The incidence and prognosis of central nervous system disease in systemic lupus erythematosus.

In a review of our experience with systemic lupus erythematosus (SLE) since 1975, we found 48 of 266 patients with major central nervous system (CNS) manifestations for which a non-SLE explanation could not be identified. Eleven patients developed more than one type of CNS event. The commonest symptom was seizure (18 patients), followed by brainstem dysfunction (12 patients), psychosis (11 patients), organic brain syndrome (11 patients) and stroke (7 patients). In 19% of cases, CNS manifestations were accompanied by a flare of multisystem SLE disease activity. Anticonvulsants were able to be discontinued safely in the majority of patients with seizures. Most CNS events were self-limited, reversible and not associated with poor outcome unless accompanied by multisystem disease activity. Therapy with corticosteroids did not appear to offer substantial benefit.