PDF(Pubmed)
Abstract：
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare chronic central-nervous-system inflammatory disorder that became known only recently, and the pathogenesis of CLIPPERS remains poorly understood. This report presents clinical and radiological features of a rare case: a young female patient who rapidly died of suspected CLIPPERS. Helpful multiparametric MRI diagnostic criteria are proposed that can help discriminate CLIPPERS from non-CLIPPERS pathologies. We reviewed clinical history, symptoms, quantitative data from brain multiparametric MRI before and after treatment, and histopathological data. Perfusion-weighted imaging revealed a decrease in regional cerebral blood flow by 31% and in cerebral blood volume by 64%, with a moderate increase in transit time and in time to peak by up to 23% in affected pontine and cerebral white matter. As estimated by diffusion tensor imaging, there was elevated density of tracts (n/mm2) and a decrease of fraction anisotropy (×10-3 mm/s2) in the patient\'s pons as compared to a healthy control: density of tracts = 13.5 vs 12.4 and fraction anisotropy = 0.32 vs 0.45, respectively. Macromolecular proton fraction values proved to be reduced (15.8% and 14.5% in the control, respectively) in the patient\'s cerebral peduncles by 3% and in the pons by 4.1% and in a periventricular white matter lesion by 6.4% (11.3% in the normal-looking contralateral hemisphere). Based on our findings, we argue that quantitative MRI techniques may be a valuable source of biomarkers and reliable diagnostic criteria and can shed light on the pathogenesis and exact nosological position of this disorder.
摘要：
对类固醇有反应的慢性淋巴细胞性炎症伴脑桥血管周围增强（CLIPPERS）是一种罕见的慢性中枢神经系统炎症性疾病，最近才知道，和CLIPPERS的发病机制仍然知之甚少。本报告介绍了罕见病例的临床和放射学特征：一名年轻女性患者因疑似CLIPPERS而迅速死亡。提出了有用的多参数MRI诊断标准，可以帮助区分CLIPPERS与非CLIPPERS病理。我们回顾了临床病史，症状,治疗前后脑部多参数MRI的定量数据，和组织病理学数据.灌注加权成像显示局部脑血流量减少31%，脑血容量减少64%，在受影响的脑桥和脑白质中，运输时间适度增加，峰值达到23％。根据扩散张量成像估计，与健康对照相比，患者脑桥的束密度（n/mm2）升高，部分各向异性（×10-3mm/s2）降低：束密度=13.5vs.12.4，部分各向异性=0.32vs.0.45。大分子质子分数值被证明是降低的(15.8%和14.5%的对照,分别）在患者的脑花梗中占3％，在脑桥中占4.1％，在脑室周围白质病变中占6.4％（在正常的对侧半球中占11.3％）。根据我们的发现,我们认为,定量MRI技术可能是一个有价值的生物标志物来源和可靠的诊断标准,并且可以揭示该疾病的发病机制和确切的疾病学位置.
