Background The primary antiphospholipid syndrome (APS) is characterized by arterial and/or venous thrombosis and pregnancy morbidity in the presence of anticardiolipin antibodies (aCL) and/or lupus anticoagulant (LA). In addition to prothrombotic effects of aPL on the brain, there are immunologic effects with proof of direct binding of aPL to various types of brain cells, presented as cognitive dysfunction (CD),migraine,seizure, multiple sclerosis-like syndrome (MS-like), transverse myelitis (TM), movement disorders, or psychiatric symptoms.
Methods We examined 3 patients who were diagnosed primary APS.The patients were assessed with careful history taking, physical examination, blood laboratory evaluation, andMRI or head-CT.Our main objective was to describe neurologic manifestations of APS in our patients at Valme University Hospital and the response to treatment with hydroxychloroquine
Results Among the 3 patients, there was male preponderance with 3 men and no-women.The mean age of presentation was 33.66±5.2 years (range, 23 50 years) and with a current mean age of 37±14,93 years.There was no mortality in our series. One of them debuted with sudden loss of consciousness along with jaw stiffness and posterior amnesia.The other one presented prickling sensation and involuntary movement of his right upper limb extending to right lower limbs without posterior generalization, and the third patient consulted with frontal, pulsating headache and binocular diplopia.100% were LA positive with prolongated Activated Partial Thromboplastin Time and dilute Russell viper venom time and negative for ANA with no collagenosis sign or symptoms. Neither presented complement alteration or cerebrospinal fluid variation.We observed positivity for aCL in one patient (33%). In 2 of 3 patients (66.66%), cortico-subcortical space occupant lesions (SOL) were observed, some of them with contrast enhancement, mimicking demyelinating lesion, while the other patient didn’t present any abnormality in the MRI images.All patients presented an appropriate response to treatment with prednisone,in downward treatment regimen, aspirin and hydroxychloroquine 200 mg twice a day. At the four-year follow-up, all of them remain asymptomatic.We observed a lessening of SOL in MRI images due to the treatment in two patients but the third one presented new lesions due to suspension od prednisone, which was reintroduced, lessening of SOL.
Conclusions The neurological affection presented in APS can mimic multiple sclerosis symptoms and it is difficult to differentiate both entities That is why aPL determination should be part of screening tests and should not be delayed if the diagnosis of APS is suspected.Hydroxychloroquine could be a valid treatment in CNS manifestations in patients with APS
Funding Source(s): None
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