Bornavirus Encephalitis Shows a Characteristic Magnetic Resonance Phenotype in Humans

Bornavirus Encephalitis Shows a Characteristic Magnetic Resonance Phenotype in Humans
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DOI:
10.1002/ana.25873
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发表时间:
2020-08-29
影响因子:
11.2
通讯作者:
Wiesinger, Isabel
Wiesinger, Isabel
中科院分区:
医学1区
文献类型:
--
作者:
Finck, Tom;Liesche-Starnecker, Friederike;Wiesinger, Isabel

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目的自经典博尔纳病病毒(BoDV-1)被证实可引起人类感染以来,已确诊的由BoDV-1引起的人类致死性脑炎病例数量不断增加。然而,对这种实体的认识很低,并且尚未确定特定的成像模式。因此,我们提供了第一个全面的描述人类BoDV-1脑炎的形态,与成像异常的组织病理学验证。方法在一项机构审查委员会批准的多中心研究中,我们对19例确诊为BoDV-1脑炎的55例磁共振成像(MRI)检查进行了回顾性分析。系统分析了50个脑区(T1 w、T2 w、T2*w、T1 w + Gd和DWI),以辨别特定的炎症模式。1例患者25个部位的组织学分析与MRI异常相关。结果基线成像,平均11 +/- 10天后症状发作,除了后续扫描的16名患者,发现特征性的T2高信号,以头部的尾状核,小脑,皮质扩散到边缘系统的偏好,而枕叶和小脑半球不受影响。该梯度通过组织学证实。9例患者(47.4%)基底节出现T1高信号,对应于组织学上的脂质吞噬细胞积聚,是晚期坏死的典型表现。解释BoDV-1脑炎在疾病的早期和晚期都显示出不同的炎症模式。MRI表现与散发性Creutzfeldt-Jakob病相似,在临床表现不典型的情况下,应将其作为鉴别诊断。ANN NEUROL 2020
Objective The number of diagnosed fatal encephalitis cases in humans caused by the classical Borna disease virus (BoDV-1) has been increasing, ever since it was proved that BoDV-1 can cause human infections. However, awareness of this entity is low, and a specific imaging pattern has not yet been identified. We therefore provide the first comprehensive description of the morphology of human BoDV-1 encephalitis, with histopathological verification of imaging abnormalities. Methods In an institutional review board-approved multicenter study, we carried out a retrospective analysis of 55 magnetic resonance imaging (MRI) examinations of 19 patients with confirmed BoDV-1 encephalitis. Fifty brain regions were analyzed systematically (T1w, T2w, T2*w, T1w + Gd, and DWI), in order to discern a specific pattern of inflammation. Histopathological analysis of 25 locations in one patient served as correlation for MRI abnormalities. Results Baseline imaging, acquired at a mean of 11 +/- 10 days after symptom onset, in addition to follow-up scans of 16 patients, revealed characteristic T2 hyperintensities with a predilection for the head of the caudate nucleus, insula, and cortical spread to the limbic system, whereas the occipital lobes and cerebellar hemispheres were unaffected. This gradient was confirmed by histology. Nine patients (47.4%) developed T1 hyperintensities of the basal ganglia, corresponding to accumulated lipid phagocytes on histology and typical for late-stage necrosis. Interpretation BoDV-1 encephalitis shows a distinct pattern of inflammation in both the early and late stages of the disease. Its appearance can mimic sporadic Creutzfeldt-Jakob disease on MRI and should be considered a differential diagnosis in the case of atypical clinical presentation. ANN NEUROL 2020