nach oben

Acta Neurologica Belgica

Erschienen in:

01.06.2014 | Original Article

Neurological presentations of the antiphospholipid syndrome: three illustrative cases

verfasst von: Ch. De Maeseneire, M. C. Duray, M. P. Rutgers, M. Gille

Erschienen in: Acta Neurologica Belgica | Ausgabe 2/2014

Einloggen, um Zugang zu erhalten

Abstract

The antiphospholipid syndrome (APS) is defined by the association of high titers of antiphospholipid antibodies (aPLs) with thrombotic events and/or obstetrical problems. APS can be isolated or associated with immune system diseases. Several central nervous system (CNS) manifestations have been reported in APS, but are still not included in the international diagnostic criteria. We present here three cases of APS revealed by CNS manifestations. The first patient had a primary APS with stroke, dementia, epilepsy and the “so-called” Liebman–Sacks disease, a subacute thrombotic non-bacterial valvulopathy. The second one developed a primary APS with a Sneddon syndrome, while the third case is a neurolupus-associated APS with subacute encephalopathy, chorea, stroke, and epilepsy. The pathogenesis of the APS is related to both prothrombotic and immunologic effects of the aPLs. Long-term anticoagulation by low-weight heparin or warfarin is currently recommended in APS. We propose to search the presence of aPLs in any case of young adults’ stroke, unexplained dementia, and acquired chorea.

Hughes G, Harris NN, Gharavi AE (1986) The anticardiolipin syndrome. J Rheumatol 13(3):486–489PubMed

Miyakis S, Lockshin MD, Atsumi T et al (2006) International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 4(2):295–306PubMedCrossRef

Urbanus RT, Siegerink B, Roest M et al (2009) Antiphospholipid antibodies and risk of myocardial infarction and ischaemic stroke in young women in the RATIO study: a case–control study. Lancet Neurol 8(11):998–1005PubMedCrossRef

Bodiguel E (1992) Antiphospholipid antibodies and the central nervous system. Rev Neurol 148(11):655–662PubMed

Morrissette N, Gorman T (2010) Antiphospholipid syndrome and homozygous factor V Leiden mutation in a young patient with Libeman–sacks endocarditis and stroke. Am J Med 123(9):e3–e4CrossRef

Giannakopoulos B, Krilis SA (2013) The pathogenesis of the antiphospholipid syndrome. N Engl J Med 368(11):1033–1044PubMedCrossRef

Cervera R, Bucciarelli S, Plasín MA et al (2009) Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the “CAPS Registry”. J Autoimmun 32(3–4):240–245PubMedCrossRef

Asherson RA, Khamashta MA, Gil A et al (1989) Cerebrovascular disease and antiphospholipid antibodies in systemic lupus erythematosus, lupus-like disease, and the primary antiphospholipid syndrome. Am J Med 86:391–399PubMedCrossRef

Briley DP, Coull BM, Goodnight SH Jr (1989) Neurological disease associated with antiphospholipid syndrome. Ann Neurol 25:221–227PubMedCrossRef

10.

Krause I, Lev S, Fraser A et al (2005) Close association between valvar heart disease and central nervous system manifestations in the antiphospholipid syndrome. Ann Rheum Dis 64(10):1490–1493PubMedCentralPubMedCrossRef

11.

Brey R (2005) Antiphospholipid syndrome in young adults with stroke. J Thromb Thrombolysis 20(2):105–112PubMedCrossRef

12.

Muscal E, Brey RL (2010) Antiphospholipid syndrome and the brain in pediatric and adult patients. Lupus 19(4):406–411PubMedCentralPubMedCrossRef

13.

Ferro JM, Canhão P, Stam J et al (2004) Prognosis of cerebral vein and dural sinus thrombosis: results of the international study on cerebral vein and dural sinus thrombosis (ISCVT). Stroke 35:664–670PubMedCrossRef

14.

Cervera R, Piette JC, Font J et al (2002) Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1000 patients. Arthritis Rheum 46(4):1019–1027PubMedCrossRef

15.

Gomez-Puerta JA, Cervera R, Calvo LM et al (2005) Dementia associated with the antiphospholipid syndrome: clinical and radiological characteristics of 30 patients. Rheumatology 44:95–99PubMedCrossRef

16.

Marinho JL, Piovesan EJ, Pereira Leite Neto M et al (2007) Clinical, neurovascular and neuropathological features in Sneddon’s syndrome. Arq Neuropsiquiatr 65(2B):390–395PubMedCrossRef

17.

Montané de la Roque P, Michard JF, Pinganaud-Shrestha C et al (2006) Syndrome démentiel révélant un syndrome de Sneddon. Rev Med Interne 27:156–164CrossRef

18.

Uthman IW, Khamashta MA (2006) Livedo racemosa: a striking dermatological sign for the antiphospholipid syndrome. J Rheumatol 33:12

19.

Maamar M, Rahmani M, Aidi S et al (2007) Sneddon’s syndrome: 15 cases with cerebral angiography. Revue Neurologique 163(8–9):809–816PubMedCrossRef

20.

Dutra L, Braga-Neto P, Pedroso JL et al (2012) Sneddon’s syndrome: case report and review of its relationship with antiphospholipid syndrome. Einstein 10(2):230–232PubMedCrossRef

21.

El Benaye J, Hsaini Y, Zoobo T et al (2013) Le syndrome de Sneddon. Presse Med 42:138–144PubMedCrossRef

22.

Stockhammer G, Stephan R, Felber R et al (1993) Sneddon’s syndrome. Diagnostic by skin biopsy and RMI in 17 patients. Stroke 24:685–690PubMedCrossRef

23.

Francès C, Papo T, Wechsler B et al (1999) Sneddon syndrome with or without antiphospholipid antibodies. A comparative study in 46 patients. Medicine (Baltimore) 78(4):209–219CrossRef

24.

Brey RL, Holliday SL, Saklad AR et al (2002) Neuropsychiatric syndromes in lupus: prevalence using standardized definitions. Neurology 58(8):1214–1220PubMedCrossRef

25.

Joseph FG, Lammie GA, Scolding NJ et al (2007) CNS lupus: a study of 41 patients. Neurology 69:644–654PubMedCrossRef

26.

Spinosa MJ, Bandeira M, Liberalesso PB et al (2007) Clinical, laboratory and neuroimage findings in juvenile systemic lupus erythematosus presenting involvement of the nervous system. Arq Neuropsiquiatr 65(2B):433–439PubMedCrossRef

27.

Asherson RA, Derksen RH, Harris EN et al (1987) Chorea in systemic lupus erythematosus and “lupus-like” disease: association with antiphospholipid antibodies. Semin Arthritis Rheum 16(4):253–259PubMedCrossRef

28.

Baizabal-Carvallo J, Alonso-Juarez M, Koslowski M (2011) Chorea in systemic lupus erythematosus. J Clin Rheumatol 17(2):69–72PubMedCrossRef

29.

Cervera R, Asherson RA, Font J et al (1997) Chorea in the antiphospholipid syndrome. Clinical, radiologic, and immunologic characteristics of 50 patients from our clinics and the recent literature. Medicine (Baltimore) 76(3):203–212CrossRef

30.

de la Fuente-Fernández R (1997) Lupus anticoagulant and chorea. Neurology 49(2):639–640PubMedCrossRef

31.

Avcin T, Benseler SM, Tyrrell PN et al (2008) A follow-up study of antiphospholipid antibodies and associated neuropsychiatric manifestations in 137 children with systemic lupus erythematosus. Arthritis Rheum 59(2):206–213PubMedCrossRef

32.

Erkan D, Harrison MJ, Levy R et al (2007) Aspirin for primary thrombosis prevention in the antiphospholipid syndrome: a randomized, double-blind, placebo-controlled trial in asymptomatic antiphospholipid antibody-positive individuals. Arthritis Rheum 56(7):2382–2391PubMedCrossRef

33.

Cervera R, Khamashta MA, Shoenfeld Y et al (2009) Morbidity and mortality in the antiphospholipid syndrome during a 5-year period: a multicentre prospective study of 1000 patients. Ann Rheum Dis 68(9):1428–1432PubMedCrossRef

34.

Giannakopoulos B, Krilis SA (2013) The pathogenesis of the antiphospholipid syndrome. N Engl J Med 368(11):1033–1044PubMedCrossRef

35.

Lim W, Crowther MA, Eikelboom JW (2006) Management of antiphospholipid antibody syndrome: a systematic review. JAMA 295(9):1050–1057PubMedCrossRef

36.

Crowther MA, Ginsberg JS, Julian J et al (2003) A comparison of two intensities of warfarin for the prevention of recurrent thrombosis in patients with the antiphospholipid antibody syndrome. NEJM 349:1133–1138PubMedCrossRef

37.

Finazzi G, Marchioli R, Brancaccio V et al (2005) A randomized clinical trial of high-intensity warfarin vs. conventional antithrombotic therapy for the prevention of recurrent thrombosis in patients with the antiphospholipid syndrome (WAPS). J Thromb Haemost 3:848–853PubMedCrossRef

38.

Saadoun D, Piette J-C, Wahl D et al (2012) Management of antiphospholipid syndrome. Rev Med Interne 33(4):217–222PubMedCrossRef

39.

Asherson RA, Cervera R, Piette JC et al (2001) Catastrophic antiphospholipid syndrome: clues to the pathogenesis from a series of 80 patients. Medicine (Baltimore) 80:355–376CrossRef

40.

Erkan D, Cervera R, Asherson RA (2003) Catastrophic antiphospholipid syndrome: where do we stand? Arthritis Rheum 48(12):3320–3327PubMedCrossRef

41.

Statkute L, Traynor A, Oyama Y et al (2005) Antiphospholipid syndrome in patients with systemic lupus erythematosus treated by autologous hematopoietic stem cell transplantation. Blood 106(8):2700–2709PubMedCrossRef

42.

Erkan D, Vega J, Ramón G et al (2013) A pilot open-label phase II trial of rituximab for non-criteria manifestations of antiphospholipid syndrome. Arthritis Rheum 65(2):464–471PubMedCrossRef

43.

Arachchillage DJ, Cohen H (2013) Use of new oral anticoagulants in antiphospholipid syndrome. Curr Rheumatol Rep 15(6):331PubMedCrossRef

44.

Comarmond C, Cacoub P (2013) Antiphospholipid syndrome: from pathogenesis to novel immunomodulatory therapies. Autoimmun Rev 12(7):752–757

45.

Lopez-Pedrera C, Ruiz-Limon P, Aguirre MA et al (2012) Potential use of statins in the treatment of antiphospholipid syndrome. Curr Rheumatol Rep 14(1):87–94PubMedCrossRef

Titel: Neurological presentations of the antiphospholipid syndrome: three illustrative cases
verfasst von: Ch. De Maeseneire
M. C. Duray
M. P. Rutgers
M. Gille
Publikationsdatum: 01.06.2014
Verlag: Springer Milan
Erschienen in: Acta Neurologica Belgica / Ausgabe 2/2014
Print ISSN: 0300-9009
Elektronische ISSN: 2240-2993
DOI: https://doi.org/10.1007/s13760-013-0275-6

Leitlinien kompakt für die Neurologie

Mit medbee Pocketcards sicher entscheiden.

^{Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag}

Kostenlos registrieren

Neu im Fachgebiet Neurologie

Niedriger diastolischer Blutdruck erhöht Risiko für schwere kardiovaskuläre Komplikationen

25.04.2024 Hypotonie Nachrichten

Wenn unter einer medikamentösen Hochdrucktherapie der diastolische Blutdruck in den Keller geht, steigt das Risiko für schwere kardiovaskuläre Ereignisse: Darauf deutet eine Sekundäranalyse der SPRINT-Studie hin.

Frühe Alzheimertherapie lohnt sich

25.04.2024 AAN-Jahrestagung 2024 Nachrichten

Ist die Tau-Last noch gering, scheint der Vorteil von Lecanemab besonders groß zu sein. Und beginnen Erkrankte verzögert mit der Behandlung, erreichen sie nicht mehr die kognitive Leistung wie bei einem früheren Start. Darauf deuten neue Analysen der Phase-3-Studie Clarity AD.

Viel Bewegung in der Parkinsonforschung

25.04.2024 Parkinson-Krankheit Nachrichten

Neue arznei- und zellbasierte Ansätze, Frühdiagnose mit Bewegungssensoren, Rückenmarkstimulation gegen Gehblockaden – in der Parkinsonforschung tut sich einiges. Auf dem Deutschen Parkinsonkongress ging es auch viel um technische Innovationen.

Demenzkranke durch Antipsychotika vielfach gefährdet

23.04.2024 Demenz Nachrichten

Wenn Demenzkranke aufgrund von Symptomen wie Agitation oder Aggressivität mit Antipsychotika behandelt werden, sind damit offenbar noch mehr Risiken verbunden als bislang angenommen.

Update Neurologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen

Springer Medizin

Abstract

Bitte loggen Sie sich ein, um Zugang zu diesem Inhalt zu erhalten

Weitere Artikel der Ausgabe 2/2014

Right hand knob infarction

Rupture of the middle cerebral artery aneurysm as a presenting symptom of late-onset Pompe disease in an adult with a novel GAA gene mutation

Delayed intravenous thrombolysis based on MRI mismatch in posterior circulation stroke

Cerebral venous sinus thrombosis in a child with iron-deficiency anemia

Progressive bilateral medial medullary infarction