摘要
目的探讨以中枢神经系统损害为首发表现的原发性干燥综合征(primary Sjogren's Syndrome,pSS)患者的临床特点。方法纳入15例以中枢神经系统损害为首发表现的pSS患者,回顾性分析其临床表现、实验室指标及影像学结果,采用描述性分析方法对结果进行统计分析。结果15例患者中男女比例为1∶4,首发年龄(45.53±16.19)岁,平均发病32.37 m后确诊。首发症状包括头痛(6.67%)、头晕(20%)、呕吐(20%)、言语不清(6.67%)、面瘫(6.67%)、偏侧肢体无力(26.67%)、双下肢麻木无力(13.33%)、四肢麻木无力(46.67%)、胸背部疼痛(26.67%)、视物模糊(6.67%)。病变累及大脑半球(33.33%)、脑干(53.33%)、小脑(6.67%)、脊髓(53.33%)、视神经(6.67%),表现为脑梗死(33.33%)、蛛网膜下腔出血(6.67%)、横贯性脊髓炎(6.67%)、延髓病变(6.67%)、视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorders,NMOSD)(53.33%),其中NMOSD合并脑梗死1例(6.67%)。pSS确诊前复发3例(20%)。确诊时抗SSA抗体、抗核抗体阳性率100%,抗SSB抗体阳性率73.33%,抗R0-52阳性率80%,抗ds-DNA抗体阳性率6.67%,RF阳性率20%,IgG、IgA升高率20%,补体C3降低率20%。脑脊液压力(80~240)mmH_(2)O,白细胞(4~130)×106/L,蛋白(0.27~1.46)g/L,IgG升高5例,IgM升高2例,IgA升高4例。结论pSS可以中枢神经系统损害为首发表现,未及时筛查、监测自身免疫抗体指标是pSS延误诊断的重要原因。对不明原因的中枢神经系统病变,尤其是表现为复发性脱髓鞘事件或脑梗死时,应积极行抗SSA、SSB等自身免疫抗体检查,并注意询问有无眼干、口干等症状,对早期诊断有重要意义。
Objective To investigate the clinical characteristics of primary Sjogren's Syndrome(pSS)patients with central nervous system(CNS)damage as the initial manifestation.Methods Fifteen pSS patients with CNS damage as the initial manifestation were recruited.Clinical manifestations,laboratory results and imaging findings were retrospectively analyzed.Descriptive statistics was used for statistical analysis.Results Among the 15 patients,the ratio of male to female was 1∶4,and the first onset age was(45.53±16.19)years old.The diagnosis was confirmed after an average of 32.37 months.The initial symptoms included headache(6.67%),dizziness(20%),vomiting(20%),slurred speech(6.67%),facial paralysis(6.67%),hemilateral limb weakness(26.67%),numbness and weakness of both lower limbs(13.33%)or the limbs(46.67%),chest and back pain(26.67%),blurred vision(6.67%).The lesions involved the cerebral hemisphere(33.33%),brain stem(53.33%),cerebellum(6.67%),spinal cord(53.33%),and optic nerve(6.67%),presenting as cerebral infarction(33.33%),SAH(6.67%),transverse myelitis(6.67%),bulbar lesion(6.67%),neuromyelitis optica spectrum disorder(NMOSD)(53.33%)and NMOSD complicated with cerebral infarction in 1 case(6.67%).CNS symptoms recurred in 3 cases(20%)before pSS was diagnosed.At the time of diagnosis,the positive rate of autoimmune antibodies was anti-SSA antibody 100%,anti-nuclear antibody 100%,anti-SSB antibody 73.33%,anti-R0-52 antibody 80%,anti-ds-DNA antibody 6.67%,respectively.The positive rate of RF was 20%.The increase rate of IgG and IgA were 20%,and the decrease rate of complement C3 was 20%.The cerebrospinal fluid study showed:CSF pressure(80-240)mmH_(2)O,white blood cells cout(4-130)×106/L,protein(0.27-1.46)g/L;CSF IgG increased in 5 cases,and IgM increased in 2 cases,and IgA increased in 4 cases.Conclusion CNS damage can be the initial manifestation of pSS.The lack of timely screening and monitoring of autoimmune antibody is an important reason for the delayed diagnosis of pSS.For unexplained CNS lesions,especially those presenting as recurrent demyelinating events or cerebral infarction,autoimmune antibodies such as anti-SSA and SSB should be examed actively,while the symptoms such as dry eyes and dry mouth should be questioned.It is important for early diagnosis.
作者
项薇
康健捷
熊铁根
邓文婷
班俊杰
邓兵梅
XIANG Wei;KANG Jianjie;XIONG Tiegen(Department of Neurology,General Hospital of the Southern Theater Command,PLA,Guangzhou 510010,China)
出处
《中风与神经疾病杂志》
CAS
2022年第11期1008-1013,共6页
Journal of Apoplexy and Nervous Diseases
基金
广州市科技计划项目(202102080514)。
关键词
干燥综合征
中枢神经系统
自身免疫抗体
Sjogren's syndrome
Central nervous system
Autoimmune antibodies
