Objective: Central nervous system (CNS) abnormalities have been reported in 30-60% of children with systemic lupus erythematosus (SLE) during the course of the disease. Unlike most other manifestations of childhood lupus, few laboratory studies and imaging modalities aid in the documentation of CNS lupus. Single photon emission computed tomography (SPECT) provides a means of assessing cerebral blood flow and may reveal subtle areas of decreased perfusion or loss of functioning brain parenchyma.
Methods: We evaluated 5 children with clinical signs of CNS lupus using SPECT, lumbar puncture, electroencephalogram (EEG), computerized 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 encephalopathy or psychosis (2) and transverse myelitis (1), focal seizure and depression (1), and severe headache and ophthalmitis (1).
Results: Four patients had anticardiolipin (aCL) antibodies. One girl with positive 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 analyses. No correlation was found between serologic variables and CNS disease. All 5 children had abnormal SPECT perfusion studies. CT and MRI failed to demonstrate abnormalities in 3 children. Although CT and MRI documented parietal lobe infarcts in one child and focal hemorrhage in another, poor perfusion found with SPECT extended beyond these abnormalities and into areas which appeared intact using the conventional imaging techniques. All children improved clinically and 4/5 had additional SPECT studies. In all 4, the perfusion abnormalities improved but did not resolve. One of these patients had a recurrence of hallucinations and worsening of SPECT findings which improved again after the patient stabilized.
Conclusions: We conclude that the cerebral perfusion SPECT scan is a sensitive tool and may prove useful in the documentation of CNS lupus in children.