Comparing the Pathology, Clinical, and Demographic Characteristics of Younger and Older-Onset Multiple Sclerosis.

Comparing the Pathology, Clinical, and Demographic Characteristics of Younger and Older-Onset Multiple Sclerosis.
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比较年轻和老年发病的多发性硬化症的病理学、临​​床和人口学特征。

DOI:
10.1002/ana.26843
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发表时间:
2023
影响因子:
11.2
通讯作者:
Knowles S
Knowles S
中科院分区:
医学1区
文献类型:
--
作者:
Knowles S

文献摘要

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老年多发性硬化症(MS)患者的放射学和临床表现不太活跃,但许多人仍然达到显着的残疾水平;但是什么导致这一群体的残疾恶化?我们使用来自英国MS登记的数据来描述晚发性多发性硬化症(LOMS;症状发作≥50岁)的人口统计学和临床特征,并与成人发病MS(AOMS;发病18-49岁)进行比较。我们进行了一项病理学研究的一个单独的MS队列与晚发病(n = 18,平均发病年龄54岁)与AOMS(n = 23,平均发病年龄29岁)。ResultsIn登记队列,有1,608(9.4%)与LOMS。与AOMS相比,女性比例较低,原发性进展性MS比例较高,诊断时残疾水平较高(中位MS影响量表为36.7 vs. 28.3,p < 0.001),步态相关初始症状比例较高。LOMS患者不太可能接受高效的疾病改善治疗,并且更快地获得实质性残疾。控制死亡年龄和性别,丘脑和脑桥的神经元密度随发病年龄而降低,而主动脱髓鞘病变和区室化炎症在AOMS中最大。只有神经元密度,而不是脱髓鞘或区室化炎症的程度,与老年发病的MS patients.InterpretationThe老年发病的MS更渐进的性质与显着的神经变性,但罕见的炎性脱髓鞘残疾的结果。这些发现对老年人MS的评估和治疗具有重要意义。神经网络2024;95:471-486
ObjectiveOlder people with multiple sclerosis (MS) have a less active radiological and clinical presentation, but many still attain significant levels of disability; but what drives worsening disability in this group?MethodsWe used data from the UK MS Register to characterize demographics and clinical features of late‐onset multiple sclerosis (LOMS; symptom onset at ≥50 years), compared with adult‐onset MS (AOMS; onset 18–49 years). We performed a pathology study of a separate MS cohort with a later onset (n = 18, mean age of onset 54 years) versus AOMS (n = 23, mean age of onset 29 years).ResultsIn the Register cohort, there were 1,608 (9.4%) with LOMS. When compared with AOMS, there was a lower proportion of women, a higher proportion of primary progressive MS, a higher level of disability at diagnosis (median MS impact scale 36.7 vs. 28.3,p< 0.001), and a higher proportion of gait‐related initial symptoms. People with LOMS were less likely to receive a high efficacy disease‐modifying treatment and attained substantial disability sooner. Controlling for age of death and sex, neuron density in the thalamus and pons decreased with onset‐age, whereas actively demyelinating lesions and compartmentalized inflammation was greatest in AOMS. Only neuron density, and not demyelination or the extent of compartmentalized inflammation, correlated with disability outcomes in older‐onset MS patients.InterpretationThe more progressive nature of older‐onset MS is associated with significant neurodegeneration, but infrequent inflammatory demyelination. These findings have implications for the assessment and treatment of MS in older people. ANN NEUROL 2024;95:471–486