朗讀老師：司東雷  石家莊市第三人民醫(yī)院

翻譯老師：王長耿   福建省晉安醫(yī)院

審校老師：姜春雷   青島市第九人民醫(yī)院

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

病史：25 歲男性，有漸進性言語困難。

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.

以下是平掃和增強的顱腦MR圖像：軸位彌散加權(quán)成像 (DWI)、表觀彌散系數(shù) (ADC) 和液體衰減反轉(zhuǎn)恢復(fù)序列 (FLAIR)。

Additional MR images

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

其他MR圖像

腦部MR增強圖像（軸位 T1 增強）如下所示。

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

影像表現(xiàn)與臨床診斷：

影像學：雙側(cè)大腦半球亞急性梗死伴強化。CTA 顯示雙側(cè)大腦中動脈和右側(cè)頸內(nèi)動脈巖部不規(guī)則串珠樣表現(xiàn)，與血管炎有關(guān)。

實驗室：經(jīng)腰椎穿刺腦脊液 (CSF) 顯示白細胞和蛋白升高。梅毒血清學檢測呈陽性。

診斷：梅毒性中樞神經(jīng)系統(tǒng) (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.

關(guān)鍵點

中樞神經(jīng)系統(tǒng) (CNS) 血管炎

以非動脈粥樣硬化性炎和血管壁壞死為特征。動脈和靜脈都可能受累及。

CT或MRI可以檢測到繼發(fā)性征象，例如以低密度區(qū)和T2高信號區(qū)為特征的缺血或梗塞，可能彌散受限。

尋找基底節(jié)、皮層和皮層下白質(zhì)受累情況。

數(shù)字減影血管造影 (DSA) 是最敏感的成像方法，可顯示局灶性光滑或略微不規(guī)則狹窄與擴張段交替表現(xiàn)。動脈粥樣硬化性疾病表現(xiàn)不典型。

常見病因有：細菌、結(jié)核、真菌、梅毒、膠原血管疾病和濫用藥物。

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.

典型的影像學表現(xiàn)

CT：不敏感，但可能會看到影響不同血管分布的相對低密度區(qū)。

MRI：T2加權(quán)成像上出現(xiàn)多灶性高信號，可能彌散受限，并且在增強后可見斑片狀強化。

CTA/MRA：可能會看到管腔不規(guī)則的狹窄或擴張區(qū)域；然而，DSA比較敏感，是金標準檢查。

臨床診斷

如果懷疑中樞神經(jīng)系統(tǒng)血管炎，可與其他臨床發(fā)現(xiàn)相關(guān)聯(lián)，包括腰椎穿刺和毒理學。如果需要確切的病因?qū)W，可考慮對受累血管區(qū)域進行活檢。