Background: Cardiac involvement in Sneddon syndrome (SS) is rare, the physiopathology is still unclear. We report a first case of SS without antiphospholipid antibodies who had coexisting ischaemia with no obstructive coronary arteries and aortic valve diseases.
Case Summary: A 34-year-old woman with SS without antiphospholipid antibodies, was admitted for aphasia, and paresthaesia with confirmed right opercular ischaemic lesions at brain magnetic resonance imaging. Transthoracic echocardiographic examination showed akinesis of apical segments, moderate aortic valve stenosis, and moderate aortic insufficiency. Coronary angiogram was normal. Cardiac magnetic resonance showed transmural necrosis in the territory of the left anterior descending artery. Seven years later, our patient had no change or progression of myocardial ischaemic lesions or valvular disease.
Conclusion: We will discuss different hypothesis, diagnosis, treatment, and evolution of cardiac involvement in SS. Close follow-up should be regularly performed for early diagnosis, hence the importance of multimodality imaging, to guide treatment and prevent further complications.
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http://dx.doi.org/10.1093/ehjcr/ytab211 | DOI Listing |
J Cent Nerv Syst Dis
December 2024
Faculty of Medicine, The Hashemite University, Zarqa, Jordan.
Background: Sneddon's syndrome is a rare thrombotic vasculopathy characterized by the coexistence of both cerebrovascular events and livedo reticularis.
Objective: This review aims to raise awareness among physicians by discussing the whole clinical spectrum of the disease. Typically, Sneddon syndrome presents in middle-aged women with a cerebrovascular accident and a preexisting skin rash, which is livedo reticularis.
Ann Neurol
February 2025
Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Skinmed
August 2024
Department of Dermatology, Venereology & Leprosy, Himalayan Institute of Medical Sciences, Swami Rama Himalayan University, Dehradun, Uttarakhand, India.
J Dtsch Dermatol Ges
July 2024
Department of Dermatology and Venereology, Martin Luther University Halle-Wittenberg, Halle (Saale), Germany.
Background: Sneddon syndrome is an occlusive vasculopathy that presents clinically with generalized livedo racemosa on the skin and transient ischemic attacks, strokes, and cognitive or motor deficits in the central nervous system. Antiplatelet or anticoagulant therapy is recommended. Due to the limited therapeutic efficacy and the resulting serious complications, we propose combination therapy with additional infusion cycles of alprostadil and captopril and report initial long-term results.
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