Antiphospholipid-Related Chorea

Abstract

Chorea is a movement disorder which may be associated with immunologic diseases, in particular in the presence of antiphospholipid antibodies (aPL). Choreic movements have been linked to the isolated presence of plasmatic aPL, or to primary, or secondary antiphospholipid syndrome. The highest incidence of aPL-related chorea is detected in children and females. The presentation of chorea is usually subacute and the course monophasic. Choreic movements can be focal, unilateral, or generalized. High plasmatic titers of aPL in a choreic patient can suggest the diagnosis of aPL-related chorea; neuroimaging investigation does not provide much additional diagnostic information. The most relevant target of aPL is β2-glycoprotein I, probably responsible for the thrombotic manifestations of antiphospholipid syndrome. Etiology of the movement disorder is not well understood but a neurotoxic effect of aPL has been hypothesized, leading to impaired basal ganglia cell function and development of neuroinflammation. Patients affected by aPL-related chorea have an increased risk of thrombosis and should receive antiplatelet or anticoagulant treatment.

Conventional and intravenous immunoglobulin therapy in paediatric antiphospholipid antibodies-related chorea

“…13 In adult patients other atypical neuroleptics and xenazine could represent effective alternatives, but there have been no controlled studies in children. 14 Valproic acid (20-25 mg/kg/day in two doses for 12 weeks) was considered effective and safe in the treatment of chorea, especially in severe cases when haloperidol treatment has failed. 13 Genel et al compared the efficacy of carbamazepine (15 mg/kg/day) with valproic acid (20-25 mg/kg/day) in 24 children affected by SC, showing no significant difference between the groups in term of clinical improvement, time to remission and recurrence rates.…”

Pediatric Motor Inflammatory Neuropathy: The Role of Antiphospholipid Antibodies

“…In the last few years, APS was increasingly recognized, both as a secondary condition to other autoimmune diseases, mainly systemic lupus erythematosus, and as a primary condition [1,[7][8][9][10]. APS and aPL are also increasingly being recognized in children.…”

Antiphospholipid syndrome: A diagnostic challenge

“…Genetic and environmental factors have also been shown to determine the occurrence and clinical expression of APS. 10 A small amount of antiphospholipid antibody is normal as it helps in removing dying and damaged cells. However people with APLA manifesting as APS have too much of APLA/abnormal variant of APLA and/or abnormal b2-glycoproteinI.…”