脑脊液检测正常狼疮性脑病患者临床特点

倪军喜; 李毅; 马勇; 杨振; 孙晓月; 朱华栋; 于学忠

doi:10.3969/j.issn.1674-9081.2017.05.013

脑脊液检测正常狼疮性脑病患者临床特点

The Clinical Features of Neuropsychiatric Lupus Erythematosus with Normal Cerebrospinal Fluid

摘要

摘要:
目的分析脑脊液检测正常狼疮性脑病(neuropsychiatic lupus erythematosus, NPLE)患者的临床特点, 以提高NPLE的诊断。
方法 2013年1月至2016年12月北京协和医院确诊为NPLE的住院患者95例, 收集患者入院第一次留取的脑脊液标本检测结果, 其中脑脊液正常者18例(研究组), 脑脊液异常(压力、细胞数、蛋白任何一项指标异常即为脑脊液异常)者77例(对照组)。对两组性别、年龄、入院症状、起病方式、累及系统、炎症学指标、磁共振成像(magnetic resonance imaging, MRI)结果、治疗情况及住院时间等进行统计学分析, 并进一步对18例脑脊液正常NPLE患者的年龄分布、系统性红斑狼疮(systemic lypus erythematosus, SLE)病程、治疗方案及MRI结果进行描述性分析。
结果 18例脑脊液正常患者中以精神症状起病者10例, 77例脑脊液异常患者中精神症状起病者10例, 两组起病方式差异有统计学意义(55.6%比13.0%, P < 0.05);脑脊液正常患者中NPLE复发患者12例, 脑脊液异常患者中NPLE复发患者20例, 两组比较有统计学意义(66.7%比26.0%, P < 0.05)。18例脑脊液正常NPLE患者中育龄期女性16例(88.9%), 诊断SLE后一年内发病9例(50.0%), 因激素量不足起病17例(94.4%); 9例MRI结果亦正常患者48 h后脑脊液复查均出现异常。
结论以精神症状起病的SLE患者, 既往有NPLE病史, 入院首次脑脊液即使正常, 也应及时行MRI检查或复查脑脊液, 防止漏诊NPLE。

Abstract:
Objective The aim of our study was to analyze clinical features of neuropsychiatric lupus erythematosus(NPLE)with normal cerebrospinal fluid, in order to improve the diagnosis of NPLE.
Methods From January 2013 to December 2016, a total of 95 patients diagnosed with NPLE at Peking Union Medical College Hospital were included in our study. Results of the first time exam of cerebrospinal fluid after hospitalization were collected. Cerebrospinal fluid was normal in 18 cases(study group) and abnormal(including any abnormalities in pressure, cell number or protein) in 77 cases(control group). Gender, age, symptoms, mode of onset, involved system, inflammatory markers, magnetic resonance imaging(MRI) findings, dsDNA, treatment, and length of hospitalization were analyzed statistically. Age distribution, systemic lupus erythematosus(SLE) course, treatment, and results of MRI of the 18 patients with NPLE and normal cerebrospinal fluid were further descriptively analyzed.
Results Ten of the 18 patients with normal cerebrospinal fluid and 10 of the 77 cases with abnormal cerebrospinal fluid started with mental symptoms. There were significant differences between the two groups(55.6% vs. 13.0%, P < 0.05). Twelve of the study group and 20 of the control group had relapse of NPLE(66.7% vs. 26.0%, P < 0.05). There was no significant difference between the two groups in gender, age, involved system, inflammatory indicators, MRI results, double stranded DNA, treatment, and length of hospitalization(P>0.05). Sixteen of 18 cases of the study group were women at reproductive age(88.9%); 9 occurred in the first year of SLE(50.0%); 17 were initiated from inadequate medication(94.4%); 9 patients with normal MRI findings presented abnormal CSF examination 48 hours later.
Conclusion SLE patients with onset of mental symptoms and medical history of NPLE, even with normal cerebrospinal fluid exam, should perform MRI and repeated cerebrospinal fluid exams to avoid the misdiagnosis of NPLE.

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