Repeated MRI-negative demyelinating attacks linked to MOG-IgG antibodies and silent lesions – Case series and literature review

IF 1.6 4区 医学 Q3 CLINICAL NEUROLOGY
Tal Friedman-Korn , Ariel Rechtman , Omri Zveik, Adi Vaknin-Dembinsky
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Abstract

Background

Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD) is an inflammatory demyelinating disorder of the central nervous system. While spinal MRI in MOGAD typically reveals longitudinally extensive transverse myelitis lesions, often involving the conus, rare cases of clinical myelitis without radiological abnormalities have been reported. This study aimed to characterize such MRI-negative presentations in MOGAD and compare them with previously published cases.

Methods

We retrospectively reviewed 138 patients with MOGAD and identified 37 who had experienced at least one episode of clinical myelitis. Among these, five female patients presented with clinical relapses—four involving the spinal cord and one involving the brain—without corresponding lesions on brain or spinal MRI at the time of the attack. We analyzed their clinical, radiological, and serological profiles in detail. Additionally, we conducted a comprehensive literature review to contextualize our findings, focusing on the demographic and clinical characteristics of similar MRI-negative MOGAD cases, and explored potential explanations for this phenomenon.

Results

All five patients were MOG-IgG positive (one borderline), and most showed a favorable clinical response to corticosteroids or plasmapheresis. One patient later developed a radiologically apparent but clinically silent spinal lesion. Comparison with 14 cases reported in the literature revealed similar features, including high disease severity at onset, responsiveness to immunotherapy, and frequent diagnostic delays. Notably, our cohort exhibited exclusive female representation, in contrast to the mixed demographic profiles reported elsewhere.

Conclusion

Clinical attacks in MOGAD may occur despite normal spinal and brain MRI findings, emphasizing the limitations of current imaging modalities. A normal MRI does not exclude active or evolving disease. Early MOG-IgG testing, empirical treatment when clinically indicated, and scheduled follow-up imaging are essential to prevent misdiagnosis and mitigate morbidity. Increased awareness of MRI-negative MOGAD is crucial for timely recognition and effective management.
反复mri阴性脱髓鞘发作与MOG-IgG抗体和无症状病变相关-病例系列和文献综述
髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)是一种中枢神经系统炎症性脱髓鞘疾病。虽然MOGAD的脊柱MRI通常显示纵向广泛的横贯脊髓炎病变,通常累及椎圆锥,但罕见的临床脊髓炎病例没有影像学异常的报道。本研究旨在描述MOGAD患者的mri阴性表现,并将其与先前发表的病例进行比较。方法回顾性分析138例MOGAD患者,其中37例至少经历过一次临床脊髓炎发作。其中,5名女性患者表现为临床复发(4例累及脊髓,1例累及大脑),发作时脑或脊髓MRI无相应病变。我们详细分析了他们的临床、放射学和血清学资料。此外,我们进行了全面的文献综述,将我们的发现置于背景下,重点关注类似mri阴性MOGAD病例的人口学和临床特征,并探讨了这一现象的潜在解释。结果5例患者均为MOG-IgG阳性(1例为临界),大多数患者对皮质类固醇或血浆置换有良好的临床反应。一名患者后来发展为放射学上明显但临床无症状的脊柱病变。与文献中报道的14例病例比较,发现了类似的特征,包括发病时疾病严重程度高,对免疫治疗反应性强,诊断经常延迟。值得注意的是,与其他地方报道的混合人口统计资料相比,我们的队列显示出完全的女性代表。结论尽管脊柱和脑部MRI显示正常,但MOGAD的临床发作仍可能发生,这强调了当前成像方式的局限性。正常的核磁共振检查不能排除活动性或进展性疾病。早期MOG-IgG检测、临床指征时的经验性治疗以及定期随访影像学检查对于预防误诊和减轻发病率至关重要。提高对mri阴性MOGAD的认识对于及时识别和有效管理至关重要。
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来源期刊
Clinical Neurology and Neurosurgery
Clinical Neurology and Neurosurgery 医学-临床神经学
CiteScore
3.70
自引率
5.30%
发文量
358
审稿时长
46 days
期刊介绍: Clinical Neurology and Neurosurgery is devoted to publishing papers and reports on the clinical aspects of neurology and neurosurgery. It is an international forum for papers of high scientific standard that are of interest to Neurologists and Neurosurgeons world-wide.
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