Department of Neuroscience, Sorbonne Université, Paris Brain Institute, CNRS, Inserm, 75013 Paris, France.
AP-HP, Hôpital Universitaire Pitié-Salpêtrière, 75013 Paris, France.
Brain. 2024 Apr 4;147(4):1331-1343. doi: 10.1093/brain/awae024.
Cortical myelin loss and repair in multiple sclerosis (MS) have been explored in neuropathological studies, but the impact of these processes on neurodegeneration and the irreversible clinical progression of the disease remains unknown. Here, we evaluated in vivo cortical demyelination and remyelination in a large cohort of people with all clinical phenotypes of MS followed up for 5 years using magnetization transfer imaging (MTI), a technique that has been shown to be sensitive to myelin content changes in the cortex. We investigated 140 people with MS (37 clinically isolated syndrome, 71 relapsing-MS, 32 progressive-MS), who were clinically assessed at baseline and after 5 years and, along with 84 healthy controls, underwent a 3 T-MRI protocol including MTI at baseline and after 1 year. Changes in cortical volume over the radiological follow-up were computed with a Jacobian integration method. Magnetization transfer ratio was employed to calculate for each patient an index of cortical demyelination at baseline and of dynamic cortical demyelination and remyelination over the follow-up period. The three indices of cortical myelin content change were heterogeneous across patients but did not significantly differ across clinical phenotypes or treatment groups. Cortical remyelination, which tended to fail in the regions closer to CSF (-11%, P < 0.001), was extensive in half of the cohort and occurred independently of age, disease duration and clinical phenotype. Higher indices of cortical dynamic demyelination (β = 0.23, P = 0.024) and lower indices of cortical remyelination (β = -0.18, P = 0.03) were significantly associated with greater cortical atrophy after 1 year, independently of age and MS phenotype. While the extent of cortical demyelination predicted a higher probability of clinical progression after 5 years in the entire cohort [odds ratio (OR) = 1.2; P = 0.043], the impact of cortical remyelination in reducing the risk of accumulating clinical disability after 5 years was significant only in the subgroup of patients with shorter disease duration and limited extent of demyelination in cortical regions (OR = 0.86, P = 0.015, area under the curve = 0.93). In this subgroup, a 30% increase in cortical remyelination nearly halved the risk of clinical progression at 5 years, independently of clinical relapses. Overall, our results highlight the critical role of cortical myelin dynamics in the cascade of events leading to neurodegeneration and to the subsequent accumulation of irreversible disability in MS. Our findings suggest that early-stage myelin repair compensating for cortical myelin loss has the potential to prevent neuro-axonal loss and its long-term irreversible clinical consequences in people with MS.
皮质脱髓鞘和修复在多发性硬化症 (MS) 的神经病理学研究中已经得到了探索,但是这些过程对神经退行性变和疾病的不可逆转的临床进展的影响仍然未知。在这里,我们使用磁化传递成像 (MTI) 评估了在经过 5 年随访的具有所有临床表型的 MS 患者的大队列中的皮质脱髓鞘和髓鞘再生,该技术已被证明对皮质髓鞘含量变化敏感。我们研究了 140 名 MS 患者(37 名临床孤立综合征、71 名复发型 MS、32 名进展型 MS),他们在基线和 5 年后进行了临床评估,并与 84 名健康对照者一起进行了 3T-MRI 方案,包括基线和 1 年后的 MTI。使用雅可比积分方法计算了放射随访过程中皮质体积的变化。采用磁化转移比为每位患者计算基线时的皮质脱髓鞘指数以及随访期间的动态皮质脱髓鞘和髓鞘再生指数。三个皮质髓鞘含量变化指数在患者之间存在异质性,但在临床表型或治疗组之间没有显着差异。皮质髓鞘再生在靠近 CSF 的区域趋于失败(-11%,P<0.001),在一半的队列中广泛发生,并且独立于年龄、疾病持续时间和临床表型。较高的皮质动态脱髓鞘指数(β=0.23,P=0.024)和较低的皮质髓鞘再生指数(β=-0.18,P=0.03)与 1 年后皮质萎缩的程度显著相关,独立于年龄和 MS 表型。虽然皮质脱髓鞘的程度预测了整个队列在 5 年后发生临床进展的更高可能性[优势比(OR)=1.2;P=0.043],但皮质髓鞘再生在降低 5 年后累积临床残疾风险方面的影响仅在疾病持续时间较短和皮质区域脱髓鞘程度有限的患者亚组中具有统计学意义(OR=0.86,P=0.015,曲线下面积=0.93)。在该亚组中,皮质髓鞘再生增加 30%几乎将 5 年后的临床进展风险降低了一半,独立于临床复发。总的来说,我们的结果强调了皮质髓鞘动力学在导致神经退行性变和随后的 MS 中不可逆转的残疾累积的事件级联中的关键作用。我们的研究结果表明,早期髓鞘修复补偿皮质髓鞘丢失有可能预防 MS 患者的神经轴突丢失及其长期不可逆的临床后果。