Neuropsychological Impairment in Systemic Lupus Erythematosus: A Comparison with Multiple Sclerosis

Abstract

In this manuscript, we review literature describing the neuropsychological and brain imaging characteristics of systemic lupus erythematosus (SLE) patients. The findings are compared and contrasted with multiple sclerosis (MS) studies, revealing similarities and differences of interest to clinicians and researchers. While cognitive impairment is somewhat less common in SLE than MS, the diseases share a similar cognitive profile with deficits most prominent on tests emphasizing the speed of information processing, working memory, and visual/spatial learning, and memory. In early or more mildly affected patients, diffuse white matter damage, which may not be apparent on conventional brain imaging, plays a major role in clinical presentation and cognitive testing. The causes of white matter damage are very different, however, and in later stages of the disease MS and SLE appear to give rise to different forms of cerebral pathology. MS may be characterized by increasing brain atrophy affecting especially the cortical and deep gray matter, at least after conversion to secondary progressive course. There is less evidence for neurodegenerative changes in SLE, but patients are increasingly at risk for cerebrovascular disease. We conclude by offering some suggestions for future clinical and imaging research. KeywordsSystemic lupus erythematosus; Multiple sclerosis; Cognition; Memory; Processing speed; MRI; Effect size Systemic lupus erythematosus (SLE) is a relapsing-remitting autoimmune disease with wide ranging organ involvement and clinical symptoms varying from mild and transient symptoms to death. Cognitive impairment is reported in SLE, even among those patients without documented or overt signs of central nervous system (CNS) involvement. Presumably, cognitive disorder is the result of underlying brain disease although the precise mechanisms remain to be elucidated. The prevalence and degree of cognitive impairment is also uncertain.In this article we will endeavor to evaluate the evidence for neuropsychological impairment in SLE, and to compare the findings with that of MS, another immunological disorder whose symptoms wax and wane and which affects women more than men. Unlike SLE, MS is a disease that always impacts the CNS, and the causes of cognitive disorder in MS are better understood.

Changes in Fatigue and Cognition in Patients with Relapsing Forms of Multiple Sclerosis Treated with Natalizumab

“…6,7 Aspects of cognitive functioning most commonly affected in MS patients include processing speed, learning, and memory. [6][7][8][9] Characterization of the relationship between cognitive performance and fatigue is complicated by the subjective experience of patients. 10 While patients often believe that fatigue contributes to poor cognitive performance, most research has not found a consistent association between fatigue and actual cognitive performance.…”

Cognitive and White Matter Tract Differences in MS and Diffuse Neuropsychiatric Systemic Lupus Erythematosus

“…2 Although SLE and MS have notable differences in etiology, there is a marked overlap concerning the CNS inflammatory and neurodegenerative nature of these 2 diseases. 3 In 1972, Fulford et al 4 first coined the term "lupoid sclerosis" when referring to patients with SLE who presented with symptoms similar to those in MS. Additionally, neuropsychological and cognitive testing revealed similarities in cognitive profiles of patients with MS and those with SLE that may be the result of similar dysfunctional CNS structures. 5,6 These neuropsychological findings included deficits in working memory and processing speed and general lower cognitive scores, even in the absence of a diagnosed neurologic or psychiatric condition.…”

Magnetic Resonance Imaging in Neuropsychiatric Lupus

“…However, many previous reports already proved increased frequency of WMH in SLE and NPSLE 21, 29– 36 . WMH had been shown to involve preferentially the frontal and parietal lobes, consistent with an anterior to posterior gradient, similar to other causes of WMH, but different from inflammatory demyelinating etiologies such as multiple sclerosis 8, 37 . In a quantitative cerebral MRI assessment, Appenzeller et al 34 showed that age, duration of neuropsychiatric manifestations and total corticosteroid dosage were independent predictors for WMH in SLE.…”

“…Nonetheless, advanced MRI sequences in NPSLE demonstrated underlying abnormalities in normal appearing white and grey matter, which shows the limitations of conventional sequences 51, 62, 63 . Recent studies used advanced MRI techniques in the analysis of NPSLE, as the assessment of tissue-specific atrophy by morphometric methods 37, 51, 64 , diffusion-tensor imaging 51, 63, 65, 66 , magnetization transfer imaging 51, 65, 66 , magnetic resonance spectroscopy 66, 67 and perfusion MRI.…”