成年晚期髓鞘少突胶质细胞糖蛋白抗体相关疾病的临床特征和预后相关因素

IF 7.7 1区 医学 Q1 CLINICAL NEUROLOGY
Alessandro Dinoto,Laura Cacciaguerra,Nisa Vorasoot,Vyanka Redenbaugh,Sebastian A Lopez-Chiriboga,Cristina Valencia-Sanchez,Kai Guo,Smathorn Thakolwiboon,Susan E Horsman,Stephanie B Syc-Mazurek,Nanthaya Tisavipat,Jay Mandrekar,Deena Tajfirouz,Eric R Eggenberger,Misha L Pless,Kevin Chodnicki,Jan-Mendelt Tillema,Sean J Pittock,John Jing-Wei Chen,Eoin P Flanagan
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引用次数: 0

摘要

背景和目的关于成年晚期髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)的数据很少。本研究旨在评估成人晚期MOGAD的频率、特征和结果,并确定其与早期MOGAD的差异。方法:这项回顾性观察性研究纳入了梅奥诊所2023诊断标准的MOGAD患者,发病年龄在50岁或以上。收集了50-59岁和60岁及以上患者的临床、实验室、放射学、治疗和结局数据并进行了比较。最后,将成人晚发型MOGAD患者(50岁或以上)的特征和预后与成人早发型MOGAD患者(18-49岁)的对照组(n = 141)进行比较。结果纳入107例成人晚发型MOGAD患者,占MOGAD队列的25% (n = 436)。中位发病年龄为59岁(范围:50-88岁),其中71例(66%)为女性。105例患者中有86例(83%)存在医学合并症。视神经炎是最常见的发作(77/107,72%),最低时扩展残疾状态量表(EDSS)得分中位数为3(范围:1-9)。107例中有32例(30%)指出了潜在的触发因素,主要是感染。在32例(30%)患者中,在确诊MOGAD之前考虑了其他诊断,最常见的是巨细胞动脉炎(n = 15[11例行颞动脉活检])。中位随访时间为22个月(范围:0-306个月),50名患者(47%)出现复发,53名患者(50%)接受了预防性治疗。药物副作用较为常见(41/107,38%)。50-59岁的MOGAD患者与60岁及以上的MOGAD患者无差异。最后,成年晚期发病的患者有更多的视神经受累(p = 0.015;OR: 1.9;95% CI 1.1-3.3),脑干/小脑受累(p = 0.008;OR: 3.4;95% CI 1.1-3.3),认知能力下降(p = 0.01;OR: 5.3;95% CI 1.4-19.4)和较少的脊髓炎(p < 0.001;OR: 0.4;(95% CI 0.2-0.7)与早期成人发病MOGAD患者比较,但EDSS评分和复发频率相似。成人晚期MOGAD占MOGAD患者的四分之一。视神经炎是该年龄组MOGAD的主要表型,未被充分认识并经常误诊。成人晚期发病MOGAD的结局与早期发病MOGAD相似。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Clinical Features and Factors Associated With Outcome in Late Adult-Onset Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease.
BACKGROUND AND OBJECTIVES Data regarding late adult-onset myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are scant. This study sought to assess the frequency, characteristics, and outcome of late adult-onset MOGAD and identify differences from early adult-onset MOGAD. METHODS This retrospective, observational study included Mayo Clinic patients with MOGAD per 2023 diagnostic criteria and with onset age 50 years or older. Clinical, laboratory, radiologic, treatment, and outcome data were collected and compared between patients aged 50-59 years and those aged 60 years or older. Finally, the characteristics and outcome of patients with late adult-onset MOGAD (aged 50 or older) were compared with a reference group of patients with early adult-onset (aged 18-49) MOGAD (n = 141). RESULTS There were 107 patients with late adult-onset MOGAD included, representing 25% of the MOGAD cohort (n = 436). The median age at onset was 59 years (range: 50-88), and 71 (66%) were female. Medical comorbidities were noted in 86 of 105 (83%). Optic neuritis was the most frequent onset attack (77/107, 72%), and the median Expanded Disability Status Scale (EDSS) score at nadir was 3 (range: 1-9). In 32 of 107 (30%), a potential trigger was noted, mostly infections. In 32 patients (30%), an alternative diagnosis was considered before MOGAD identification, most commonly giant cell arteritis (n = 15 [11 undergoing temporal artery biopsy]). By a median follow-up duration of 22 months (range: 0-306), 50 patients (47%) had a relapsing course, and preventive treatment was administered in 53 (50%). Medication side effects were common (41/107, 38%). No differences were observed in patients with MOGAD aged 50-59 years vs 60 years or older. Lastly, late adult-onset patients had more optic nerve involvement (p = 0.015; OR: 1.9; 95% CI 1.1-3.3), brainstem/cerebellar involvement (p = 0.008; OR: 3.4; 95% CI 1.1-3.3), and cognitive decline (p = 0.01; OR: 5.3; 95% CI 1.4-19.4) and less frequent myelitis (p < 0.001; OR: 0.4; 95% CI 0.2-0.7) vs those with early adult-onset MOGAD, but EDSS scores and frequency of a relapsing course were similar. DISCUSSION Late adult-onset MOGAD accounts for one-quarter of patients with MOGAD. Optic neuritis is the dominant phenotype in this age group with MOGAD and is under-recognized and frequently misdiagnosed. Outcomes in late adult-onset MOGAD are similar to those in early adult-onset disease.
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来源期刊
Neurology
Neurology 医学-临床神经学
CiteScore
12.20
自引率
4.00%
发文量
1973
审稿时长
2-3 weeks
期刊介绍: Neurology, the official journal of the American Academy of Neurology, aspires to be the premier peer-reviewed journal for clinical neurology research. Its mission is to publish exceptional peer-reviewed original research articles, editorials, and reviews to improve patient care, education, clinical research, and professionalism in neurology. As the leading clinical neurology journal worldwide, Neurology targets physicians specializing in nervous system diseases and conditions. It aims to advance the field by presenting new basic and clinical research that influences neurological practice. The journal is a leading source of cutting-edge, peer-reviewed information for the neurology community worldwide. Editorial content includes Research, Clinical/Scientific Notes, Views, Historical Neurology, NeuroImages, Humanities, Letters, and position papers from the American Academy of Neurology. The online version is considered the definitive version, encompassing all available content. Neurology is indexed in prestigious databases such as MEDLINE/PubMed, Embase, Scopus, Biological Abstracts®, PsycINFO®, Current Contents®, Web of Science®, CrossRef, and Google Scholar.
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