Reversible basal ganglia lesions in neuropsychiatric lupus: a report of three pediatric cases.

OBJECTIVE

Central nervous system involvement represents a serious and common complication of systemiclupus erythematosus (SLE). We describe the characteristics of patients with neuropsychiatric (NP) SLE complicated with reversible basal ganglia lesions.

METHODS

We describe the cases of three NPSLE patients.

RESULTS

They presented with NP manifestations such as headache, cognitive dysfunction, tremors, seizures, and mood disorder. The levels of autoantibodies to NMDA (N-methyl-d-aspartate) receptor antibodies and antiribosomal-P antibodies were elevated, indicating the presence of an acute phase. Marked elevation of interleukin-6 in cerebrospinal fluid was noted when these patients showed NP symptoms. Moreover, the patients presented with high-intensity lesions in the basal ganglia on T2-weighted images, fluid-attenuated inversion recovery (FLAIR) images, diffusion-weighted images (DWI) and apparent diffusion coefficient (ADC) maps. Following immunosuppressive treatment, almost complete improvement of the lesions was noted.

CONCLUSIONS

The reported cases indicate that reversible vasculopathies represent vasogenic edema localized in basal ganglia lesions and that activation of the autoimmune system and inflammation could lead to NP manifestations in SLE.