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The antiphospholipid syndrome is characterised by thrombotic and/or recurrent fetal loss/pregnancy morbidity associated with persistent antiphospholipid antibodies, namely lupus anticoagulant, anticardiolipin antibodies or anti-ß2 glycoprotein-1 antibodies. It is defined as primary when occurring without any underlying autoimmune disorder or secondary when associated with chronic inflammatory conditions, such as systemic lupus erythematosus, Sjögren’s syndrome and rheumatoid arthritis.
Although persistent antiphospholipid antibodies are associated with many other clinical manifestations, these are not considered diagnostic for the antiphospholipid syndrome. The most commonly associated neurological disorders are stroke, transient ischaemic attacks and migraine, but also sometimes epilepsy, transverse myelitis, multiple sclerosis-like presentations, ocular symptoms, Guillain-Barré syndrome, cognitive impairment, dementia and very rarely chorea.
Neurologists frequently see patients who test positive for antiphospholipid antibody or lupus anticoagulant. These might be only mildly elevated, present transiently or have no discernible clinical relationship, and we therefore often appropriately dismiss them. Indeed, up to 5% of the normal population test positive for these antibodies. However, when significantly elevated and clinically relevant, they …
Competing interests None declared.
Provenance and peer review Commissioned; internally peer reviewed.