Clinical Evaluation of Neuropsychiatric Lupus Erythematosus

Lee, Chan Hee; Lee, Ji Soo; Song, Chang Ho; Lee, Soo Kon

Abstract

OBJECTIVES
To evaluate the clinical characteristics, diagnostic METHOD:s, 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 i 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 anticoagu]ant 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.l% (1/ll), 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 (lmg/kg/day) or steroid pulse therapy (intravenous methylprediniso]one lg/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 antiphospholipid antibody.