Department of Neurology, Mayo Clinic, Rochester, MN, USA.
Department of Radiology, Mayo Clinic, Rochester, MN, USA.
Mult Scler. 2021 Jun;27(7):1037-1045. doi: 10.1177/1352458520948745. Epub 2020 Aug 19.
New inflammatory activity is of unclear frequency and clinical significance in progressive multiple sclerosis (MS); it is uncertain in patient cohorts with motor progression due to critical demyelinating lesions.
The aim of this study is to determine the likelihood of central nervous system (CNS) inflammatory activity, assessed by new clinical relapses or active magnetic resonance imaging (MRI) lesions, following onset of motor progression due to critical demyelinating lesions.
Patients with progressive upper motor neuron impairment for ⩾1 year attributable to critical demyelinating lesions with single CNS lesion (progressive solitary sclerosis (PSS)), 2 to 5 total CNS demyelinating lesions (progressive "pauci-sclerosis" (PPS)), or >5 CNS demyelinating lesions and progressive exclusively unilateral monoparesis or hemiparesis (PUHMS) were identified. Clinical data were reviewed for acute MS relapses, and subsequent MRI was reviewed for active T1-gadolinium-enhancing or T2-demyelinating lesions.
None of the 91 patients (22 PSS, 40 PPS, 29 PUHMS) identified experienced clinical relapses over a median clinical follow-up of 93 months (range: 12-518 months). Nine patients (10%) developed active lesions over median 84 months radiologic follow-up (range: 12-518 months). Active lesions occurred in 24% PUHMS, 5% PSS, and 3% PPS cohorts.
New inflammatory activity, defined by active lesions and clinical relapses following motor progression in patients with critical demyelinating lesions, is low. Disease-modifying therapies that reduce demyelinating relapses and active MRI lesions are of uncertain benefit in these cohorts.
新的炎症活动在进展性多发性硬化症（MS）中频率和临床意义尚不清楚；在由于严重脱髓鞘病变导致运动进展的患者队列中，情况并不明确。
本研究旨在确定因严重脱髓鞘病变导致单一中枢神经系统（CNS）病变（进行性单纯性硬化症（PSS））、2 至 5 个总 CNS 脱髓鞘病变（进行性“少硬化症”（PPS））或 >5 个 CNS 脱髓鞘病变和进行性单侧偏瘫或单瘫（PUHMS）的运动进展后，中枢神经系统炎症活动的可能性，其通过新的临床复发或活跃的磁共振成像（MRI）病变来评估。
确定了因严重脱髓鞘病变导致 ⩾1 年的进行性上运动神经元损害患者，这些患者有单一 CNS 病变（进行性单纯性硬化症（PSS））、2 至 5 个总 CNS 脱髓鞘病变（进行性“少硬化症”（PPS））或 >5 个 CNS 脱髓鞘病变和进行性单侧偏瘫或单瘫（PUHMS）。回顾了临床数据以评估急性 MS 复发，随后回顾了后续 MRI 以评估活跃的 T1-钆增强或 T2-脱髓鞘病变。
在中位临床随访 93 个月（范围：12-518 个月）期间，91 例患者（22 例 PSS、40 例 PPS、29 例 PUHMS）均未发生临床复发。在中位 84 个月的放射学随访期间（范围：12-518 个月），9 例患者（10%）出现活跃病变。活跃病变出现在 24%的 PUHMS、5%的 PSS 和 3%的 PPS 患者中。
新的炎症活动，定义为在有严重脱髓鞘病变的患者中，运动进展后出现活跃病变和临床复发，其频率较低。在这些队列中，减少脱髓鞘复发和活跃 MRI 病变的疾病修正疗法的获益尚不确定。
