原发性干燥综合征中枢神经系统损害4例分析

The clinical analysis of central neurological dysfunctions in primary Sjogren' s syndrome:4 cases report

目的探讨原发性干燥综合征中枢神经系统损害的临床特点,以提高其早期诊断、治疗效果。方法回顾性分析4例原发性干燥综合征中枢神经系统损害患者的临床特征、诊疗情况,并结合国内外相关文献进行分析。结果 4例患者均为女性,年龄30～52岁。3例患者中枢神经系统损害表现先于干燥综合征诊断,1例患者先诊断干燥综合征后出现中枢神经系统损害。1例患者出现脊髓损害表现,2例患者为脑实质损害表现,1例具有脊髓、脑、视神经损害表现。4例患者MR均表现为点状或小片状长T1长T2信号影。治疗上1例患者单用糖皮质激素,其余3例患者均合用免疫抑制剂,治疗后病情均好转,随访无再新发中枢神经系统损害表现。结论对不能用其他原因解释的中枢神经系统损害,尤其是中老年女性,应警惕原发性干燥综合征的可能。重视干燥综合征症状的询问,加深对干燥综合征中枢神经系统损害临床表现的认识,进行相关检查,以提高早期诊断、治疗效果,减少患者的致残率。

Objective To explore the clinical features of central neurological dysfunctions in primary Sjogren＇s syndrome, and to improve its early diagnosis and treatment.Methods Retrospective review the clinical features,diagnosis and treatment in four patients of central neurological dysfunctions in primary Sjogren＇s syndrome,and analyze the relative literatures. Results The four patients were female,aged from 30 years old to 52 years old.Three patients of them presented the manifestation of central nervous system damage ahead of the diagnosis of Sjogren＇ s syndrome.One patient with spinal cord injury, two patients with brain parenchymal damage,and one patient with spinal cord injury,brain parenchyma and optic nerve damage.All of the patients showed punctate or small pieces of long T1,T2 signal intensity in the MR examination.In terms of treatment,one patient was treated with glucocorticoid;the others were treated with＇ glucocorticoid and immunosuppressant. The conditions of the four patients were improved after treatment.No new performances of central nervous system damage in the clinical follow-up.Conclusion The possibility of primary Sjogren＇ s syndrome in patients with central nervous system damage which cannot be explained by other reasons should be considered,especially of old women.More attentions should be payed to ask the symptoms of primary Sjogren＇s syndrome and have better Understanding of the manifestations of the central neurological dysfunctions in primary Sjogren＇s syndrome and then carry out necessary examinations.Eventually,make an early diagnosis,treated in time and reduce the morbidity of patients.