CEREBRAL PERFUSION ABNORMALITIES IN CHILDREN WITH CENTRAL-NERVOUS-SYSTEM MANIFESTATIONS OF LUPUS DETECTED BY SINGLE-PHOTON EMISSION COMPUTED-TOMOGRAPHY

Objective. Central nervous system (CNS) abnormalities have been report ed in 30-60% of children with systemic lupus erythematosus (SLE) durin g the course of the disease. Unlike most other manifestations of child hood lupus, few laboratory studies and imaging modalities aid in the d ocumentation of CNS lupus. Single photon emission computed tomography (SPECT) provides a means of assessing cerebral blood flow and may reve al subtle areas of decreased perfusion or loss of functioning brain pa renchyma. Methods. We evaluated 5 children with clinical signs of CNS lupus using SPECT, lumbar puncture, electroencephalogram (EEG), comput erized tomogram (CT) and magnetic resonance Imaging (MRI), as well as autoantibody and complement serologic testing. All patients fulfilled classification criteria for SLE and within one year of onset presented with the following CNS manifestations: grand mal seizures with enceph alopathy or psychosis (2) and transverse myelitis (1), focal seizure a nd depression (1), and severe headache and ophthalmitis (1). Results. Four patients had anticardiolipin (aCL) antibodies. One girl with posi tive aCL had a concurrent ischemic event involving both parietal lobes and another had a CNS bleed. Both of these children had abnormal EEG, CT and MRI scans. All children had normal cerebral spinal fluid analy ses. No correlation was found between serologic variables and CNS dise ase. All 5 children had abnormal SPECT perfusion studies. CT and MRI f ailed to demonstrate abnormalities in 3 children. Although CT and MRT documented parietal lobe infarcts in one child and focal hemorrhage in another, poor perfusion found with SPECT extended beyond these abnorm alities and into areas which appeared intact using the conventional im aging techniques. All children improved clinically and 4/5 had additio nal SPECT studies. In all 4, the perfusion abnormalities improved but did not resolve. One of these patients had a recurrence of hallucinati ons and worsening of SPECT findings which improved again after the pat ient stabilized.