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APS and the Nervous System

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Antiphospholipid Antibody Syndrome

Part of the book series: Rare Diseases of the Immune System ((RDIS))

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Abstract

Cerebrovascular disease is the most frequent clinical manifestation of the antiphospholipid syndrome (APS) at disease onset, after deep venous thrombosis. At a 10-year follow-up, it represents the overall most common clinical event related to antiphospholipid antibodies (aPL). Besides thrombotic events affecting brain circulation, a wide range of “non-criteria” neurological manifestations has been associated with aPL such as dementia, epilepsy, chorea, headache, multiple sclerosis, myelopathy, peripheral neuropathy, hearing loss, and ocular syndromes. aPL display a particular tropism for cerebral circulation, which might be partially explained by the peculiarity of brain endothelial cells. However, aPL might induce neurological manifestations not only because of pro-thrombotic mechanisms but also because they can bind to neurons and astrocytes, disrupting their function. Ischemic manifestations of APS always require the initiation of either antiplatelet drugs or long-term anticoagulants; no standard treatment is available for nonvascular neurological manifestations of APS. The wide heterogeneity in neurological presentation of APS represents a challenge for clinicians: it is important to promptly recognize and effectively treat them in early stages, in order to avoid diagnostic and therapeutic delay.

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Authors and Affiliations

  1. Department of Clinical Sciences and Community Health, University of Milan, Via Festa del Perdono 7, Milan, 20122, Italy

    Cecilia Beatrice Chighizola

  2. Immunology Research Laboratory, IRCCS Istituto Auxologico Italiano, Via Zucchi 18, Cusano Milanino, Milan, 20095, Italy

    Cecilia Beatrice Chighizola

  3. Department of Neurology – Stroke Unit, IRCCS Istituto Auxologico Italiano, University of Milan, P. Brescia 20, Milan, 20149, Italy

    Davide Sangalli, Barbara Corrà, Vincenzo Silani & Laura Adobbati

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  1. Cecilia Beatrice Chighizola
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  2. Davide Sangalli
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  3. Barbara Corrà
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  4. Vincenzo Silani
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  5. Laura Adobbati
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Correspondence to Cecilia Beatrice Chighizola .

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Editors and Affiliations

  1. Department of Rheumatology, Gaetano Pini Institute, Milan, Milano, Italy

    Pier Luigi Meroni

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Chighizola, C.B., Sangalli, D., Corrà, B., Silani, V., Adobbati, L. (2015). APS and the Nervous System. In: Meroni, P. (eds) Antiphospholipid Antibody Syndrome. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-319-11044-8_8

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  • DOI: https://doi.org/10.1007/978-3-319-11044-8_8

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