性评分的临床模型提供了 0.62 的 AUC，而临床和 MRI 综合模型达到了 0.77 的 AUC。
我们的研究表明，大脑 MRI 对运动和运动外结构损伤的测量，结合临床和认知特征，是 MND 患者生存的有用预测指标，特别是在做出肌萎缩侧索硬化症诊断时。
