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目的：探讨小儿神经精神性狼疮（ＮＰＬＥ）的临床表现、诊断和发病机理。方法：对１２年来诊断为系统性红斑狼疮２６例中有神经精神症状的８例ＮＰＬＥ进行系统的临床观察，分析其ＣＳＦ、ＥＥＧ和影像学检查所见。结果：临床表现有精神症状、器质性脑功能障碍、颅神性损害、外周神经病变和惊厥发作；ＣＳＦ变化主要是蛋白增高，ＥＥＧ均有不同程度的脑电波异常，颅脑影像学检查４例有３例异常。尸检１例示脑及多个脏器血管病变。结论：ＮＰＬＥ的临床表现多样化，需结合ＣＳＦ、ＥＥＧ和影像学检查在排除其他神经精神症状的病因后作出诊断。其发病机理是血管病变、免疫损伤和抗神经原的自身抗体等多种因素的作用。
Objective: To investigate the clinical manifestations, diagnosis and pathogenesis of neuropathic lupus (NPLE) in children. Methods: Eight patients with neuropsychiatric symptoms diagnosed as systemic lupus erythematosus in the past 12 years were systematically observed and analyzed for CSF, EEG and imaging findings. Results: The clinical manifestations of mental symptoms, organic brain dysfunction, cranial degeneration, peripheral neuropathy and seizures; CSF changes are mainly protein, EEG have varying degrees of brain wave abnormalities, brain imaging 4 There are 3 cases of abnormalities. An autopsy 1 case of multiple brain and brain organ disease. CONCLUSION: The clinical manifestations of NPLE are diverse and need to be combined with CSF, EEG and imaging studies to diagnose the etiology of other neuropsychiatric symptoms. The pathogenesis of vascular disease, immune damage and anti-neuronal autoantibodies and other factors.