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瘤样脱髓鞘病变诊断中的陷阱:一例病例报告。

Pitfalls in the diagnosis of a tumefactive demyelinating lesion: A case report.

作者信息

Gavra Maria, Boviatsis Efstathios, Stavrinou Lampis C, Sakas Damianos

机构信息

Department of CT and MRI, Children's Hospital, "Agia Sophia'', Thivon and Papadiamantopoulou Street, Athens, Greece.

出版信息

J Med Case Rep. 2011 Jun 7;5:217. doi: 10.1186/1752-1947-5-217.

DOI:10.1186/1752-1947-5-217
PMID:21649896
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3138437/
Abstract

INTRODUCTION

In rare instances, demyelinating disorders manifest as tumefactive lesions that simulate brain tumors. We report a patient with a space-occupying lesion in the parietal lobe, which presented a serious diagnostic dilemma, between a rare tumefactive demyelinating disease, such as Balo concentric sclerosis and a glioma. This case report highlights important diagnostic clues in the differential diagnosis of Balo concentric sclerosis.

CASE PRESENTATION

A 20-year-old Caucasian woman with acute onset of left-sided weakness and numbness was admitted to hospital with neurologic signs of left-sided hemiparesis and hypoesthesia. Brain magnetic resonance imaging showed a mass lesion of abnormal signal intensity with concentric enhancing rings in the right parietal lobe, without perifocal edema. The characteristic concentric pattern detected on the magnetic resonance images was highly suggestive of Balo disease, and corticosteroids were administered. Evoked potentials, cerebrospinal fluid analysis, and magnetic spectroscopy findings were not specific, and glioma was also included in the differential diagnosis. A stereotactic biopsy was not diagnostic.After one month the patient showed moderate clinical improvement, and during 12 months follow-up, no further relapses occurred. In the follow-up magnetic resonance imaging, the concentric pattern had completely disappeared, and only a low-signal, gliotic lesion remained.

CONCLUSION

We hope this case presentation will advance our understanding of clinical and radiologic appearance of Balo concentric sclerosis, which is a rare demyelinating disease. Although this is a specific entity, it has a broader clinical impact across medicine, because it must be differentiated from other space-occupying lesions in the central nervous system.

摘要

引言

在罕见情况下,脱髓鞘疾病表现为类似脑肿瘤的肿胀性病变。我们报告了一名顶叶有占位性病变的患者,该病变在一种罕见的肿胀性脱髓鞘疾病(如巴洛同心性硬化)和胶质瘤之间造成了严重的诊断困境。本病例报告突出了巴洛同心性硬化鉴别诊断中的重要诊断线索。

病例介绍

一名20岁的白种女性,急性起病出现左侧肢体无力和麻木,因左侧偏瘫和感觉减退的神经学体征入院。脑部磁共振成像显示右侧顶叶有一个信号强度异常的肿块病变,伴有同心性强化环,周围无水肿。磁共振图像上检测到的特征性同心模式高度提示巴洛病,遂给予皮质类固醇治疗。诱发电位、脑脊液分析和磁共振波谱结果均不具有特异性,胶质瘤也被列入鉴别诊断。立体定向活检未能明确诊断。一个月后患者临床症状有中度改善,在12个月的随访中未再复发。在随访的磁共振成像中,同心模式已完全消失,仅留下一个低信号的胶质增生性病变。

结论

我们希望本病例报告能增进我们对巴洛同心性硬化这种罕见脱髓鞘疾病的临床和影像学表现的理解。尽管这是一种特定的疾病实体,但它在医学领域具有更广泛的临床影响,因为它必须与中枢神经系统的其他占位性病变相鉴别。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/885d/3138437/6f1e0366ee6c/1752-1947-5-217-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/885d/3138437/6f1e0366ee6c/1752-1947-5-217-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/885d/3138437/6f1e0366ee6c/1752-1947-5-217-1.jpg

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本文引用的文献

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Balò's concentric sclerosis presenting with benign clinical course and multiple sclerosis-like lesions on magnetic resonance images.巴洛同心性硬化症,临床表现良性,磁共振成像显示为多发性硬化样病变。
Neurol India. 2009 Jan-Feb;57(1):66-8. doi: 10.4103/0028-3886.48815.
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Characterization of tumefactive demyelinating lesions using MR imaging and in-vivo proton MR spectroscopy.使用磁共振成像和体内质子磁共振波谱对瘤样脱髓鞘病变进行特征分析。
Mult Scler. 2009 Feb;15(2):193-203. doi: 10.1177/1352458508097922.
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Tumefactive demyelinating lesions: nine cases and a review of the literature.
肿胀性脱髓鞘病变:9例病例及文献综述
Neurosurg Rev. 2009 Apr;32(2):171-9; discussion 179. doi: 10.1007/s10143-009-0185-5. Epub 2009 Jan 27.
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Successful pregnancy of a patient with Balo's concentric sclerosis.一名患有巴洛同心性硬化症的患者成功怀孕。
Mult Scler. 2005 Jun;11(3):346-8. doi: 10.1191/1352458505ms1158oa.
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Tumefactive demyelinating lesions: conventional and advanced magnetic resonance imaging.肿块样脱髓鞘病变:传统及先进的磁共振成像
Mult Scler. 2005 Apr;11(2):135-9. doi: 10.1191/1352458505ms1145oa.
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CT-guided stereotactic biopsies of brain stem lesions: personal experience and literature review.CT引导下脑干病变的立体定向活检:个人经验及文献综述
Neurol Sci. 2003 Oct;24(3):97-102. doi: 10.1007/s10072-003-0093-3.
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Use of serial proton magnetic resonance spectroscopy to differentiate low grade glioma from tumefactive plaque in a patient with multiple sclerosis.在一名多发性硬化症患者中,使用连续质子磁共振波谱法鉴别低度胶质瘤与瘤样斑块。
Br J Radiol. 2003 Sep;76(909):662-5. doi: 10.1259/bjr/85069069.
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Baló's concentric sclerosis: clinical and radiologic features of five cases.巴洛同心性硬化:5例临床及影像学特征
AJNR Am J Neuroradiol. 2001 Aug;22(7):1362-7.
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Acute focal demyelinating disease simulating brain tumors: histopathologic guidelines for an accurate diagnosis.酷似脑肿瘤的急性局灶性脱髓鞘疾病:准确诊断的组织病理学指南
Neuropathology. 2001 Mar;21(1):25-31. doi: 10.1046/j.1440-1789.2001.00365.x.