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一名年轻男性身上的多个大型瘤样脱髓鞘斑块:诊断难题与治疗挑战

Multiple Large Tumefactive MS Plaques in a Young Man: A Diagnostic Enigma and Therapeutic Challenge.

作者信息

Kalanie Hossein, Harandi Ali Amini, Bakhshandehpour Reza, Heidari Daryoosh

机构信息

Mehr Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

出版信息

Case Rep Radiol. 2012;2012:363705. doi: 10.1155/2012/363705. Epub 2012 Jul 12.

Abstract

Tumefactive demyelinating lesion is defined as large solitary demyelinating lesion with imaging characteristics mimicking neoplasm. These atypical features include size more than 2 cm, mass effect, edema, and/or ring enhancement. Distinguishing tumefactive lesions from other etiologies of intracranial space occupying lesions is essential to avoid inadvertent surgical or toxic chemotherapeutic intervention. Symptoms are generally atypical for multiple sclerosis (MS) and usually related to the pressure of a focal mass lesion without a history of MS. The clinical presentation and MRI appearance of these lesions often lead to biopsy. Here, we present a young man with fulminating neurological symptoms and multiple large tumefactive lesions on either hemisphere. Since patient and parents were not agreed on brain biopsy, a course of steroid therapy was commenced which ended to considerable improvement and confirmed the diagnosis of tumefactive MS. Thirteen months later, he experienced another relapse when his treatment was continued by weekly intramuscular injection of interferon b1a (Avonex). Two further MRIs showed shrinkage of tumefactive plaques and resolution of edema in the periphery of lesions.

摘要

瘤样脱髓鞘病变被定义为具有类似肿瘤影像学特征的大型孤立性脱髓鞘病变。这些非典型特征包括大小超过2厘米、占位效应、水肿和/或环形强化。将瘤样病变与颅内占位性病变的其他病因区分开来,对于避免意外的手术或毒性化疗干预至关重要。症状通常不符合多发性硬化症(MS)的典型表现,且通常与局灶性肿块病变的压迫有关,患者无MS病史。这些病变的临床表现和MRI表现常导致进行活检。在此,我们报告一名年轻男性,他出现暴发性神经症状,双侧大脑半球有多个大型瘤样病变。由于患者及其父母不同意进行脑活检,于是开始了一个疗程的类固醇治疗,结果病情有了显著改善,并确诊为瘤样MS。13个月后,当通过每周肌肉注射干扰素β-1a(阿沃尼克斯)继续治疗时,他再次复发。另外两次MRI显示瘤样斑块缩小,病变周边水肿消退。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6596/3403125/6afbf49d3ecf/CRIM.RADIOLOGY2012-363705.001.jpg

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