Chorea and Systemic Lupus erythematosus

Abstract

A critical analysis of 52 cases of systemic lupus erythematosus (SLE) and chorea is presented. Choreatic movements occurred early in the course of the disease in most cases; the duration varied from several days to 3 years. No sex predominance was found and no relationship could be demonstrated between chorea and other neurological signs, or between chorea and other SLE manifestations. Also, the neuropathological findings offered no simple explanation for the emergence of chorea. Immunological pathomechanisms might be present.

Regional Specific Changes of Cerebral Metabolism in Systemic Lupus erythematosus Identified by Positron Emission Tomography

“…This may support the putative relation between (i) diffuse neurological symptoms of which chorea is an example, (ii) increased striatal glucose consumption and (iii) immune-mediated pathology with antibodies against neuronal tissue. In our study, we did not measure neuronal antibodies, but a causal role of such antibodies in the origin of diffuse neurological symptoms in SLE has been suggested in the literature [3, 4, 19]. In contrast, occlusion of small blood vessels, due to either vasculitis induced by immune-complex deposition or phospholipid-antibody-associated thrombosis, appears to play a dominant role in the genesis of focal neurological symptoms [4].…”

“…Focal deficit, presenting as a stroke-like event, has been described to be the result of small-vessel disease. On the other hand, the occurrence of diffuse symptoms, such as psychosis, chorea and cognitive impairment, may suggest a pathogenesis including immunoreactivity targeted against neuronal antigens [2, 3, 4]. Such a direct effect on cerebral tissue may implicate regional vulnerability to immune-mediated disease and makes one speculate on a possible equivalence with a syndrome such as paraneoplastic limbic encephalitis [5].…”