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15 例肿块样脱髓鞘病变患者:临床-放射学特征、处理及文献复习。

Tumefactive demyelinating lesions of 15 patients: Clinico-radiological features, management and review of the literature.

机构信息

Demyelinating Disorders Unit, Neurology Department, Hospital Universitario de La Princesa, Instituto de Investigación Sanitaria La Princesa, Madrid, Spain.

Demyelinating Disorders Unit, Neurology Department, Hospital Universitario de La Princesa, Instituto de Investigación Sanitaria La Princesa, Madrid, Spain.

出版信息

J Neurol Sci. 2017 Oct 15;381:32-38. doi: 10.1016/j.jns.2017.08.005. Epub 2017 Aug 12.

Abstract

BACKGROUND AND OBJECTIVE

Tumefactive demyelinating lesions (TDLs) are large inflammatory lesions that can mimic tumors or other space-occupying lesions. Differential diagnosis and management of these lesions remain challenging for neurologists. We aim to review the clinico-radiological features of patients with TDLs, as well as their management.

METHODS

We performed a retrospective review of cases of TDLs treated in our center from January 2010 to February 2017. We reviewed the literature.

RESULTS

Out of 711 patients, we found 15 with TDLs (12 women and 3 men), with a mean age of TDL onset of 36years. Out of the 15 patients, 9 had TDLs as a first demyelinating event and 44% (4/15) of these converted to MS by McDonald 2010 criteria in a mean time of 8months (SD 3.10). Clinical presentation was polysymptomatic and the most common radiological findings included solitary lesions (66.66%), located primarily in the frontal (40%) or parietal (33%) lobes, showing mostly an infiltrative morphological pattern (50%) and an open-ring enhancement (43%). Multiple Sclerosis was the most common diagnosis (67%, 10/15). Acute treatment included steroids, plasmapheresis, rituximab and cyclophosphamide.

CONCLUSIONS

Prevalence of tumefactive demyelination in MS was higher than expected in MS patients, according to literature. Time to conversion to MS was significantly shorter than other published series. Clinical presentation was polysymptomatic and the most common radiological findings were isolated frontal lesions with an open-ring enhancement.

摘要

背景与目的

肿块样脱髓鞘病变(TDL)是一种可模拟肿瘤或其他占位性病变的大型炎症性病变。神经科医生对这些病变的鉴别诊断和治疗仍然具有挑战性。我们旨在回顾 TDL 患者的临床-影像学特征及其治疗方法。

方法

我们对 2010 年 1 月至 2017 年 2 月在我院治疗的 TDL 病例进行了回顾性分析。我们查阅了相关文献。

结果

在 711 例患者中,我们发现 15 例 TDL(12 名女性和 3 名男性),TDL 发病的平均年龄为 36 岁。15 例患者中,9 例 TDL 为首次脱髓鞘事件,其中 44%(4/15)按 2010 年 McDonald 标准转化为多发性硬化症,平均时间为 8 个月(SD 3.10)。临床表现为多症状性,最常见的影像学发现包括单发病变(66.66%),主要位于额(40%)或顶(33%)叶,主要表现为浸润性形态模式(50%)和开放环强化(43%)。多发性硬化症是最常见的诊断(67%,10/15)。急性治疗包括类固醇、血浆置换、利妥昔单抗和环磷酰胺。

结论

根据文献报道,TDL 在多发性硬化症患者中的发病率高于预期。转化为多发性硬化症的时间明显短于其他已发表的系列。临床表现为多症状性,最常见的影像学发现为孤立性额部病变,伴有开放环强化。

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