Stork Lidia, Stephan Schirin, Kutllovci Adriane, Brück Wolfgang, Metz Imke
Institute of Neuropathology, University Medical Center Göttingen, Robert-Koch-Str, 40, 37075, Göttingen, Germany.
Acta Neuropathol. 2025 Apr 1;149(1):30. doi: 10.1007/s00401-025-02868-5.
A reduced regenerative capacity may contribute to faster disease progression and poorer relapse recovery in multiple sclerosis patients with disease onset after the age of 50, a condition known as late-onset multiple sclerosis (LOMS). We hypothesized that lesions in LOMS patients show more pronounced axonal damage, less remyelination and an altered inflammatory composition, and performed a detailed histopathological analysis of MS biopsies in patients with early-stage LOMS. The number of T cells, B cells, plasma cells, microglia/macrophages, different oligodendrocyte populations as well as the axonal density and acute axonal damage were assessed in 31 LOMS and 30 normal-onset MS (NOMS, 20-40 years old) patients. No major differences in the inflammatory infiltrate or axonal damage were found. BCAS1-positive oligodendrocytes indicating early myelinating oligodendrocytes, and mature oligodendrocytes were significantly lower in the normal-appearing white matter of LOMS compared to NOMS patients (p = 0.05; p = 0.01), with a negative correlation with age (r = - 0.5, p = 0.01). In active demyelinating lesions, the number of BCAS1-positive oligodendrocytes did not differ between LOMS and NOMS, but NOMS lesions showed a higher proportion of ramified cells indicating active remyelination. In LOMS, BCAS1-positive oligodendrocytes decreased with increasing lesion age, with the lowest numbers found in inactive demyelinated lesions. In contrast, NOMS patients showed high numbers of BCAS1-positive cells with an activated morphology, even in inactive demyelinated lesions. At the last follow-up, LOMS patients had a significantly higher EDSS score (median 3.5) than NOMS patients (median 3.0, p = 0.05). A higher EDSS score correlated with fewer mature and oligodendrocyte precursor cells in active demyelinating lesions (r = - 0.4, p = 0.01 and r = - 0.6, p = 0.003). These findings suggest a clinically relevant impaired oligodendrocyte differentiation and remyelination in LOMS. Since remyelination is essential for axonal protection, it will be necessary to consider the complex and dynamic tissue environment when researching therapeutics aimed at fostering the differentiation of oligodendrocyte precursor cells into myelinating oligodendrocytes.
再生能力降低可能导致50岁后发病的多发性硬化症患者疾病进展更快且复发恢复情况更差,这种情况被称为晚发性多发性硬化症(LOMS)。我们推测,LOMS患者的病灶显示出更明显的轴突损伤、更少的髓鞘再生以及炎症成分改变,并对早期LOMS患者的多发性硬化症活检组织进行了详细的组织病理学分析。评估了31例LOMS患者和30例正常发病的多发性硬化症(NOMS,20 - 40岁)患者的T细胞、B细胞、浆细胞、小胶质细胞/巨噬细胞数量、不同少突胶质细胞群体以及轴突密度和急性轴突损伤情况。在炎症浸润或轴突损伤方面未发现主要差异。与NOMS患者相比,LOMS患者正常外观白质中表明早期髓鞘形成少突胶质细胞的BCAS1阳性少突胶质细胞和成熟少突胶质细胞显著减少(p = 0.05;p = 0.01),且与年龄呈负相关(r = - 0.5,p = 0.01)。在活动性脱髓鞘病灶中,LOMS和NOMS患者的BCAS1阳性少突胶质细胞数量没有差异,但NOMS病灶中显示出更高比例的分支状细胞,表明有活跃的髓鞘再生。在LOMS中,BCAS1阳性少突胶质细胞随病灶年龄增加而减少,在非活动性脱髓鞘病灶中数量最少。相比之下,即使在非活动性脱髓鞘病灶中,NOMS患者也有大量具有活化形态的BCAS1阳性细胞。在最后一次随访时,LOMS患者的扩展残疾状态量表(EDSS)评分(中位数3.5)显著高于NOMS患者(中位数3.0,p = 0.05)。更高的EDSS评分与活动性脱髓鞘病灶中成熟和少突胶质前体细胞数量减少相关(r = - 0.4,p = 0.01和r = - 0.6,p = 0.003)。这些发现表明,LOMS中存在与临床相关的少突胶质细胞分化和髓鞘再生受损情况。由于髓鞘再生对轴突保护至关重要,在研究旨在促进少突胶质前体细胞分化为髓鞘形成少突胶质细胞的治疗方法时,有必要考虑复杂且动态的组织环境。