신경정신루프스에 대한 임상적 연구

Abstract

Objectives: To evaluate the clinical characteristics, diagnostic methods, serologic tests and treatment modalities of neuropsychiatric lupus (NPSLE). Methods: Systemic lupus erythematosus(SLE) patients manifesting NPSLE at Severance Hospital, Yonsei University College of Medicine were retrospectively studied in the period of Jan 1994 to March 1996. Results: The results were as follows; 1) Neuropsychiatric manifestations were observed in 24 patients of the total 144 SLE patients (16.6%). 2) Of the 24 NPSLE patients, there was 1 male, 23 female and the mean age was 32 years (range 14-70). 3) Eight patients presented neuropsychiatric symptoms as an initial manifestation and the rest presented during the follow up period (mean: 25 months, range: 2 months-10 years). 4) Preceding cause was identified in seven patients and was classified as secondary NPSLE: drug in 1 case, infection in 2 cases, reactive depression in 2 cases, and cerebral hemorrhage due to thrombocytopenia in 2 case. 5) NPSLE was classified into diffuse manifestation and focal manifestation. The diffuse manifestation was found in 22 cases and the focal manifestation was found in 6 cases. Patients with focal manifestation showed higher rate of anticardiolipin antibody or lupus anticoagulant test than patients with diffuse manifestation with of 100% (3/3) in the former and 30% (6/20) in the latter(p<0.01). 6) The sensitivity of diagnostic tests were as follows: cerebrospinal fluid study 7.1% (1/11), electroencephalogram 62.5% (5/8), brain computed tomogram 52.9% (9/17) , magnetic resonance imaging 66.7% (6/9), cerebral angiogram 100% (1/1), and myelogram 0% (01). 7) The treatment modalities were as follows; high dose steroid (1mg/kg/day) or steroid pulse therapy (intravenous methylpredinisolone 1g/day for 3 days) alone in 13 cases, high dose steroid or steroid plus other drugs (anticonvulants, psychiatric medication, warfarin) in 5 cases, steroid pulse plus plasmapheresis in 3 cases, steroid pulse and plasmapheresis plus other drugs (intravenous cyclophosphamide, anticonvulsant) in 2 patients, low dose steroid (alone or with analgesics) in 2 patient, psychiatric treatment in 2 patients and no treatment in 1 patient. 26 patients responded to treatment. 8) The mortality rate was 12.5% (3/24) and the cause of death was pulmonary hemorrhage, septic shock, and status epilepticus. Conclusions: NPSLE is a frequent and morbid manifestation of SLE but diagnosis is difficult due to lack of specific test. Focal manifestation of NPSLE was associated with anti phospholipid antibody.