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Aimee 上海某医院 狼疮性脑炎 History: Seventy-year-old woman with history of seizures and a chronic disease. 病史：70岁女性，伴有癫痫和慢性病变 Click these T2W MR images to enlarge them. Here now are some images from a follow-up scan 4 days later. Rows are: T2W, enhanced T1W, gradient echo, and FLAIR 4天后再次行磁共振检查：每一排依次为：T2W、T1W增强、梯度回波序列和黑水序列 Findings: Diffuse, predominantly white matter T2/FLAIR hyperintense signal spares only the anterior temporal lobes and has increased/worsened from prior study. Interval development of focal lesions with hypointense T1 signal, low T2 signal, are heterogeneously hypointense on gradient echo, do not show restricted diffusion, and rim enhance. Ventricles, sulci, and cisterns remain normally configured and are of normal size for patient's age. No midline shifts. Normal flow-voids in the major intracranial arteries. 影像学表现：除了颞叶前部，余大脑白质均呈弥漫、显著的T2/FLAIR高信号，较上次检查有所进展。新发的局灶性病变呈T1W、T2W低信号，回波序列呈不均匀高信号，未见弥散受限或环状强化。脑室、脑沟和脑池结构、大小未见明显异常。中线结构居中，大脑大血管可见流空效应。 Differential diagnosis: 鉴别诊断 · Lupus cerebritis /ˌserɪ'braɪtɪs/ 狼疮性脑炎 · Atypical infection不典型感染 · Severe progressive PRES (posterior reversible encephalopathy syndrome) with high-dose steroids严重进展性可逆性后部白质脑病伴高剂量激素治疗后表现 Actual diagnosis: Lupus cerebritis (presumed). 诊断：狼疮性脑炎（推测） Commentary: Initial study has findings typical of lupus encephalopathy. The focal lesions in the second study are almost certainly hemorrhage. Given the rapid progression of these abnormalities, patient's history of lupus, and the development of multiple hemorrhagic foci, lupus cerebritis is most likely, with less likely differential diagnoses including severe progressive PRES with high dose steroids or atypical infection. 评论：初步影像学检查提示典型的狼疮性脑病。第二次检查中的局灶性病变基本可以判定为出血。鉴于上述异常表现进展速度之快、患者的狼疮病史以及多发出血灶，基本可以给出狼疮性脑炎的诊断。鉴别诊断包括严重进展性可逆性后部白质脑病伴高剂量激素治疗后表现或不典型的感染，可能性均比较低。 Key points - Lupus cerebritis: 要点-狼疮性脑炎 · One acute subcategory of neuropsychiatric systemic lupus erythematosus (NPSLE).是急性神经精神系统性红斑狼疮脑病的亚分类。 · Clinical临床 · Neurologic disorder which can present with any neurologic sign or symptom. · 神经系统疾病可表现为任何神经症状或体征 · Focal局灶性 · Seizure癫痫 · Diffuse弥漫性 · Psychosis精神病 · Laboratory实验室 · May be associated with high anti-ribosomal P autoantibody. · 可能与高浓度的抗核糖体P蛋白抗体有关 · Role of imaging影像学证据 · Assessment of acute focal (stroke-like) neurologic deficits. · 急性局灶神经系统病变评估（类似卒中改变） · CNS vasculitis.中枢系统血管炎 · Reported in up to 7% of SLE patients. · 报道可发生于高达7%的狼疮患者当中 · High T2 signal in white matter. · 脑白质T2W高信号 · Sometimes in vascular distribution. · 有时位于血管分布区域 · Involves cortical and subcortical areas. · 累及皮质和皮质下脑区 · Particularly occipital region. · 尤其是枕叶 · Relative sparing of periventricular white matter relative to MS. · 相对于多发性硬化，不累及脑室周围白质 · Atrophy萎缩 · Commonly found, secondary to · 常见，一般为继发性改变 · Encephalopathy脑病 · Steroid use激素治疗 · Lesions may evolve in 7–10-day course · 病灶在7-10天进展 · Also, possible同样也可能出现 · Hemorrhage出血 · Subarachnoid蛛网膜下腔出血 · Intraparenchymal脑实质内出血 · Infarct梗死 · Aneurysms also reported某些报道中可见动脉瘤 |
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