Chorea, a little-known manifestation in systemic lupus erythematosus: short literature review and four case reports

Abstract

Chorea is a movement disorder that may be found in children due to several causes. Here we focus especially on Systemic Lupus Erythematosus associated chorea. First we outline its epidemiology, hypothesized pathogenesis, clinical presentation and treatment, then we report four significant clinical cases, which represent well the extreme variability of set of symptoms that may accompany lupus chorea. Our experience, according to literature, suggests that choreic movements in a child should alert the pediatrician and lead him to investigate a potential neurological involvement of Systemic Lupus Erythematosus.

A Rare Presentation of Systemic Lupus Erythematosus during Childhood: Chorea

“…Many studies have described a close relationship between chorea that is seen in patients with SLE and antiphospholipid antibodies (1,2,7,8). In our case, antiphospholipid antibodies tested negative.…”

“…It has been reported that immune complexes, autoantibodies, and cytokines cause neurological disorders in SLE with direct effects and that vasculopathy developing in arterioles that feed the basal ganglia, coagulopathy, and bleeding disorders cause neurological disorders with indirect effects (2,5).…”

“…The most common type of movement disorder is chorea, and the child age group has been shown as 0-5% in the course of SLE in different series (1)(2)(3)(4).…”

“…Other causes include cerebrovascular diseases, hyperthyroidism, poisoning, Huntington's disease, and collagen vascular diseases such as SLE (7). Chorea may occur at any time of SLE; it may also be a symptom for presenting to a clinic (2,3). Our patient presented due to involuntary movements that started at the extremities.…”

“…It may occur in the extremities, face, neck, and trunk. Sydenham's chorea, which is one of the major cause of ARF, is the most common cause of chorea in children (2,6). Other causes include cerebrovascular diseases, hyperthyroidism, poisoning, Huntington's disease, and collagen vascular diseases such as SLE (7).…”