Brod Staley A, Lindsey J William, Nelson Flavia
Department of Neurology, University of Texas, USA.
Mult Scler J Exp Transl Clin. 2019 Jun 18;5(2):2055217319855755. doi: 10.1177/2055217319855755. eCollection 2019 Apr-Jun.
Large demyelinating lesions with possible mass effect (tumefactive multiple sclerosis or tumefactive demyelination) can be mistaken for tumour-like space-occupying lesions suggesting a malignant outcome.
We reviewed our own experience of multiple sclerosis subjects ( = 28) with tumefactive demyelination to determine the relationship between clinical outcomes and lesion evolution, clinical outcomes and their relationship to different therapies. Patients with central nervous system demyelinating disease were identified from our database over the last 10 years.
No patient increased in extended disability status scale (EDSS). Overall, lesion regression was associated with improved EDSS. Lesion regression was also associated with therapy versus no therapy. No specific therapy or corticosteroid infusions improved EDSS over the long term. The absence of enhancement on follow up on magnetic resonance imaging portended lesion regression.
Tumefactive demyelination may predict a more benign overall course and is susceptible to traditional immunomodulatory treatments.
具有可能的占位效应的大型脱髓鞘病变（瘤样多发性硬化或瘤样脱髓鞘）可能被误诊为提示恶性结果的肿瘤样占位性病变。
我们回顾了自己对28例患有瘤样脱髓鞘的多发性硬化患者的经验，以确定临床结果与病变演变之间的关系，临床结果及其与不同治疗方法的关系。在过去10年中，从我们的数据库中识别出患有中枢神经系统脱髓鞘疾病的患者。
没有患者的扩展残疾状态量表（EDSS）增加。总体而言，病变消退与EDSS改善相关。病变消退也与接受治疗与否相关。从长期来看，没有特定的治疗方法或皮质类固醇输注能改善EDSS。磁共振成像随访时无强化预示病变消退。
瘤样脱髓鞘可能预示总体病程更良性，并且易受传统免疫调节治疗的影响。
