Aimee 上海某医院
40-year-old man with rapid-onset neurological manifestations
40岁男性，突发神经系统症状
History and MR images
History: A 40-year-old man presents to the accident and emergency department with a two-day history of headache, slurred speech, and right-sided weakness. He reported a long-standing history of mouth and genital ulcers.
病史：40岁男性，头痛2天、言语含糊、右侧肢体无力前来急诊。患者有长期口腔和生殖器溃疡。
MR images are shown below. Click to enlarge.
In order: Axial fluid-attenuated inversion-recovery (FLAIR) image, diffusion-weighted image (DWI), and MR venography (MRV) image.
水抑制，弥散加权，磁共振静脉成像。
Findings
Axial FLAIR (first image) shows bilateral symmetrical basal ganglia high signal. DWI (second image) shows no restriction of water diffusion. MRV (third image) shows patent dural venous sinuses and internal cerebral veins.
影像学表现：
磁共振水抑制图像轴位可见双侧基底节区对称高信号影。弥散加权图像未见明显受限信号。磁共振静脉成像可见硬膜静脉窦及颅内静脉显示良好
Differential diagnosis
· Venous infarcts
· Hypoxic ischemic encephalopathy
· Toxic encephalopathy
· Mitochondrial cytopathy
鉴别诊断
l 静脉梗死
l 缺氧缺血性脑病
l 中毒性脑病
l 线粒体细胞病变
Diagnosis: Neuro-Behçets disease
诊断：神经白塞病
Axial FLAIR image from follow-up MRI one year after treatment with prednisolone /'pred'nisələun/ is shown below. Click to enlarge.
随访图像：激素治疗1年后随访磁共振水抑制图像轴位如下
The classic triad of Behçet disease includes oral ulceration, genital ulceration, and uveitis.
白塞病的典型三联征包括口腔溃疡、生殖器溃疡和眼炎。
Parenchymal brain lesions in neuro-Behçet disease are mainly the result of perivascular infiltration with lymphocytes, neutrophils, and rarely eosinophils with or without signs of necrosis. This presents on MRI as areas of inflammation and vasogenic edema that gradually resolves and disappears completely in most cases. Timing of reimaging studies following treatment should be planned with the treating clinician. The main sites of involvement are in the basal ganglia and brainstem. However, other sites in the brain can be involved. Awareness of the key MR imaging features will help radiologists avoid misinterpretations that alter patient management.
神经系统白塞病的脑实质病灶主要源于血管周围淋巴细胞、中性粒细胞以及少量嗜酸性粒细胞浸润，伴或不伴有坏死。在磁共振图像表现为炎性病灶和血管源性水肿，大多病例病变可逐渐吸收消散。接受治疗的病人有必要进行治疗后影像学复查。病灶主要累及基底节区和脑干，同时也会累及颅内其他部位。放射科医生对于该病变磁共振典型征象的把握可以避免误诊影像患者临床治疗。
· Neuro-Behçet disease presents with rapid but not sudden-onset manifestation.
· 神经系统白塞病颅内病变进展迅速，并非突发。
· It is basically vasogenic (not cytotoxic) edema.
· 本质上属于血管源性水肿（并非细胞毒性）
· MR findings improve with treatment, so consider a follow-up scan.
· 磁共振所示病变随着治疗好转，因此可纳入随访影像学检查。
· Neuro-Behçet disease parenchymal brain lesions are mainly located in the basal ganglia and brainstem.
· 神经系统白塞病脑实质病变主要位于基底节区和脑干。
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