CAJ | 학술논문

目的：总结原发性中枢神经系统血管炎临床、磁共振成像（magnetic resonance imaging，MRI）和病理学特点。方法收集2012年3月～2014年12月首都医科大学附属北京天坛医院神经病学中心收治的原发性中枢神经系统血管炎患者资料，采用描述性方法对其临床表现、MRI及病理学特点进行分析。结果共收集9例患者，其中男性5例（55.56%），女性4例（44.44%），年龄范围10～47岁，中位年龄30岁。痫性发作4例（44.44%），行为认知异常3例（33.33%），局灶性感觉运动异常5例（55.56%），头晕2例（22.22%），钝性头痛2例（22.22%），面部疼痛1例（11.11%），视物模糊1例（11.11%），行走不稳1例（11.11%）。腰穿异常4例（44.44%）。MRI表现为双侧病灶6例（66.67%），单侧病灶3例（33.33%），其中受累部位分别为额叶9例（100%），顶叶5例（55.56%），颞枕叶4例（44.44%），合并皮层下白质受累6例（66.67%），合并脑膜/脊膜受累3例（33.33%），合并基底节受累1例（11.11%），合并脊髓受累1例（11.11%）；病变边界不清8例（88.89%），边界清1例（11.11%）；皮层萎缩伴脑室扩大3例（33.33%）；病灶及脑脊膜强化6例（66.67%）；7例患者行磁共振T2*或磁敏感加权序列（susceptibility weighted imaging，SWI），其中病灶表现低信号4例（57.14%）。2例行脑病理学检查，表现为脑实质水肿，血管周围炎性细胞浸润，血管壁坏死和胶质增生。结论原发性中枢神经系统血管炎临床表现及影像学多样，病灶合并脑膜和（或）脊膜强化，以及磁共振T2*或SWI序列低信号是其重要影像学特征，脑活检仍是目前重要的确诊手段。
목적：총결원발성중추신경계통혈관염림상、자공진성상（magnetic resonance imaging，MRI）화병이학특점。방법수집2012년3월～2014년12월수도의과대학부속북경천단의원신경병학중심수치적원발성중추신경계통혈관염환자자료，채용묘술성방법대기림상표현、MRI급병이학특점진행분석。결과공수집9례환자，기중남성5례（55.56%），녀성4례（44.44%），년령범위10～47세，중위년령30세。간성발작4례（44.44%），행위인지이상3례（33.33%），국조성감각운동이상5례（55.56%），두훈2례（22.22%），둔성두통2례（22.22%），면부동통1례（11.11%），시물모호1례（11.11%），행주불은1례（11.11%）。요천이상4례（44.44%）。MRI표현위쌍측병조6례（66.67%），단측병조3례（33.33%），기중수루부위분별위액협9례（100%），정협5례（55.56%），섭침협4례（44.44%），합병피층하백질수루6례（66.67%），합병뇌막/척막수루3례（33.33%），합병기저절수루1례（11.11%），합병척수수루1례（11.11%）；병변변계불청8례（88.89%），변계청1례（11.11%）；피층위축반뇌실확대3례（33.33%）；병조급뇌척막강화6례（66.67%）；7례환자행자공진T2*혹자민감가권서렬（susceptibility weighted imaging，SWI），기중병조표현저신호4례（57.14%）。2례행뇌병이학검사，표현위뇌실질수종，혈관주위염성세포침윤，혈관벽배사화효질증생。결론원발성중추신경계통혈관염림상표현급영상학다양，병조합병뇌막화（혹）척막강화，이급자공진T2*혹SWI서렬저신호시기중요영상학특정，뇌활검잉시목전중요적학진수단。
Objective To summarize the characteristics of primary central nervous system vasculitis from clinical, imaging and pathological aspects by retrospective study. Methods From March 2012 to December 2014, the data of the inpatients of primary central nervous system vasculitis in Beijing Tiantan Hospital were collected, and their clinical manifestation, imaging and pathological characteristics were analyzed by using a descriptive method. Results There were 9 patients, 5 males (55.56%) and 4 females (44.44%) separatively. The age ranges from 10 years old to 47 year old, with the median age of 30 years old. There were 4 cases (44.44%) of epileptic seizure, 3 cases (33.33%) of abnormal behavior and cognition, 5 cases (55.56%) with sensorimotor abnormalities, 2 cases (22.22%) with dizziness, 2 cases (22.22%) with headache, 1 case (11.11%) with facial pain, 1 case (11.11%) with blurred vision and 1 case (11.11%) with unstable walking. There were 4 patients (44.44%) with cerebral spinal lfuid (CSF) abnormalities. There were 6 cases (66.67%) with bilateral lesions, 3 cases (33.33%) with unilateral lesions, 9 cases (100%) involved the frontal lobe, 5 cases (55.56%) involved the parietal lobe,4 cases (44.44%) involved the temporal and occipital lobe, 6 cases (66.67%) combined with subcortical white matter involvement, 3 cases (33.33%) combined with meningeal involvement, 1 case (11.11%) complicated with basal ganglia involvement and 1 case (11.11%) complicated with spinal cord involvement. The lesions were 8 cases (88.89%) with unclear border and 1 case (11.11%) with clear border. There were 3 cases (33.33%) with cortical atrophy. There were 6 cases (66.67%) with the enhancement of the lesions and meningeal. 57.14% (4/7 patients) showed that the lesions were low signal in T2*/susceptibility weighted imaging (SWI) sequence of magnetic resonance imaging (MRI). Conclusion The clinical manifestation and imaging in primary central nervous system vasculitis are diverse. It is important characteristics of MRI that the meningeals with lesion are enhanced and the lesions in T2*/SWI sequence are low signal. The brain biopsy is still the important diagnostic method.