Background Epilepsy and seizures are commonly reported among the neurological manifestations of Antiphospholipid Syndrome (APS).
Objectives To discuss the epidemiological, pathophysiological, laboratory, clinical, radiological, and therapeutic aspects of epilepsy/seizures in APS/ antiphospholipid antibody (aPL)-positive patients.
Methods We searched the literature through Google Scholar and PubMed for all publications reporting on the epidemiology, pathogenesis, laboratory, imaging and associated clinical findings, and management of epilepsy/seizures in APS/aPL-positive patients. The following keywords were used: antiphospholipid, Hughes syndrome, anticardiolipin, lupus anticoagulant, anti-β2-glycoprotein I, partial, complex, generalized, and idiopathic epilepsy, seizure, and convulsion. All reports published between 1971 and 2015 were included.
Results Temporal lobe epilepsy is particularly prominent in many APS cases. The prevalence of epilepsy/seizures in APS patients is approximately 8%, which is nearly 10 times the prevalence of epilepsy in the general population (0.5–1%). The pathogenesis is complex and may involve micro-thrombosis and/or immune-mediated neuronal damage. The association of seizures with aPL is more significant in the presence of SLE. Seizure patients with positive aPL tend to develop thrombocytopenia and livedo racemosa more frequently than those without aPL. The presence of SLE and the presence of neurological symptoms significantly correlate with the presence of white matter changes on MRI. In contrast, the correlation between aPL positivity and the presence of white matter changes is very weak. However, MRI can be normal in more than 30–40% of patients. New MRI techniques can better elucidate the pathology of brain damage in neuro-APS. The theurapeutic management of epileptic APS patients relies on anti-epileptic treatment and anticoagulant agents when there is evidence of a thrombotic event. The anti-thrombotic benefit of hydroxychloroquine and statins is supported by several studies.
Conclusions MRI remains the imaging modality of choice. aPL testing is recommended in young patients presenting with atypical seizures and multiple hyper-intensity lesions on brain MRI in the absence of other possible conditions. In the absence of consensual recommendations, the decision of lifelong anticoagulation in epileptic APS patients is discussed on a case-by-case basis.
Disclosure of Interest None declared