Cerebral watershed infarct as the first manifestation of systemic lupus erythematosus and antiphospholipid syndrome

Abstract

Introduction: Cerebral watershed infarcts are strokes that affect regions supplied by two main arterial territories. It is usually related to hemodynamic compromise or hypercoagulable state as noted in antiphospholipid syndrome. We present a case of a young patient, who developed this type of stroke as the initial manifestation of antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE). Objectives: To report an unusual case of watershed zone stroke in the context of APS and SLE. Case report: A previously healthy 15-year-old male patient presented fever and myalgia followed by dropped head and quadriparesis, which progressively worsened in the next five days. Initial neurological examination revealed quadriparesis, predominantly in the proximal muscles MRC grade II with hypertonia and pyramidal signs. Brain magnetic resonance imaging evidenced acute internal borderzone infarct in the territories of the anterior cerebral artery, and middle cerebral artery, bilaterally. Vessel wall imaging and cerebrospinal fluid showed no evidence of vasculitis. Computed tomography angiography revealed no large vessel occlusion, aneurysm or dissection, but showed right sigmoid sinus thrombosis. Laboratory tests revealed pancytopenia(hemoglobin 8,9; white blood cell 3510, 580 lymphocytes and 71.000 platelets) with positive direct Coombs, complement consumption, elevated erythrocyte sedimentation rate ( 47 mm/1st hour), positive anti-dsDNA (>1:1280), anti-SSA and anti-SM-RNP. The Lupus anticoagulant, anti-beta2GPI and anticardiolipin antibodies were positive, filling laboratorial criteria for SLE and APS. Transthoracic and transesophagic echocardiography were unremarkable. The patient was treated with high-dose corticosteroids (1.5 g methylprednisolone in three days) and anticoagulants, presenting a progressive recovery of strength. Conclusion: This case report reasures the important correlation between borderzone infarcts and hypercoagulable states. Antiphospholipid syndrome must be always remembered in these patients.