Neurologic Manifestations of Systemic Lupus Erythematosus in Children and Adults
Section snippets
Prevalence of NPSLE Manifestations in Adults and Children
In adults, approximately 28% to 40% of NPSLE manifestations develop before or around the time of the diagnosis of SLE.5 Estimates of the prevalence of NPSLE have ranged from 14% to more than 80% in adults2, 5, 10, 11, 12 and 22% to 95% in children.4, 6, 7, 13, 14 A retrospective study of NPSLE in 185 Chinese children in a 20-year period found that 11% had NPSLE manifestations at the time of diagnosis and an additional 16% developed them within 1 year. The mortality rate in this study was 45% in
Pathophysiology and pathogenesis
The pathogenic causes of NPSLE manifestations are likely to be multifactorial and may involve autoantibody production, microangiopathy, intrathecal production of proinflammatory cytokines, and premature atherosclerosis.25 Cellular and parenchymal changes in lupus murine models include neuronal cytotoxicity and atrophy of dendritic spines.26 Cerebral spinal fluid from lupus-prone mice and adult patients with NPSLE reduce the viability of proliferating neural cell lines.27 Postmortem
Laboratory evaluation
There is no single diagnostic test that is sensitive and specific for SLE-related neuropsychiatric manifestations. The assessment of individual patients is based on clinical neurologic and rheumatologic evaluation, immunoserologic testing, brain imaging, and psychiatric and neuropsychological assessment. These examinations are used to support or refute the clinical diagnostic impression, exclude alternative explanations, and form the basis for prospective monitoring of clinical evolution and
Brain imaging
Appenzeller and colleagues48 have demonstrated a reduction in cerebral and corpus callosum volumes in adult SLE patients that are associated with disease duration and cognitive impairment and other CNS manifestations, but not total corticosteroid dose or the presence of aPL. Focal neurologic and neuropsychologic symptoms of SLE-related stroke correlate with structural magnetic resonance imaging (MRI) abnormalities. Using structural MRI, the majority (40%–80%) of abnormalities in NPSLE are small
Treatment
The management of patients with NPSLE includes symptomatic and immunosuppressive therapies, but evidence for the efficacy of the treatment modalities commonly used is largely limited to uncontrolled clinical trials and anecdotal experience.60 The key to treatment is to first establish the correct diagnosis by carefully following the guidelines for the diagnosis of NPSLE syndromes in the ACR 1999 case definitions.9 For many NPSLE syndromes, symptomatic treatment may also be needed in addition to
Summary
NPSLE manifestations are common in children and adults and a significant source of morbidity and mortality. The adoption of the NPSLE case definitions by the ACR has led to major advances in our ability to study nervous system manifestation of SLE and to identify homogeneous groups of patients from multiple studies for comparison purposes. The integrity of the blood-brain barrier seems to be important in preventing some brain pathology associated with SLE. It is possible that some of the
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