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Marfan syndrome is a genetically inherited systemic disease of connective tissue, usually characterized by skeletal, cardiovascular and ocular involvement. We present a 44 years-old male, with heart valve and lens subluxation surgery, presented to the outpatient clinic with complaint of weakness on the left side. He was receiving anticoagulant treatment. His parents were second-degree relatives, and his 16-years-old son had aortic valve regurgitation and lens subluxation. The patient was extremely tall and thin with typical musculoskeletal features of Marfan syndrome. These included pectus excavatus deformity, ulnar deviation in both hands, as well as swan neck deformity, long fingers, joint hypermobility, and right-sided scoliosis. Neurological examination revealed irregular pupil on the right, left-sided hemiparesis, hyperreflexia, and piramidal signs on the left side. Brain magnetic resonance imaging showed infarction in the temporo-parietal region of the right hemisphere
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