Abstract

Neuropsychiatric manifestations have a strong impact on morbidity and mortality in systemic lupus erythematosus (SLE). CNS manifestations have been described in 18–67 % of cases. The diagnosis of cerebral involvement can be difficult and has to be differentiated from neurological complications which result from infection, uraemia, hypertension, drugs, or metabolic changes. The pathogenesis of numerous CNS manifestations remains unclear. Immune complex-mediated vasculitis or bland vasculopathy induced by antiphospholipid antibodies might result in infarction and consecutive neurological deficits. Diffuse Psychiatric Symptoms may be induced by autoantibodies and cytokines, which may interact with the neuronal network. In neuropsychiatric SLE, the diagnostic approach includes brain imaging, duplex sonography of carotid and vertebral arteries, echocardiography to exclude embolism from valve lesions, serological tests including antiphospholipid antibodies, and analysis of cerebrospinal fluid of the brain. The imaging method of choice to investigate brain anatomy is magnetic resonance imaging (MRI) with gadolinium contrast enhancement. While MRI scans are highly sensitive in detecting infarction, atrophy and white matter lesions, patients with Psychiatric manifestations frequently have normal MRI results. Thus, inconspicuous MRI findings do not exclude neuropsychiatric lupus. In these patients, nuclear medicine techniques such as single-photon emission computed tomography (SPECT) and positron emission tomography (PET) may be useful in detecting perfusion defects and alterations of metabolism in morphologically normal appearing brain regions. However, due to the lack of specificity and oversensitivity, the findings of functional imaging have to be interpreted with some caution. Future studies will show whether the combination of MRI, functional brain imaging, serological, cerebrospinal and neuropsychological tests can be more specific than Single diagnostic procedures.

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