Lupus in the Child’s Mind

A pediatric version of the ANAM (Ped-ANAM) software has been designed for use in children who are age 10 years or older; this may be a good screening tool for neurocognitive dysfunction in children with SLE.¹¹ Ped-ANAM testing takes about 30–40 minutes and can be done by the child with little guidance. This test is designed to assess sustained concentration and attention, mental flexibility, spatial processing, cognitive-processing efficiency, arousal/fatigue level, learning, recall, and working memory. More details can be obtained at www.c-shop.ou.edu.

Imaging to Diagnose Neurocognitive Dysfunction

Neuroimaging is a logical means to noninvasively investigate SLE-associated neurocognitive dysfunction, because clinical and laboratory tests have not facilitated timely diagnosis or the understanding of its pathology. Although there has been evidence of cortical and subcortical neuropathology demonstrated by magnetic resonance imaging (MRI), magnetization transfer imaging (MTI), and FLAIR images, correlation of these findings with clinical symptoms has not helped diagnose neurocognitive dysfunction or assess its response to treatment. Findings on conventional MRI have been inconclusive, as lesions are common with SLE in general, and not specifically correlated with, nor sensitive to, change of active neurocognitive dysfunction.

Given the lack of utility of these standard MRI techniques, functional neuroimaging using positron emission tomography and single-photon emission computed tomography have been investigated. Disadvantages of these methods include exposure to radiation and contrast, poor spatial resolution, and a lack of correlation to specific NPSLE syndromes. Metabolic brain studies using magnetic resonance spectrometry (MRS) suggest that the level of cognitive functioning, and NPSLE in general, are associated with both white matter and gray matter changes.¹²

Figure 2: fMRI images for assessing the effects of childhood-onset SLE on brain activation patterns when testing attention using a continuous performance paradigm. Differences in activation (yellow) and suppression (blue) patterns in children with SLE and healthy controls. In SLE, larger brain areas need to be activated for performing an fMRI task and fewer are suppressed.

Other recently used techniques include diffusion-weighted imaging (DWI), which can provide quantitative measurements of brain diffusivity.^13,14 Interestingly, DWI shows that patients with SLE who test positive for anti-NR2 antibodies have more severe damage to the amygdala compared with SLE patients without anti-NR2 antibodies. The results from advanced imaging techniques, such as diffusion tensor imaging, functional MRI, and diffusion-weighted imaging, in a group of adult patients with various NPSLE syndromes, suggest that the combination of various imaging techniques provides complementary and synergistic information. However, none of these studies is diagnostic.