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双语病例——梅毒性中枢神经系统 (CNS) 血管炎

 juntao859 2021-11-16

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朗读老师:司东雷  石家庄市第三人民医院

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翻译老师:王长耿   福建省晋安医院

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审校老师:姜春雷   青岛市第九人民医院

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History: A 25-year-old man with progressive speech difficulty.

病史:25 岁男性,有渐进性言语困难。

Below are brain MR images without and with contrast: axial diffusion-weighted imaging (DWI), apparent diffusion coefficient (ADC), and fluid-attenuated inversion recovery (FLAIR). Click to enlarge.

以下是平扫和增强的颅脑MR图像:轴位弥散加权成像 (DWI)、表观弥散系数 (ADC) 和液体衰减反转恢复序列 (FLAIR)。

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Additional MR images

Additional brain MR images (axial T1 postcontrast) are shown below.

其他MR图像

脑部MR增强图像(轴位 T1 增强)如下所示。

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Findings and diagnosis

Findings

Imaging: Bilateral cerebral hemispheric subacute infarcts with enhancement. CTA shows irregular beading of the bilateral middle cerebral arteries and right petrous internal carotid artery concerning for vasculitis.

Laboratory: Cerebrospinal fluid (CSF) obtained via lumbar puncture showed elevated white blood cells and protein. Serologic tests for syphillis were positive.

Diagnosis: Syphilitic central nervous system (CNS) vasculitis

影像表现与临床诊断:

影像学:双侧大脑半球亚急性梗死伴强化。CTA 显示双侧大脑中动脉和右侧颈内动脉岩部不规则串珠样表现,与血管炎有关。

实验室:经腰椎穿刺脑脊液 (CSF) 显示白细胞和蛋白升高。梅毒血清学检测呈阳性。

诊断:梅毒性中枢神经系统 (CNS) 血管炎

Key points

Central nervous system (CNS) vasculitis

Characterized by nonatheromatous inflammation and necrosis of blood vessel walls. Both arteries and veins maybe affected.

CT/MRI can detect secondary signs such as ischemia/infarction characterized by regions of hypoattenuation and T2 hyperintensities with possible restricted diffusion, respectively.

Look for involvement of the basal ganglia, cortex, and subcortical white matter.

Digital subtraction angiography (DSA) is the most sensitive imaging study and will show multifocal areas of smooth or slightly irregular-shaped stenoses alternating with dilated segments. Pattern will be atypical for atherosclerotic disease.

Common culprits: Bacterial, tuberculosis, mycotic, syphilitic, collagen vascular disease, and drug abuse.

关键点

中枢神经系统 (CNS) 血管炎

以非动脉粥样硬化性炎和血管壁坏死为特征。动脉和静脉都可能受累及。

CT或MRI可以检测到继发性征象,例如以低密度区和T2高信号区为特征的缺血或梗塞,可能弥散受限。

寻找基底节、皮层和皮层下白质受累情况。

数字减影血管造影 (DSA) 是最敏感的成像方法,可显示局灶性光滑或略微不规则狭窄与扩张段交替表现。动脉粥样硬化性疾病表现不典型。

常见病因有:细菌、结核、真菌、梅毒、胶原血管疾病和滥用药物。

Typical imaging findings

CT: Insensitive but may see relative areas of low-density areas affecting different vascular distributions.

MRI: Multifocal hyperintensities on T2-weighted imaging with possible restricted diffusion, and may see patchy areas of enhancement on postcontrast images.

CTA/MRA: May see regions of luminal irregularities and stenoses/dilatations; however, DSA is more sensitive and the gold standard imaging test.

Clinical

If CNS vasculitis is suspected, can correlate with other clinical findings, including lumbar puncture and toxicology. If exact etiology is needed, can consider performing biopsy of involved vessels/regions.

典型的影像学表现

CT:不敏感,但可能会看到影响不同血管分布的相对低密度区。

MRI:T2加权成像上出现多灶性高信号,可能弥散受限,并且在增强后可见斑片状强化。

CTA/MRA:可能会看到管腔不规则的狭窄或扩张区域;然而,DSA比较敏感,是金标准检查。

临床诊断

如果怀疑中枢神经系统血管炎,可与其他临床发现相关联,包括腰椎穿刺和毒理学。如果需要确切的病因学,可考虑对受累血管区域进行活检。

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