Skip to main content
Top
Published in: Acta Neurologica Belgica 2/2014

01-06-2014 | Original Article

Neurological presentations of the antiphospholipid syndrome: three illustrative cases

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

Published in: Acta Neurologica Belgica | Issue 2/2014

Login to get access

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.
Literature
1.
go back to reference Hughes G, Harris NN, Gharavi AE (1986) The anticardiolipin syndrome. J Rheumatol 13(3):486–489PubMed Hughes G, Harris NN, Gharavi AE (1986) The anticardiolipin syndrome. J Rheumatol 13(3):486–489PubMed
2.
go back to reference 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 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
3.
go back to reference 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 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
4.
go back to reference Bodiguel E (1992) Antiphospholipid antibodies and the central nervous system. Rev Neurol 148(11):655–662PubMed Bodiguel E (1992) Antiphospholipid antibodies and the central nervous system. Rev Neurol 148(11):655–662PubMed
5.
go back to reference 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 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
6.
go back to reference Giannakopoulos B, Krilis SA (2013) The pathogenesis of the antiphospholipid syndrome. N Engl J Med 368(11):1033–1044PubMedCrossRef Giannakopoulos B, Krilis SA (2013) The pathogenesis of the antiphospholipid syndrome. N Engl J Med 368(11):1033–1044PubMedCrossRef
7.
go back to reference 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 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
8.
go back to reference 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 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
9.
go back to reference Briley DP, Coull BM, Goodnight SH Jr (1989) Neurological disease associated with antiphospholipid syndrome. Ann Neurol 25:221–227PubMedCrossRef Briley DP, Coull BM, Goodnight SH Jr (1989) Neurological disease associated with antiphospholipid syndrome. Ann Neurol 25:221–227PubMedCrossRef
10.
go back to reference 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 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.
go back to reference Brey R (2005) Antiphospholipid syndrome in young adults with stroke. J Thromb Thrombolysis 20(2):105–112PubMedCrossRef Brey R (2005) Antiphospholipid syndrome in young adults with stroke. J Thromb Thrombolysis 20(2):105–112PubMedCrossRef
13.
go back to reference 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 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.
go back to reference 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 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.
go back to reference 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 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.
go back to reference 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 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.
go back to reference 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 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.
go back to reference Uthman IW, Khamashta MA (2006) Livedo racemosa: a striking dermatological sign for the antiphospholipid syndrome. J Rheumatol 33:12 Uthman IW, Khamashta MA (2006) Livedo racemosa: a striking dermatological sign for the antiphospholipid syndrome. J Rheumatol 33:12
19.
go back to reference Maamar M, Rahmani M, Aidi S et al (2007) Sneddon’s syndrome: 15 cases with cerebral angiography. Revue Neurologique 163(8–9):809–816PubMedCrossRef 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.
go back to reference 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 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.
22.
go back to reference 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 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.
go back to reference 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 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.
go back to reference Brey RL, Holliday SL, Saklad AR et al (2002) Neuropsychiatric syndromes in lupus: prevalence using standardized definitions. Neurology 58(8):1214–1220PubMedCrossRef Brey RL, Holliday SL, Saklad AR et al (2002) Neuropsychiatric syndromes in lupus: prevalence using standardized definitions. Neurology 58(8):1214–1220PubMedCrossRef
25.
go back to reference Joseph FG, Lammie GA, Scolding NJ et al (2007) CNS lupus: a study of 41 patients. Neurology 69:644–654PubMedCrossRef Joseph FG, Lammie GA, Scolding NJ et al (2007) CNS lupus: a study of 41 patients. Neurology 69:644–654PubMedCrossRef
26.
go back to reference 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 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.
go back to reference 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 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.
go back to reference Baizabal-Carvallo J, Alonso-Juarez M, Koslowski M (2011) Chorea in systemic lupus erythematosus. J Clin Rheumatol 17(2):69–72PubMedCrossRef Baizabal-Carvallo J, Alonso-Juarez M, Koslowski M (2011) Chorea in systemic lupus erythematosus. J Clin Rheumatol 17(2):69–72PubMedCrossRef
29.
go back to reference 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 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.
31.
go back to reference 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 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.
go back to reference 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 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.
go back to reference 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 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.
go back to reference Giannakopoulos B, Krilis SA (2013) The pathogenesis of the antiphospholipid syndrome. N Engl J Med 368(11):1033–1044PubMedCrossRef Giannakopoulos B, Krilis SA (2013) The pathogenesis of the antiphospholipid syndrome. N Engl J Med 368(11):1033–1044PubMedCrossRef
35.
go back to reference Lim W, Crowther MA, Eikelboom JW (2006) Management of antiphospholipid antibody syndrome: a systematic review. JAMA 295(9):1050–1057PubMedCrossRef Lim W, Crowther MA, Eikelboom JW (2006) Management of antiphospholipid antibody syndrome: a systematic review. JAMA 295(9):1050–1057PubMedCrossRef
36.
go back to reference 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 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.
go back to reference 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 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.
go back to reference Saadoun D, Piette J-C, Wahl D et al (2012) Management of antiphospholipid syndrome. Rev Med Interne 33(4):217–222PubMedCrossRef Saadoun D, Piette J-C, Wahl D et al (2012) Management of antiphospholipid syndrome. Rev Med Interne 33(4):217–222PubMedCrossRef
39.
go back to reference 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 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.
go back to reference Erkan D, Cervera R, Asherson RA (2003) Catastrophic antiphospholipid syndrome: where do we stand? Arthritis Rheum 48(12):3320–3327PubMedCrossRef Erkan D, Cervera R, Asherson RA (2003) Catastrophic antiphospholipid syndrome: where do we stand? Arthritis Rheum 48(12):3320–3327PubMedCrossRef
41.
go back to reference 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 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.
go back to reference 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 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.
go back to reference Arachchillage DJ, Cohen H (2013) Use of new oral anticoagulants in antiphospholipid syndrome. Curr Rheumatol Rep 15(6):331PubMedCrossRef Arachchillage DJ, Cohen H (2013) Use of new oral anticoagulants in antiphospholipid syndrome. Curr Rheumatol Rep 15(6):331PubMedCrossRef
44.
go back to reference Comarmond C, Cacoub P (2013) Antiphospholipid syndrome: from pathogenesis to novel immunomodulatory therapies. Autoimmun Rev 12(7):752–757 Comarmond C, Cacoub P (2013) Antiphospholipid syndrome: from pathogenesis to novel immunomodulatory therapies. Autoimmun Rev 12(7):752–757
45.
go back to reference 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 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
Metadata
Title
Neurological presentations of the antiphospholipid syndrome: three illustrative cases
Authors
Ch. De Maeseneire
M. C. Duray
M. P. Rutgers
M. Gille
Publication date
01-06-2014
Publisher
Springer Milan
Published in
Acta Neurologica Belgica / Issue 2/2014
Print ISSN: 0300-9009
Electronic ISSN: 2240-2993
DOI
https://doi.org/10.1007/s13760-013-0275-6

Other articles of this Issue 2/2014

Acta Neurologica Belgica 2/2014 Go to the issue