CHILDHOOD CENTRAL-NERVOUS-SYSTEM LUPUS - LONGITUDINAL ASSESSMENT USING SINGLE-PHOTON EMISSION COMPUTED-TOMOGRAPHY

Objective. Neuropsychiatric manifestations in children with systemic l upus erythematosus (SLE) occur in 30-60% of patients during the course of disease. Unlike other manifestations of childhood SLE, few laborat ory studies and imaging modalities aid in the diagnosis of central ner vous system (CNS) lupus. We and others have reported the usefulness of single photon emission computed tomography (SPECT) in the initial ass essment of cerebral blood flow in children with active CNS involvement . We extend these observations to longterm followup using the SPECT sc an to determine its usefulness in the subsequent course of CNS lupus i n children. Methods. Eleven children who developed CNS disease and ful filled the classification criteria for SLE were included in an open pi lot study. The patients were followed up to 3.5 years and presented wi th CNS manifestations: encephalopathy with or without grand mal seizur es (N = 4), focal seizures with depression or hallucinations (N = 3), optic neuritis with transverse myelitis (N = 2), and psychosis with au diovisual hallucinations (N = 2). Initially, all children had lumbar p uncture, SPECT and serologic testing; 9 children had electroencephalog ram (EEG), 7 had computerized tomography (CT), and 10 had magnetic res onance imaging (MRI). SPECT was repeated in 7 patients 1-4 months afte r the initial CNS event and thereafter in 10 patients annually. Result s. At the time of the initial CNS event, 9/11 children (82%) had norma l results for lumbar puncture, 7/9 (78%) for EEG, 5/7 (71%) for CT, an d 6/10 (60%) for MRI. All patients (100%) had diffusely abnormal SPECT . In addition, 5/11 (45%) tested positive for IgG antibodies to cardio lipin and dsDNA, and 4/11 (36%) had antibodies to Sm. In 5/7 children whose SPECT was repeated 1 to 4 months after the CNS event, additional perfusion defects were documented compared with initial SPECT During the subsequent 1-3.5 years and concomitant with treatment, CNS manifes tations resolved clinically, but none of the SPECT scans became normal . Perfusion defects improved over time in 4 patients and worsened in 6 . Conclusion. SPECT scan remains a sensitive tool during initial CNS e vents in children with CNS lupus documenting the presence of damage du ring short term followup of 1-4 months. However, during longterm follo wup abnormalities documented by SPECT no longer correlate with the pat ient's clinical course, limiting the usefulness of SPECT as a clinical tool in children who recover from CNS disease.