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      Rapidly progressive intracranial artery stenosis in primary antiphospholipid syndrome

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          Abstract

          Antiphospholipid antibody syndrome (APS) is usually a disease of young adults. In elderly stroke patients APS was not associated with progressive intracerebral stenosis in the past. Here, we report a 65-year-old patient who presented with recurrent ischemic strokes associated with progressive stenosis of the right middle cerebral artery. Antiphospholipid antibodies were detected and treatment with plasma exchange, tapered steroids, and anticoagulants was successful. This case demonstrates that APS should be considered also in elderly stroke patients. This is of particular relevance since APS confers a significant risk to angioplasty and stenting procedures which therefore should be avoided in APS.

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          Stenting versus aggressive medical therapy for intracranial arterial stenosis.

          Marc I Chimowitz, Michael J Lynn, Colin Derdeyn … (2011)
          Atherosclerotic intracranial arterial stenosis is an important cause of stroke that is increasingly being treated with percutaneous transluminal angioplasty and stenting (PTAS) to prevent recurrent stroke. However, PTAS has not been compared with medical management in a randomized trial. We randomly assigned patients who had a recent transient ischemic attack or stroke attributed to stenosis of 70 to 99% of the diameter of a major intracranial artery to aggressive medical management alone or aggressive medical management plus PTAS with the use of the Wingspan stent system. The primary end point was stroke or death within 30 days after enrollment or after a revascularization procedure for the qualifying lesion during the follow-up period or stroke in the territory of the qualifying artery beyond 30 days. Enrollment was stopped after 451 patients underwent randomization, because the 30-day rate of stroke or death was 14.7% in the PTAS group (nonfatal stroke, 12.5%; fatal stroke, 2.2%) and 5.8% in the medical-management group (nonfatal stroke, 5.3%; non-stroke-related death, 0.4%) (P=0.002). Beyond 30 days, stroke in the same territory occurred in 13 patients in each group. Currently, the mean duration of follow-up, which is ongoing, is 11.9 months. The probability of the occurrence of a primary end-point event over time differed significantly between the two treatment groups (P=0.009), with 1-year rates of the primary end point of 20.0% in the PTAS group and 12.2% in the medical-management group. In patients with intracranial arterial stenosis, aggressive medical management was superior to PTAS with the use of the Wingspan stent system, both because the risk of early stroke after PTAS was high and because the risk of stroke with aggressive medical therapy alone was lower than expected. (Funded by the National Institute of Neurological Disorders and Stroke and others; SAMMPRIS ClinicalTrials.gov number, NCT00576693.).
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            Antiphospholipid syndrome: frequency, main causes and risk factors of mortality.

            Ricard Cervera, Gerard Espinosa (2010)
            The presence of antiphospholipid antibodies has been shown to be related to an increased risk of thrombotic events. In patients with definite antiphospholipid syndrome (APS), that is, those who have had thrombosis and at least two positive determinations of antiphospholipid antibodies, secondary thromboprophylaxis with long-term anticoagulation therapy results in a low rate of recurrent thrombotic events, ranging from 0.016 to 0.031 events per patient per year. Thrombotic complications are, however, the most common cause of death in APS. The mortality rate in a large European cohort of patients with APS during a 5-year study period was 5.3%, and up to 40% of the deaths in this cohort were attributed to severe thrombotic events such as myocardial infarction, stroke and pulmonary embolism. Catastrophic APS is an unusual form of the disease, being observed in less than 1% of reported cases of APS, which is associated with a much higher mortality rate than classical APS. The combined use of anticoagulation, corticosteroids, plasma exchange and intravenous immunoglobulin therapy could result in a dramatic reduction in mortality, by approximately 20%, in patients with catastrophic APS.
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              CAPS Registry.

              R Cervera (2012)
              Patients with catastrophic antiphospholipid syndrome (APS) have in common: a) clinical evidence of multiple organ involvement developing over a very short period of time; b) histopathological evidence of multiple small vessel occlusions, and c) laboratory confirmation of the presence of antiphospholipid antibodies (aPL), usually in high titre. Although patients with catastrophic APS represent less than 1% of all patients with APS, they are usually in a life-threatening situation. The rarity of this syndrome makes it extraordinarily difficult to study in any systematic way. In order to correlate all the published case reports as well as newly diagnosed cases from all over the world, an international registry of patients with catastrophic APS (CAPS Registry) was created in 2000 by the European Forum on aPL. Currently, it documents the clinical, laboratory and therapeutic data of more than 400 patients and can be consulted through Internet at www.med.ub.es/MIMMUN/FORUM/CAPS.HTM. The analysis of this registry has allowed the characterization of the clinical and laboratory features of the catastrophic APS as well as the establishment of preliminary criteria for its classification and guidelines for its management.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Ann Clin Transl Neurol
                Journal ID (iso-abbrev): Ann Clin Transl Neurol
                Journal ID (publisher-id): acn3
                Title: Annals of Clinical and Translational Neurology
                Publisher: John Wiley & Sons, Ltd (Chichester, UK )
                ISSN (Print): 2328-9503
                ISSN (Electronic): 2328-9503
                Publication date (Print): July 2015
                Publication date (Electronic): 25 May 2015
                Volume: 2
                Issue: 7
                Pages: 780-782
                Affiliations
                [1 ]Neurologische Klinik und Poliklinik, Klinikum rechts der Isar der Technischen Universität München Munich, Germany
                [2 ]Abteilung für Rheumatologie, Klinikum rechts der Isar der Technischen Universität München Munich, Germany
                [3 ]Abteilung für Neuroradiologie, Klinikum rechts der Isar der Technischen Universität München Munich, Germany
                Author notes
                Correspondence Christian L. Seifert, Neurologische Klinik und Poliklinik, Klinikum rechts der Isar der Technische Universität München, Ismaninger Str. 22, 81675 München, Germany., Tel: +49 89 4140 4601;, Fax: +49 89 4140 4675;, E-mail: c.seifert@ 123456lrz.tu-muenchen.de

                Funding Information There was no funding for the conduction of this case study.

                Article
                DOI: 10.1002/acn3.215
                PMC ID: 4531061
                SO-VID: a47a6c0f-c63c-48b6-9ac2-5b9bf930f753
                Copyright © © 2015 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals, Inc on behalf of American Neurological Association.
                License:

                This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

                History
                Date received : 02 March 2015
                Date revision received : 14 April 2015
                Date accepted : 23 April 2015
                Categories
                Subject: Case Study

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