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原发性干燥综合征合并中枢神经系统脱髓鞘病变临床研究

Clinical Study of Demyelinating Lesions of the Central Nervous System with Primary Sjogren’s Syndrome
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摘要 目的探究原发性干燥综合征(PSS)中枢神经系统脱髓鞘病变的特点及相关检查,为临床诊治提供理论参考。方法选取2014年1月—2019年12月该院风湿免疫科和神经内科收治的PSS患者120例,对其临床资料进行回顾性分析,研究合并中枢神经系统脱髓鞘病变患者的临床表现,分析其血清免疫学、脑脊液及影像学特点。结果全部120例PSS患者中,合并中枢神经系统脱髓鞘病变24例,其中脑部病变13例,脊髓病变11例,主要表现为头晕、视力和肌力下降、感觉和排尿障碍。肝肾功能受损16例,白细胞轻度升高3例,轻度贫血10例,血沉升高12例。抗ANA阳性19例,抗SSA阳性10例,抗SSB阳性11例,ds DNA抗体、抗Sm抗体、抗心磷脂抗体均阴性,低补体C311例。脑脊液蛋白升高且葡萄糖浓度升高5例,脑脊液氯化物升高13例,血清中均检测到寡克隆带。颅内多发性脱髓鞘病灶16例(66.67%)累及双侧侧脑室周围和脊髓中央,4例患者纵向脊髓节段超过4个。患者接受激素、丙球冲击和环磷酰胺治疗后,病情均显著改善,无病死病例。结论通过对PSS中枢神经系统脱髓鞘病变患者临床表现、血清免疫学、脑脊液及影像学检查的综合分析,有助于提升对病变的认识及早期诊断,并进一步指导治疗,改善预后。 Objective To explore the characteristics and related examination of central nervous system demyelinating lesions of primary Sjogren’s syndrome(PSS),and to provide theoretical reference for clinical diagnosis and treatment.Methods A total of 120 patients with PSS who were admitted to the Department of Rheumatology and Neurology in the hospital from January 2014 to December 2019 were selected to retrospectively analyze their clinical data to study the clinical manifestations of patients with central nervous system demyelination,its serum immunology,cerebrospinal fluid and imaging features.Results Among all 120 patients with PSS,24 patients had central nervous system demyelinating lesions,including 13 brain lesions and 11 spinal cord lesions.The main manifestations were dizziness,decreased vision and muscle strength,and sensory and urinary disorders.There were 16 patients with impaired liver and kidney function,3 patients with mildly elevated white blood cells,10 patients with mild anemia,and 12 patients with elevated ESR.19 cases were positive for anti-ANA,10 cases were positive for anti-SSA,11 cases were positive for anti-SSB,ds DNA antibody,anti-Sm antibody,and anticardiolipin antibody were all negative,and C311 cases were low complement.CSF protein increased and glucose concentration increased in 5 cases,CSF chloride increased in 13 cases,and oligoclonal bands were detected in serum.16 patients(66.67%)with multiple intracranial multiple demyelinating lesions involved bilateral periventricular and central spinal cord.4 patients had more than 4 longitudinal spinal cord segments.After receiving the hormone,globulin shock and cyclophosphamide treatment,the patient’s condition improved significantly,and there were no deaths.Conclusion The comprehensive analysis of the clinical manifestations,serum immunology,cerebrospinal fluid and imaging examination of patients with central nervous system demyelinating lesions of PSS can help to improve the understanding and early diagnosis of the lesions,and further guide the
作者 王春华 WANG Chun-hua(Department of Rheumatology,Heze Municipal Hospital,Heze,Shandong Province,274000 China)
出处 《系统医学》 2020年第13期13-15,共3页 Systems Medicine
关键词 原发性干燥综合征 中枢神经系统 脱髓鞘病变 特点 诊断检查 Primary Sjogren’s syndrome Central nervous system Demyelinating lesions Features Diagnostic examination
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