Department of Neurology, College of Medicine, Mayo Clinic, Rochester, MN, USA/Department of Neurology and Rehabilitation Medicine, University of Cincinnati, Cincinnati, OH, USA.
Department of Neurology, College of Medicine, Mayo Clinic, Rochester, MN, USA.
Mult Scler. 2021 May;27(6):895-902. doi: 10.1177/1352458520940983. Epub 2020 Jul 15.
To compare progressive motor impairment onset attributable to a "critical" central nervous system (CNS) demyelinating lesion in patients with highly restricted versus unlimited magnetic resonance imaging (MRI) lesion burden.
We identified 135 patients with progressive motor impairment for ⩾1 year attributable to a "critical" demyelinating lesion with: MRI burden of 1 lesion ("progressive solitary sclerosis"), 2-5 lesions ("progressive paucisclerosis"), or unrestricted (>5) lesions and "progressive unilateral hemiparesis." Neuroradiology review of brain and spinal cord MRI documented unequivocally demyelinating lesions.
A total of 33 (24.4%) patients had progressive solitary sclerosis; 56 (41.5%) patients had progressive paucisclerosis; and 46 (34.1%) patients had progressive unilateral hemiparesis. Median age at onset of progressive motor impairment was younger in progressive solitary sclerosis (49 years; range 24-73) and progressive paucisclerosis (50 years; range 30-64) than in progressive unilateral hemiparesis (54 years; range 39-77; = 0.02 and = 0.003, respectively). Within progressive unilateral hemiparesis, motor-progression onset was similar between those with 4-10, 11-20, or >20 brain lesions (55, 54, 53 years of age, respectively; = 0.44).
Motor-progression age is similar, but paradoxically earlier, in cohorts with highly restricted CNS lesion burden than in those with unrestricted lesion burden with progressive unilateral hemiparetic MS. The "critical" demyelinating lesion rather than total brain MRI lesion burden is the major contributor to motor-progression onset in these cohorts.
比较在高限制中枢神经系统（CNS）脱髓鞘病变与无限制磁共振成像（MRI）病变负担的患者中，归因于“关键”脱髓鞘病变的进行性运动障碍发病。
我们确定了 135 名进行性运动障碍≥1 年的患者，其归因于具有以下特征的“关键”脱髓鞘病变：1 个病变的 MRI 负担（“进行性单发硬化症”）、2-5 个病变（“进行性少发性硬化症”）或无限制（>5 个）病变和“进行性单侧偏瘫”。脑和脊髓 MRI 的神经影像学复查明确记录了脱髓鞘病变。
共有 33 名（24.4%）患者患有进行性单发硬化症；56 名（41.5%）患者患有进行性少发性硬化症；46 名（34.1%）患者患有进行性单侧偏瘫。进行性运动障碍发病的中位年龄在进行性单发硬化症（49 岁；范围 24-73）和进行性少发性硬化症（50 岁；范围 30-64）中较进行性单侧偏瘫（54 岁；范围 39-77）更年轻（ = 0.02 和 = 0.003）。在进行性单侧偏瘫中，4-10 个、11-20 个或>20 个脑病变患者的运动进展发病年龄相似（分别为 55、54、53 岁； = 0.44）。
与无限制病变负担的进行性单侧偏瘫患者相比，具有高度限制中枢神经系统病变负担的患者的运动障碍发病年龄相似，但更具悖论性。在这些队列中，“关键”脱髓鞘病变而不是脑 MRI 总病变负担是运动进展发病的主要原因。
