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KMID : 0388219960030020126
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Journal of the Korean Rheumatism Association
1996 Volume.3 No. 2 p.126 ~ p.133
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Clinical Evaluation of Neuropsychiatric Lupus Erythematosus
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Lee Chan-Hee
Song Chang-Ho
Lee Soo-Kon
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Abstract
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Objectives : To evaluate the clinical characteristics, diagnostic methods, serologic tests and treatment modalities of neuropsychiatric lupus (NPSLE).
Mothods : Sytemic lupus erythematosus (SLE) patients manifesting NPSLE at Severence 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 manifestation 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-10years).
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<0.001).
6) The sensitivity of diagnostic test 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%(0/1).
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 (anticonvulsant, 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 if difficult due to lack of specific test. Focal manifestation of NPSKE was associated with antiphospholipid antibody.
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KEYWORD
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Neuropsychiatric lupus, Clinical feature, Antiphospholipid anti-body
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