The Clinical Features of Neuropsychiatric Lupus Erythematosus with Normal Cerebrospinal Fluid

  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.