What is it about?

We present a unique case of a patient with neuropsychiatric manifestations of systemic lupus erythematosus (NPSLE). This patient developed aseptic meningitis with elevated interleukin-6 (IL-6) levels in the cerebrospinal fluid (CSF). Upon application of magnetic resonance imaging (DWI and ADC mapping), we identified symmetrical basal ganglia lesions, containing a cytotoxic edematous core, surrounded by vasogenic edema.

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Why is it important?

Because these specific brain MRI abnormalities are rare in patients with NPSLE, this represents a unique opportunity to better understand the pathogenesis of the disease. Indeed, we observed these lesions from a de novo appearance during the disease onset to its disappearance upon immunosuppressive treatment , indicating that the immune mediated mechanism underlying the pathogenesis of the vasogenic edema may be explained by blood barrier dysfunction. Further, the cytotoxic edema cores may be caused by cytokines or autoantibodies in the basal ganglia, though this warrants future studies of similar patients.

Perspectives

Further studies on similar NPSLE patients with basal ganglia involvement are warranted to better explore the role of basal ganglia involvement in the pathogenesis of NPSLE.

Tetsuji Sawada
Tokyo Ika Daigaku

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This page is a summary of: Clinical presentation of a neuropsychiatric lupus patient with symmetrical basal ganglia lesions containing cytotoxic oedema cores surrounded by vasogenic oedema, Modern Rheumatology Case Reports, August 2019, Taylor & Francis,
DOI: 10.1080/24725625.2019.1651955.
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