1122 CENTRAL NERVOUS SYSTEM (CNS) LUPUS ERYTHEMATOSUS (LE) IN CHILDREN

In 67 unselected patients under the age of 20 years with LE, evidence of CNS disease was present in 18% (3M,9F). Abnormal neurologic findings included: chorea-7, upper motor neuron signs-3, cranial nerve paresis-3, cerebellar dysfunction-2, focal seizures and coma-1, stroke associated with hypertension-2. Chorea is the major abnormality in this group; in 3 patients it presented as long as three years prior to the diagnosis of LE. Significant renal disease was present in 8 patients. Laboratory tests were not helpful in diagnosis. Lumbar puncture and brain scan were normal unless stroke was present. EEG was always abnormal but correlated with neurologic findings and improvement. Serologic parameters were only mildly abnormal during recurrences of CNS disease. Standard doses of prednisone and Imuran used to treat LE exacerbations corrected all major CNS abnormalities except neurologic deficits secondary to strokes. Three patients had recurrences of CNS disease which responded to retreatment with high-dose therapy. This study provides the first comprehensive view of the high incidence of CNS involvement in a non-selective series of childhood LE patients and emphasizes the need to recognize subtle abnormalities and initiate adequate therapy.