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为何这位“阿姨级医学影像专家”会面临诊断难题?:基于知识的巴洛同心性硬化研究方法——来自3例报告及文献中其他68例患者汇总数据

Why Is This Auntminnie a Diagnostic Conundrum?: A Knowledge-Based Approach to Balo's Concentric Sclerosis From Reports of 3 Cases and Pooled Data From 68 Other Patients in the Literature.

作者信息

Agarwal Mohit, Ulmer John L, Klein Andrew P, Mark Leighton P

机构信息

Section of Neuroradiology, Medical College of Wisconsin, Milwaukee, WI.

Section of Neuroradiology, Medical College of Wisconsin, Milwaukee, WI.

出版信息

Curr Probl Diagn Radiol. 2019 Jul-Aug;48(4):415-422. doi: 10.1067/j.cpradiol.2017.12.008. Epub 2018 Jan 6.

Abstract

INTRODUCTION

We came across 3 cases of Balo's concentric sclerosis (BCS). The first of these patients presented to an outside hospital and was transferred to our institution due to complications resulting from a biopsy. The other 2 patients, despite having a characteristic imaging appearance and despite insistence on our part on the diagnosis of BCS, underwent a surgical procedure, which could have been prevented. This led us to review the available literature on BCS.

MATERIAL AND METHODS

A total of 68 patients diagnosed with BCS between 1995 and 2015 were studied and the data collected for the clinical presentation and course, imaging, spinal fluid analysis, treatment, and clinical and imaging outcome.

CONCLUSIONS

A 25% surgery rate (biopsy or resection) was found in the study. We concluded that this relatively high surgery rate in this auntminnie nonsurgical disease is multifactorial; and includes factors like nonfamiliarity with the disease, anxiety on the part of patients and physicians, due to a sometimes rapidly deteriorating clinical picture; and resemblance of the disease with other entities such as tumor and infection. However, characteristic imaging appearance combined with acute or subacute presentation and dramatic improvement in clinical status after high-dose steroid chemotherapy; are highly suggestive of the disease, and can prevent unnecessary surgery.

摘要

引言

我们遇到了3例巴洛同心圆性硬化(BCS)病例。其中首例患者先前往外院就诊,后因活检引发的并发症转至我院。另外2例患者,尽管其影像学表现具有特征性,且我们一直坚持诊断为BCS,但他们还是接受了本可避免的手术。这促使我们回顾了关于BCS的现有文献。

材料与方法

对1995年至2015年间诊断为BCS的68例患者进行了研究,并收集了临床表现及病程、影像学、脑脊液分析、治疗以及临床和影像学结局等方面的数据。

结论

本研究中发现手术率为25%(活检或切除)。我们得出结论,在这种本质上无需手术的疾病中,如此相对较高的手术率是多因素导致的;包括对该疾病不熟悉、患者和医生的焦虑(因为临床症状有时会迅速恶化)以及该疾病与肿瘤和感染等其他病症的相似性。然而,特征性的影像学表现、急性或亚急性病程以及大剂量类固醇化疗后临床状况的显著改善,高度提示该病,可避免不必要的手术。

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