Service de Neurologie, Université de Lille, CHU de Lille, INSERM UMRS_1171, LICEND COEN Center, Lille, France.
Département de Biostastistiques, Université de Lille, CHU de Lille, Lille, France.
Sci Rep. 2018 Jan 29;8(1):1828. doi: 10.1038/s41598-018-19938-2.
For patients with amyotrophic lateral sclerosis (ALS), the primary therapeutic goal is to minimize morbidity. Non-invasive ventilation improves survival. We aim to assess whether Magnetic Resonance Imaging (MRI) of the cervical spinal cord predicts the progression of respiratory disorders in ALS. Brain and spinal MRI was repeatedly performed in the SOD1 mouse model, in 40 patients and in healthy controls. Atrophy, iron overload, white matter diffusivity and neuronal loss were assessed. In Superoxide Dismutase-1 (SOD1) mice, iron accumulation appeared in the cervical spinal cord at symptom onset but disappeared with disease progression (after the onset of atrophy). In ALS patients, the volumes of the motor cortex and the medulla oblongata were already abnormally low at the time of diagnosis. Baseline diffusivity in the internal capsule was predictive of functional handicap. The decrease in cervical spinal cord volume from diagnosis to 3 months was predictive of the change in slow vital capacity at 12 months. MRI revealed marked abnormalities at the time of ALS diagnosis. Early atrophy of the cervical spinal cord may predict the progression of respiratory disorders, and so may be of value in patient care and as a primary endpoint in pilot neuroprotection studies.
对于肌萎缩侧索硬化症（ALS）患者，主要的治疗目标是尽量减少发病率。非侵入性通气可延长生存时间。我们旨在评估颈脊髓磁共振成像（MRI）是否可预测 ALS 患者呼吸障碍的进展。对 SOD1 小鼠模型、40 名患者和健康对照者重复进行了脑和脊髓 MRI 检查。评估了萎缩、铁过载、白质弥散度和神经元丧失。在超氧化物歧化酶 1（SOD1）小鼠中，铁在症状出现时开始积聚在颈脊髓中，但随着疾病的进展而消失（在萎缩开始后）。在 ALS 患者中，运动皮层和延髓的体积在诊断时已经异常降低。内囊的基线弥散度可预测功能障碍。从诊断到 3 个月时颈脊髓体积的减少可预测 12 个月时慢肺活量的变化。MRI 在 ALS 诊断时显示出明显的异常。颈脊髓的早期萎缩可能预示着呼吸障碍的进展，因此可能对患者的护理和作为初步神经保护研究的终点有价值。
