摘要
目的:分析卵巢畸胎瘤相关抗N-甲基-D-天门冬氨酸受体(NMDAR)脑炎的MRI表现,提高对该病的认识及诊断水平。方法:收集2007年1月-2021年7月本院共152例确诊为自身免疫性脑炎的病例,其中脑脊液和/或血清抗NMDAR抗体阳性且合并卵巢畸胎瘤共10例,所有病例均有完整影像及临床资料。10例均于发病期内完成颅脑MR检查及腹部超声和/或CT检查。回顾全部病例临床病史,分析其影像特点。结果:10例患者均为年轻女性,年龄15~38岁。4例发病前3天~1个月有发热头痛,余6例无前驱症状。所有病例均表现为突发神经功能障碍和/或精神异常,并呈进行性加重,均出现自主神经功能障碍,其中9例出现精神症状或癫痫发作,5例出现意识障碍或运动障碍/不自主运动。颅脑MRI有9例显示异常,1例无明确异常。病灶均位于大脑半球,7例病灶累及双侧皮层(灰质)和/或皮层下白质,好发位置依次为扣带回6例(双侧)、额叶6例(双侧)、颞叶5例(双侧3例、单侧2例)、岛叶3例(双侧2例、单侧1例)、基底节1例(双侧)、顶叶1例(单侧)、枕叶1例(单侧),其中2例累及深部脑白质;另2例仅累及一侧深部脑白质。病灶于T_(2)FLAIR上为稍高信号(9例),DWI上为稍高信号但扩散不受限(4例);所有病例T_(1)WI、T_(2)WI序列及增强扫描均未见异常信号。10例共诊断11个卵巢畸胎瘤(1例为双侧),10个病灶长径小于4 cm,仅1个病灶长7.8 cm。病理示9例10个成熟型畸胎瘤,未成熟型畸胎瘤仅1例;镜下见脑组织或神经胶质成分者3例,余均无特殊发现。结论:卵巢畸胎瘤相关抗NMDAR脑炎常见于年轻女性,临床表现为进行性加重的神经精神症状;颅脑MRI表现轻微,以T_(2)FLAIR及DWI显示病灶较敏感;结合盆腔检查,将有助于本病的诊断。
Objective:To investigate the MR findings of anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis patients associated with ovarian teratoma to improve the level of understanding and diagnosis.Methods:A total of 152 cases of autoimmune encephalitis with complete imaging and clinical data diagnosed in our hospital from January 2007 to July 2021 were collected.Among of them,10 cases coexisted with positive anti NMDAR antibody in cerebrospinal fluid and/or serum and ovarian teratoma were confirmed and underwent brain MR examination and abdominal ultrasound and/or CT examination during the onset period.The clinical history of all cases was reviewed and their imaging characteristics were analyzed.Results:All 10 patients were young women and the range of age was 15~38 years.Four cases had fever and headache from 3 days to 1 month before onset,and the other 6 cases had no prodromal symptoms.All cases showed sudden and progressive neurological dysfunction and/or mental abnormalities.Among these 10 cases suffered with autonomic nerve dysfunction,9 cases had mental symptoms or seizures and 5 cases had disturbance of consciousness or movement/involuntary movement.Brain MRI showed abnormalities only in 9 cases and the foci were located in the cerebral hemisphere.Bilateral cortex(gray matter)and/or subcortical white matter were involved in 7 cases.Among them,the prone locations were cingulate gyrus in 6 cases(bilateral),frontal lobe in 6 cases(bilateral),temporal lobe in 5 cases(bilateral 3 cases,unilateral 2 cases),insular lobe in 3 cases(bilateral 2 cases,unilateral 1 case),basal ganglia in 1 case(bilateral),parietal lobe in 1 case(unilateral)and occipital lobe in 1 case(unilateral).Two of these 7 cases had deep white matter abnormalities.The other 2 cases only involved one side of deep white matter.The lesions showed slightly hyperintensity on T_(2) FLAIR sequence(9 cases),slightly hyperintensity on DWI sequence without diffusion limitation on ADC map(4 cases);No abnormal signal was found on T_(1)WI,T_(2)WI sequence and enhanced scan in all cases.A total of 11 ovarian teratomas were diagnosed in 10 cases(1 case was bilateral).The long diameter of 10 lesions were less than 4cm,except one with 7.8cm.The pathological results were 10 mature teratomas in 9 cases and only 1 immature teratoma in another cases;There were 3 cases with brain tissue or glial components under microscope,and the rest had no special findings.Conclusion:Anti NMDAR encephalitis patients associated with ovarian teratoma tend to occur in young women and manifest as progressive neuropsychiatric symptoms.Brain MRI abnormalities were not obvious and T_(2) FLAIR and DWI sequences were more sensitive to show foci.Pelvic examination is helpful to diagnose the disease.
作者
罗益贤
胡笑笑
唐广磊
王珂
马捷
关键
LUO Yi-xian;HU Xiao-xiao;TANG Guang-lei(Shenzhen People's Hospital,Guangdong 518109,China)
出处
《放射学实践》
CSCD
北大核心
2022年第7期818-823,共6页
Radiologic Practice
