Relapsing tumefactive demyelination lesions: A unique, distinct inflammatory brain pathology.

IF 4.8 2区 医学 Q1 CLINICAL NEUROLOGY
Paola Perini, Marta Gaggiola, Francesca Rinaldi, Paolo Gallo, Marco Puthenparampil
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引用次数: 0

Abstract

We report the case of a patient suffering from biopsy-proven relapsing tumefactive demyelinating lesions (TDLs) of the central nervous system who had five relapses in 16 years. No signs/symptoms suggestive of alternative pathologies emerged during the follow-up. A limited benefit was observed with intravenous (IV) high-dose steroids, while both plasma exchange and IV immunoglobulin G (IgG) administration were ineffective. A long-lasting (9 years) but transient clinical stabilization was obtained with cyclophosphamide. Our case supports the view that recurrent TDL is a relapsing brain inflammation not belonging to multiple sclerosis (MS) or myelin oligodendrocyte glycoprotein (MOG)-/AQP4-associated disorders. TDL concept and clinical features should be revised.

复发性肿瘤活动性脱髓鞘病变:一种独特的、与众不同的脑部炎症病理。
我们报告了一例经活检证实的复发性中枢神经系统肿瘤活动性脱髓鞘病变(TDLs)患者的病例,该患者在16年内复发了5次。在随访期间,没有出现其他病变的体征/症状。静脉注射大剂量类固醇的疗效有限,而血浆置换和静脉注射免疫球蛋白 G (IgG) 均无效。使用环磷酰胺后,患者的临床症状得到了长期(9 年)但短暂的稳定。我们的病例支持这样一种观点,即复发性 TDL 是一种复发性脑部炎症,不属于多发性硬化症(MS)或髓鞘少突胶质细胞糖蛋白(MOG)-/AQP4 相关疾病。TDL的概念和临床特征应予以修正。
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来源期刊
Multiple Sclerosis Journal
Multiple Sclerosis Journal 医学-临床神经学
CiteScore
10.90
自引率
6.90%
发文量
186
审稿时长
3-8 weeks
期刊介绍: Multiple Sclerosis Journal is a peer-reviewed international journal that focuses on all aspects of multiple sclerosis, neuromyelitis optica and other related autoimmune diseases of the central nervous system. The journal for your research in the following areas: * __Biologic basis:__ pathology, myelin biology, pathophysiology of the blood/brain barrier, axo-glial pathobiology, remyelination, virology and microbiome, immunology, proteomics * __Epidemology and genetics:__ genetics epigenetics, epidemiology * __Clinical and Neuroimaging:__ clinical neurology, biomarkers, neuroimaging and clinical outcome measures * __Therapeutics and rehabilitation:__ therapeutics, rehabilitation, psychology, neuroplasticity, neuroprotection, and systematic management Print ISSN: 1352-4585
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