Sneddon’s Syndrome as rare and underdiagnosed cause of stroke: about two cases / A Síndrome de Sneddon como causa rara e subdiagnosticada de AVC: cerca de dois casos

Case 1. Female, 27 years old, with migraine aura. Use of combined contraceptive therapy. She presented visual and cutaneous complaints for eight months, using prednisone 60mg/day due to the hypothesis systemic vasculitis. On examination, right temporal hemianopsia, normal fundus eye, Raynaud ‘s phenomenon necrosis stitches in fingers livedo were noted. Brain MRI diffusion restriction left occipital lobe, hypersignal foci parietal head caudate nucleus. Reticular Livedo (RL) compatible biopsy. Anticardiolipin IgM (13.3 U/mL), ESR (47mm/h 1st hour 52mm/h 2nd hour), VDRL 1:8 reagents. Normal CSF. Initially treated warfarin, she has been rivaroxaban two years, remaining asymptomatic. 2. 39 a report sudden dysarthria hemiparesis, previous episode vertigo. A lumbosacral spine was performed, presenting no alterations. Antiphospholipid antibodies: Beta-2-glycoprotein I 21 UR/mL IgG 9 UR/mL, lupus anticoagulant 0.98, anticardiolipin 9.4 GPL 31 MPL. Cranial showed old ischemic gaps hyperintense areas on T2/FLAIR affecting paramedian portion pons, related acute stroke. Presence distal region limbs. In current use warfarin 7.5mg/day, remission symptoms. Discussion: Sneddon Syndrome (SS) is non-inflammatory thrombotic vasculopathy characterized by concomitant presence reticularis Neurological manifestations are present up 77% cases. It more prevalent women between 20 42 old. Treatment aimed at preventing potentially serious, disabling, even fatal new vascular events. Final Comments: SS rare syndrome that probably results from series acquired or congenital hemostatic abnormalities preferentially involve cerebral beds. Although it specific biomarker, there reports associating antiphospholipid antibodies reactor. Clinical presentation, laboratory findings skin biopsy associated an excellent response treatment fundamental diagnostic elucidation.