中枢神经系统损害为首发症状的系统性红斑狼疮临床分析

Central nervous system impairment as an initial manifestation of patients with systemic lupus erythematosus

目的探讨中枢神经系统(CNS)损害为首发症状的系统性红斑狼疮(SLE)的临床特征。方法回顾性分析10例首发CNS损害的SLE患者,分析其临床表现、影像学特征和治疗转归情况。结果 10例患者首发症状均为神经系统病变,临床表现包括头痛6例(60.0%)、癫痫5例(50.0%)、发热4例(40.0%)、脑梗死2例(20.0%)、静脉窦血栓2例(20.0%)、颅内多发病灶3例(30.0%)、脑干及脊髓病变2例(20.0%)、周围神经病变1例(10.0%)。神经精神性SLE的临床表现以头痛和癫痫发作出现概率最高。神经精神性SLE的脑部损害在MRI表现为弥漫型和局灶型病变,白质和灰质均可受累,分布无规律性。给予激素治疗后临床症状改善。结论 NPSLE临床表现多样,早期诊断、及时治疗是改善NPSLE预后的关键。

Objective To investigate the clinical features of central nervous system impairment as an initial manifestation in patients with systemic lupus erythematosus（SLE）. Methods Ten cases of SLE with central nervous system impairment as an initial manifestation were reviewed. The clinical manifestations, imaging, treatment methods and results were analyzed. Results Central nervous system impairments were initial manifestations of all ten patients. Clinical manifestations included headache （ 60.0 % ）, seizures （ 50.0% ）, fever （ 40.0 % ）, cerebral infarction （ 20.0 % ）, cerebral venous thrombosis （20.0 % ）, multiple focuses of brain （30.0 % ）, focus of brain stem and spinal （ 20.0 % ）, peripheral nerve disease（10.0%）. The highest incidence was headache and seizure. Magnetic resonance imagings of nervous psychopathic SLE were diffuse and focal lesions. Grey and white matters were involved and irregularly distributed. The therapy of hormone can ameliorate the clinical characteristics. Conclusion The clinical manifestations of nervous psychopathic SLE were various. Early diagnosis and prompt therapy are critical to ameliorate the prognosis of nervous psychopathic SLE.