NEUROLOGIC MANIFESTATIONS OF PEDIATRIC SYSTEMIC LUPUS-ERYTHEMATOSUS

Central nervous system involvement is a common but rarely reviewed fea ture of pediatric systemic lupus erythematosus (SLE), We retrospective ly reviewed the charts of 91 patients with pediatric SLE and using a s tandardized data abstraction form documented 40 patients with central nervous system (CNS-SLE) involvement, The mean age of onset of SLE was 13.3 years, In 19 patients the CNS manifestation was a presenting sym ptom, in 12 patients CNS involvement was present within the first year of diagnosis, and in 9 patients it took up to 7 years for CNS disease to become evident, Nineteen children (48%) manifested neuropsychiatri c SLE, which included depression, concentration or memory problems, an d frank psychosis, Seizures were present in 8 patients (20%), 6 had ce rebral ischemic events (15%), 1 had chorea (3%), 2 had papilledema (5% ), and 2 patients had a peripheral neuropathy (5%). Nine patients (22% ) had severe headache consistent with lupus headache, Seven children h ad more than one CNS manifestation, In the investigation of CNS-SLE, c omputed tomography and/or magnetic resonance imaging scans were helpfu l in patients with focal ischemic lesions and venous sinus thrombosis, Electroencephalography was abnormal only in 33% of patients with seiz ure disorders and rarely helpful in patients with diffuse neuropsychia tric symptoms, Single-photon emission computed tomography scans were a bnormal in most patients with neuropsychiatric SLE, especially in thos e with frank psychosis, The lupus anticoagulant was present in the pat ient with chorea and was frequently present in patients with cerebral vascular events, Long-term outcome was good: only 1 child died of cere bral hemorrhagic infarction and 3 others had significant persistent CN S deficits, The majority of patients (90%) had excellent recovery from CNS-SLE.