LAUSR

A 58-year-old postmenopausal woman had a personal history of aortic mechanical valve replacement 2 years ago, for which she received oral anticoagulation therapy with AVK (acenocoumarol). A year later, she experienced recurrent sudden-onset severe headache, reaching peak intensity within 1 min, and spontaneously resolving within few minutes. On admission a day after the onset of symptoms, her examination, blood pressure, and laboratory tests were normal. The international normalized ratio (INR) was within the required range (2–3). Brain magnetic resonance imaging (MRI) with magnetic resonance angiography (MRA) of the brain and vessels of the head and neck and magnetic resonance venography (MRV) performed on admission was normal. AVK was discontinued the same day, and she received low molecular weight heparin (LMWH) at a curative dosage instead. Lumbar puncture was performed 2 days later, 12 h after the last dose of LMWH as soon as INR dropped to 1.37. Result of the cerebrospinal fluid (CSF) study (including CSF-biliary pigments and CSF pressure) was also normal. During hospitalization, she presented recurrent attacks of TCH occurring spontaneously or triggered by effortless miction or emotion. A control brain MRI with MRA and MRV was performed 10 days after the onset of symptoms and showed the characteristic Bstring of beads^ pattern of alternating diffuse segmental areas of arterial stenosis and dilation affecting bilaterally distal vessels of the insular and cortical segments of the middle cerebral arteries and collateral branches of the posterior cerebral arteries (Fig. 1a). The diagnosis of reversible cerebral vasoconstriction syndrome was considered. She received nimodipine at the dose of 60 mg every 4 h per os, with a total resolution of headache attacks within 3 days. The treatment was maintained at the same dose for 1 month. Nimodipine was progressively tapered with a total treatment duration of 2 months. AVK therapy was also reintroduced within 1 month of hospitalization, and LMWHwas discontinued as soon as INRwas within the required range. A control brain MRI with MRA, performed at 3 and 6 months, confirmed the total regression of arterial abnormalities (Fig. 1b). At 1 year follow-up, she did not experience any further headache attacks.