Vakrakou Aigli G, Tzanetakos Dimitrios, Evangelopoulos Maria-Eleptheria, Argyrakos Theodore, Tzartos John S, Anagnostouli Maria, Andreadou Elissavet, Koutsis Georgios, Velonakis Georgios, Toulas Panagiotis, Gialafos Elias, Dimitrakopoulos Antonios, Psimenou Erasmia, Stefanis Leonidas, Kilidireas Constantinos
1st Department of Neurology, Medical School of Athens, National & Kapodistrian University, Aeginition Hospital, 72 Vasilissis Sofias Ave, Athens, 11528, Greece.
Demyelinating Diseases Unit, 1st Department of Neurology, School of Medicine, Eginition Hospital, National and Kapodistrian University of Athens, Athens, Greece.
Ther Adv Neurol Disord. 2021 May 18;14:17562864211006503. doi: 10.1177/17562864211006503. eCollection 2021.
Our goal was to expand the spectrum of clinico-radiologic characteristics and the possible therapeutic choices in patients with tumefactive demyelinating lesions (TDLs).
A retrospective analysis of 50 patients with at least one TDL was performed at an academic neurology center (2008-2020).
Our cohort comprised mostly women (33/50) with a mean age of 38 years at TDL onset. The mean follow-up time was 76 months. The mean Expanded Disability Status Scale score at TDL onset and at the latest neurological evaluation was 3.7 and 2.3, respectively. We subcategorized the patients into seven groups based mainly on the clinical/radiological findings and disease course. Group A included patients presenting with a Marburg-like TDL ( = 4). Groups B and C comprised patients presenting with monophasic ( = 7) and recurrent TDLs ( = 12), respectively. Multiple sclerosis (MS) patients who subsequently developed TDL ( = 16) during the disease course were categorized as Group D. Group E comprised patients who initially presented with TDL and subsequently developed a classical relapsing-remitting MS without further evidence of TDL ( = 5). Groups F ( = 2) and G ( = 4) involved MS patients who developed TDL during drug initiation (natalizumab, fingolimod) and cessation (interferon, fingolimod), respectively. Regarding long-term treatments applied after corticosteroid administration in the acute phase, B-cell-directed therapies were shown to be highly effective especially in cases with recurrent TDLs. Cyclophosphamide was spared for more aggressive disease indicated by a poor response to corticosteroids and plasma exchange failure.
Tumefactive central nervous system demyelination is an heterogenous disease; its stratification into distinct groups according to different phenotypes can establish more efficient treatment strategies, thus improving clinical outcomes in the future.
我们的目标是扩大瘤样脱髓鞘病变（TDL）患者的临床放射学特征谱以及可能的治疗选择范围。
在一家学术性神经科中心（2008 - 2020年）对50例至少有一个TDL的患者进行了回顾性分析。
我们的队列主要由女性组成（33/50），TDL发病时的平均年龄为38岁。平均随访时间为76个月。TDL发病时和最近一次神经学评估时的平均扩展残疾状态量表评分分别为3.7和2.3。我们主要根据临床/放射学表现和病程将患者分为七组。A组包括表现为马尔堡样TDL的患者（n = 4）。B组和C组分别包括表现为单相TDL的患者（n = 7）和复发性TDL的患者（n = 12）。在病程中随后出现TDL的多发性硬化（MS）患者（n = 16）被归类为D组。E组包括最初表现为TDL，随后发展为典型复发缓解型MS且无进一步TDL证据的患者（n = 5）。F组（n = 2）和G组（n = 4）分别涉及在开始使用药物（那他珠单抗、芬戈莫德）和停用药物（干扰素、芬戈莫德）期间出现TDL的MS患者。关于急性期给予皮质类固醇后应用的长期治疗，B细胞导向疗法显示出高度有效性，尤其是在复发性TDL的病例中。对于皮质类固醇反应不佳和血浆置换失败所表明的更侵袭性疾病，保留使用环磷酰胺。
瘤样中枢神经系统脱髓鞘是一种异质性疾病；根据不同表型将其分层为不同组可以制定更有效的治疗策略，从而在未来改善临床结局。
