Mauri-Fábrega Lara, Díaz-Sánchez María, Casado-Chocán José Luis, Uclés-Sánchez Antonio José
Department of Neurology, Virgen del Rocío University Hospital, Sevilla, Spain.
Eur Neurol. 2014;72(1-2):72-8. doi: 10.1159/000358385. Epub 2014 Jun 4.
The pseudotumoral form of multiple sclerosis (MS) is a rare condition with few descriptions in the literature. It supposes a diagnostic challenge especially when appearing at the onset of disease.
We retrospectively describe a case series of pseudotumoral MS patients that attended our hospital from 2004, analyzing demographic, clinical and radiological variables. We classified the lesions according to the recently proposed morphologic classification (infiltrative, megacystic, Baló or ring-like) and according to the contrast enhancement pattern (nodular, complete ring, incomplete ring and diffuse).
Fourteen patients (11 female, 3 male), with a mean age of 35 years, were identified. All of them suffered from a relapsing-remitting form of MS. Eleven patients (78.57%) had symptomatic pseudotumoral lesions (PL), being the form of clinical presentation in the majority of those patients that were symptomatic (81.81%). Several patients presented atypical clinical manifestations such as cognitive impairment (21.42%) and epileptic seizures (14.28%). Full recovery was found in 53.84% of all symptomatic episodes. After a mean follow-up of 43 months, recurrent PL episodes were seldom observed (21.42%), the annualized relapse rate was 0.95 and the mean final Expanded Disability Status Scale score was 1.5. The majority of PLs were supratentorial, coexisted with typical demyelinating plaques and showed the ring-like morphology and the ring pattern of contrast enhancement. Three patients had more than one PL on the same scan, all of the lesions with similar morphology.
Our findings contribute to a better characterization of pseudotumoral forms of MS. However, larger studies are required to define this atypical entity more exactly.
多发性硬化症（MS）的假瘤样形式是一种罕见病症，文献中描述较少。它带来了诊断挑战，尤其是在疾病发作时出现这种情况。
我们回顾性描述了自2004年起在我院就诊的假瘤样MS患者的病例系列，分析了人口统计学、临床和放射学变量。我们根据最近提出的形态学分类（浸润性、大囊型、巴洛型或环状）以及对比增强模式（结节状、完整环、不完整环和弥漫性）对病变进行分类。
共确定了14例患者（11例女性，3例男性），平均年龄35岁。他们均患有复发缓解型MS。11例患者（78.57%）有症状性假瘤样病变（PL），在大多数有症状的患者中，这是临床表现形式（81.81%）。部分患者出现非典型临床表现，如认知障碍（21.42%）和癫痫发作（14.28%）。所有有症状发作中，53.84%实现了完全康复。平均随访43个月后，很少观察到PL复发（21.42%），年化复发率为0.95，最终扩展残疾状态量表平均评分为1.5。大多数PL位于幕上，与典型脱髓鞘斑块共存，呈现环状形态和环状对比增强模式。三名患者在同一扫描中有不止一处PL，所有病变形态相似。
我们的研究结果有助于更好地描述MS的假瘤样形式。然而，需要更大规模的研究来更准确地定义这种非典型实体。
