Central nervous system involvement in the antiphospholipid (Hughes) syndrome.

The antiphospholipid (Hughes) syndrome (APS) is characterized by arterial and/or venous thrombosis and pregnancy morbidity in the presence of anticardiolipin antibodies and/or lupus anticoagulant. APS can occur either as a primary disorder or secondary to a connective tissue disease, most frequently systemic lupus erythematosus. Central nervous system (CNS) involvement is one of the most prominent clinical manifestations of APS, and includes arterial and venous thrombotic events, psychiatric features and a variety of other non-thrombotic neurological syndromes. In this review we focus on the common and some of the less common CNS manifestations that have been reported in association with antiphospholipid antibodies.

[1]  G. Hughes,et al.  Neuropsychiatric manifestations in systemic lupus erythematosus: prevalence and association with antiphospholipid antibodies. , 2003, The Journal of rheumatology.

[2]  F. Jennekens,et al.  The central nervous system in systemic lupus erythematosus. Part 2. Pathogenetic mechanisms of clinical syndromes: a literature investigation. , 2002, Rheumatology.

[3]  F. Jennekens,et al.  The central nervous system in systemic lupus erythematosus. Part 1. Clinical syndromes: a literature investigation. , 2002, Rheumatology.

[4]  G. Hughes,et al.  Bleeding and recurrent thrombosis in definite antiphospholipid syndrome: analysis of a series of 66 patients treated with oral anticoagulation to a target international normalized ratio of 3.5. , 2002, Archives of internal medicine.

[5]  J. Moroney,et al.  Neuromyelitis optica (Devic's syndrome) in systemic lupus erythematosus: a case report. , 2002, Rheumatology.

[6]  A. Tobar,et al.  Bacterial induction of autoantibodies to beta2-glycoprotein-I accounts for the infectious etiology of antiphospholipid syndrome. , 2002, The Journal of clinical investigation.

[7]  G. Hughes,et al.  Headache and memory loss: rapid response to heparin inthe antiphospholipid syndrome , 2001, Lupus.

[8]  S. Kittner,et al.  &bgr;2-Glycoprotein 1–Dependent Anticardiolipin Antibodies and Risk of Ischemic Stroke and Myocardial Infarction: The Honolulu Heart Program , 2001, Stroke.

[9]  M. Petri Management of thrombosis in antiphospholipid antibody syndrome. , 2001, Rheumatic diseases clinics of North America.

[10]  M. Khamashta,et al.  Sticky blood and headache , 2001, Lupus.

[11]  P. Amarenco,et al.  Recurrent stroke and vascular events in elderly patients with anticardiolipin antibodies: a prospective study , 2001, Journal of Neurology.

[12]  I. Milanov,et al.  Antiphospholipid syndrome and dystonia-parkinsonism. A case report. , 2001, Parkinsonism & related disorders.

[13]  X. Montalban,et al.  Anticardiolipin antibodies are not a useful screening tool in a nonselected large group of patients with multiple sclerosis , 2001, Annals of neurology.

[14]  G. Hughes,et al.  Migraine in Hughes syndrome--heparin as a therapeutic trial? , 2001, QJM : monthly journal of the Association of Physicians.

[15]  A. Auvinen,et al.  Antiphospholipid and antinuclear antibodies in patients with epilepsy or new-onset seizure disorders. , 2000, The American journal of medicine.

[16]  G. Rechavi,et al.  Factor V Leiden and antiphospholipid antibodies are significant risk factors for ischemic stroke in children. , 2000, Stroke.

[17]  L. Weinehall,et al.  Anticardiolipin antibodies are not an independent risk factor for stroke: an incident case-referent study nested within the MONICA and Västerbotten cohort project. , 2000, Stroke.

[18]  J. Vallat,et al.  Prevalence and clinical significance of anti-phospholipid antibodies in multiple sclerosis: a study of 89 patients. , 2000, Journal of autoimmunity.

[19]  F. J. Alvarez,et al.  Ultrastructural Localization of Monoclonal Antiphospholipid Antibody Binding to Rat Brain , 2000, Experimental Neurology.

[20]  R. Hilker,et al.  Cerebral blood flow and glucose metabolism in multi-infarct-dementia related to primary antiphospholipid antibody syndrome , 2000, Lupus.

[21]  G. Hughes,et al.  Migraine and stroke in young women. , 2000, QJM : monthly journal of the Association of Physicians.

[22]  N. Gadoth,et al.  Idiopathic intracranial hypertension and anticardiolipin antibodies , 2000, Journal of neurology, neurosurgery, and psychiatry.

[23]  R. DeHoratius,et al.  Transverse myelopathy in systemic lupus erythematosus: an analysis of 14 cases and review of the literature , 2000, Annals of the rheumatic diseases.

[24]  A. Mosek,et al.  Dementia and Antiphospholipid Antibodies , 2000, Dementia and Geriatric Cognitive Disorders.

[25]  M. Khamashta,et al.  Can Neurologic Manifestations of Hughes (Antiphospholipid) Syndrome Be Distinguished from Multiple Sclerosis?: Analysis of 27 Patients and Review of the Literature , 2000, Medicine.

[26]  S. Tuhrim,et al.  Antiphosphatidyl serine antibodies are independently associated with ischemic stroke. , 1999, Neurology.

[27]  Daniel B Hier,et al.  Anticardiolipin antibodies are an independent risk factor for ischemic stroke. , 1999, Neurological research.

[28]  J. Weinberger,et al.  Elevated anticardiolipin antibody titer is a stroke risk factor in a multiethnic population independent of isotype or degree of positivity. , 1999, Stroke.

[29]  D. Isenberg,et al.  A longitudinal study of anticardiolipin antibody levels and cognitive functioning in systemic lupus erythematosus. , 1999, Arthritis and rheumatism.

[30]  J. Fisk,et al.  A prospective analysis of cognitive function and anticardiolipin antibodies in systemic lupus erythematosus. , 1999, Arthritis and rheumatism.

[31]  D. Alarcón-Segovia,et al.  Serum anti-β2-glycoprotein-I and anticardiolipin antibodies during thrombosis in systemic lupus erythematosus patients , 1999 .

[32]  W. Brooks,et al.  Neurometabolite markers of cerebral injury in the antiphospholipid antibody syndrome of systemic lupus erythematosus. , 1998, Stroke.

[33]  D. Karussis,et al.  A subgroup of multiple sclerosis patients with anticardiolipin antibodies and unusual clinical manifestations: Do they represent a new nosological entity? , 1998, Annals of neurology.

[34]  C. Besana,et al.  Partial Seizures Associated with Antiphospholipid Antibodies in Childhood , 1998, Neuropediatrics.

[35]  R. Leker,et al.  Anticardiolipin antibodies are frequently present in patients with idiopathic intracranial hypertension. , 1998, Archives of neurology.

[36]  S. Levine,et al.  Role of anticardiolipin antibodies in young persons with migraine and transient focal neurologic events , 1998, Neurology.

[37]  R. Liblau,et al.  Systemic autoimmune features and multiple sclerosis: a 5-year follow-up study. , 1998, Archives of neurology.

[38]  H. Spiera,et al.  Sudden sensorineural hearing loss in patients with systemic lupus erythematosus or lupus-like syndromes and antiphospholipid antibodies. , 1998, The Journal of rheumatology.

[39]  L. Metz,et al.  The Frequency of Phospholipid Antibodies in an Unselected Stroke Population , 1998, Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques.

[40]  D. Attias,et al.  High association of anticardiolipin antibodies with psychosis. , 1998, The Journal of clinical psychiatry.

[41]  G. Ruiz-Argüelles,et al.  Refractory hiccough heralding transverse myelitis in the primary antiphospholipid syndrome , 1998, Lupus.

[42]  G. Hughes,et al.  Outcome of patients with anticardiolipin antibodies: a 10 year follow-up of 52 patients , 1998, Lupus.

[43]  S. Levine,et al.  Cerebral venous thrombosis and anticardiolipin antibodies. , 1997, Stroke.

[44]  G. Espinosa,et al.  Optic neuropathy in the “primary” antiphospholipid syndrome: Report of a case and review of the literature , 1997, Clinical Rheumatology.

[45]  D. Nagaraja,et al.  Anticardiolipin antibodies in ischemic stroke in the young: Indian experience , 1997, Journal of the Neurological Sciences.

[46]  R. Fyffe,et al.  Monoclonal antiphosphatidylserine antibodies react directly with feline and murine central nervous system. , 1997, The Journal of rheumatology.

[47]  S. Levine,et al.  IgG anticardiolipin antibody titer > 40 GPL and the risk of subsequent thrombo-occlusive events and death. A prospective cohort study. , 1997, Stroke.

[48]  A. Korczyn,et al.  Neurological dysfunction and hyperactive behavior associated with antiphospholipid antibodies. A mouse model. , 1997, The Journal of clinical investigation.

[49]  J. Ilany,et al.  Valvular dysfunction in antiphospholipid syndrome: prevalence, clinical features, and treatment. , 1997, Seminars in arthritis and rheumatism.

[50]  L. Podoshin,et al.  Autoimmune aberration in sudden sensorineural hearing loss: association with anti-cardiolipin antibodies , 1997, Lupus.

[51]  P. Genton,et al.  Prevalence and signification of antinuclear and anticardiolipin antibodies in patients with epilepsy. , 1997, The American journal of medicine.

[52]  J. Denburg,et al.  The relationship of antiphospholipid antibodies to cognitive function in patients with systemic lupus erythematosus , 1997, Journal of the International Neuropsychological Society.

[53]  J. Schröder,et al.  Primary antiphospholipid antibody syndrome and retinal occlusive vasculopathy. , 1997, American journal of ophthalmology.

[54]  Á. Chamorro,et al.  Chorea in the Antiphospholipid Syndrome: Clinical, Radiologic, and Immunologic Characteristics of 50 Patients from Our Clinics and the Recent Literature , 1997, Medicine.

[55]  M. Khamashta,et al.  Endothelial cells as target for antiphospholipid antibodies. Human polyclonal and monoclonal anti-beta 2-glycoprotein I antibodies react in vitro with endothelial cells through adherent beta 2-glycoprotein I and induce endothelial activation. , 1997, Arthritis and rheumatism.

[56]  G. Davies-Jones,et al.  Potentially prothrombotic abnormalities of coagulation in benign intracranial hypertension. , 1997, Journal of neurology, neurosurgery, and psychiatry.

[57]  L. Wilkins Anticardiolipin Antibodies and the Risk of Recurrent Thrombo-occlusive Events and Death , 1997, Neurology.

[58]  J. Provenzale,et al.  Patients with antiphospholipid antibodies: CT and MR findings of the brain. , 1996, AJR. American journal of roentgenology.

[59]  E. Harris,et al.  In Vivo Models of Thrombosis for the Antiphospholipid Syndrome , 1996, Lupus.

[60]  F. Chédru,et al.  Coagulation studies, factor V Leiden, and anticardiolipin antibodies in 40 cases of cerebral venous thrombosis. , 1996, Stroke.

[61]  M. Tsai,et al.  Review : Elevated levels of anticardiolipin antibodies and epilepsy in lupus patients , 1996, Lupus.

[62]  J. Lasala,et al.  Activation of cultured vascular endothelial cells by antiphospholipid antibodies. , 1995, The Journal of clinical investigation.

[63]  Kristina J. Liu,et al.  Report of two cases , 1995 .

[64]  G. Hughes,et al.  Association of antiphospholipid antibodies with central nervous system disease in systemic lupus erythematosus. , 1995, The American journal of medicine.

[65]  G. Orefice,et al.  Benign intracranial hypertension: a non-thrombotic complication of the primary antiphospholipid syndrome? , 1995, Lupus.

[66]  S. Levine,et al.  Recurrent stroke and thrombo‐occlusive events in the antiphospholipid syndrome , 1995, Annals of neurology.

[67]  B. Tumiati,et al.  Sudden sensorineural hearing loss and anticardiolipin antibody. , 1995, American journal of otolaryngology.

[68]  B. Tumiati,et al.  Cogan's syndrome: A new possible complication of antiphospholipid antibodies? , 1995, Clinical Rheumatology.

[69]  G. Hughes,et al.  Association between antiphospholipid antibodies and epilepsy in patients with systemic lupus erythematosus. , 1994, Arthritis and rheumatism.

[70]  G. Hughes,et al.  Ischaemic optic neuropathy, transverse myelitis, and epilepsy in an anti-phospholipid positive patient with systemic lupus erythematosus. , 1994, Journal of neurology, neurosurgery, and psychiatry.

[71]  A. Kurita,et al.  A young case with multi-infarct dementia associated with lupus anticoagulant. , 1994, Internal medicine.

[72]  T. Scott,et al.  Antiphospholipid antibody syndrome mimicking multiple sclerosis clinically and by magnetic resonance imaging. , 1994, Archives of internal medicine.

[73]  U. Reinhold,et al.  Cerebral blood flow in Sneddon syndrome. , 1994, Journal of nuclear medicine : official publication, Society of Nuclear Medicine.

[74]  K. Muir,et al.  Anticardiolipin antibodies and cerebral infarction. , 1994, Journal of neurology, neurosurgery, and psychiatry.

[75]  M. Lockshin,et al.  Neurologic complications of antiphospholipid antibodies. , 1993, Rheumatic diseases clinics of North America.

[76]  R. Brey Anticardiolipin antibodies are an independent risk factor for first ischemic stroke , 1993, Neurology.

[77]  S. Komune,et al.  Anticardiolipin antibody-induced sudden profound sensorineural hearing loss. , 1993, American journal of otolaryngology.

[78]  J. Fisk,et al.  COGNITIVE IMPAIRMENT AND AUTOANTLBODIES IN SYSTEMIC LUPUS ERYTHEMATOSUS , 1993 .

[79]  M. Stampfer,et al.  Anticardiolipin Antibodies and the Risk for Ischemic Stroke and Venous Thrombosis , 1992, Annals of Internal Medicine.

[80]  J. Sixma,et al.  Antiphospholipid Antibody Positive Sera Enhance Endothelial Cell Procoagulant Activity – Studies in a Thrombosis Model , 1992, Thrombosis and Haemostasis.

[81]  M. Backonja,et al.  Neuropathologic findings in multi-infarct dementia associated with anticardiolipin antibody. Evidence for endothelial injury as the primary event. , 1992, Arthritis and rheumatism.

[82]  C. Tsai,et al.  Inhibition of astrocyte proliferation and binding to brain tissue of anticardiolipin antibodies purified from lupus serum. , 1992, Annals of the rheumatic diseases.

[83]  G. Tietjen Migraine and Antiphospholipid Antibodies , 1992, Cephalalgia : an international journal of headache.

[84]  J. Vianna,et al.  Lack of association between anticardiolipin antibodies and migraine in systemic lupus erythematosus , 1992, Neurology.

[85]  A. Khalili,et al.  A study of immune responses to myelin and cardiolipin in patients with systemic lupus erythematosus (SLE) , 1991, Clinical and experimental immunology.

[86]  K. Jain Systemic Lupus Erythematosus (SLE)-Like Syndromes Associated with Carbamazepine Therapy , 1991, Drug safety.

[87]  K. Welch,et al.  Cerebrovascular and neurologic disease associated with antiphospholipid antibodies , 1990, Neurology.

[88]  M. Khamashta,et al.  Association of antibodies against phospholipids with heart valve disease in systemic lupus erythematosus , 1990, The Lancet.

[89]  E. Nasonov,et al.  Anticardiolipin antibodies in Sneddon's syndrome , 1990, Neurology.

[90]  J. Sánchez-Guerrero,et al.  Antiphospholipid Antibodies and the Antiphospholipid Syndrome in Systemic Lupus Erythematosus A Prospective Analysis of 500 Consecutive Patients , 1989, Medicine.

[91]  M. Vilardell,et al.  [Multi-infarct dementia associated with antiphospholipid antibodies. Presentation of 2 cases]. , 1989, Medicina clinica.

[92]  G. Hughes,et al.  Cerebrovascular disease and antiphospholipid antibodies in systemic lupus erythematosus, lupus-like disease, and the primary antiphospholipid syndrome. , 1989, The American journal of medicine.

[93]  D. Lillicrap,et al.  Thrombotic endocarditis and lupus anticoagulant. A pathogenetic possibility for idiopathic 'rheumatic type' valvular heart disease. , 1989, Archives of pathology & laboratory medicine.

[94]  D. Briley,et al.  Neurological disease associated with antiphospholipid antibodies , 1989, Annals of neurology.

[95]  N. Simonian,et al.  Lupus anticoagulants, anticardiolipin antibodies, and cerebral ischemia. , 1989, Stroke.

[96]  R. Inzelberg,et al.  Lupus anticoagulant and late onset seizures , 1989, Acta neurologica Scandinavica.

[97]  O. Suchowersky,et al.  Migraine and Anti‐Phospholipid Antibodies , 1989, Headache.

[98]  D. Lillicrap,et al.  Lupus Anticoagulant, Antiphospholipid Antibodies and Migraine , 1988, Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques.

[99]  M. Khamashta,et al.  Chorea in systemic lupus erythematosus: association with antiphospholipid antibodies. , 1988, Annals of the rheumatic diseases.

[100]  O. Devinsky,et al.  Clinical and neuropathological findings in systemic lupus erythematosus: The role of vasculitis, heart emboli, and thrombotic thrombocytopenic purpura , 1988, Annals of neurology.

[101]  Hughes Gr,et al.  Antiphospholipid antibodies and chorea. , 1988 .

[102]  G. Hughes,et al.  Recurrent stroke and multi-infarct dementia in systemic lupus erythematosus: association with antiphospholipid antibodies. , 1987, Annals of the rheumatic diseases.

[103]  K. Welch,et al.  The spectrum of neurologic disease associated with antiphospholipid antibodies. Lupus anticoagulants and anticardiolipin antibodies. , 1987, Archives of neurology.

[104]  K. Welch,et al.  Cerebral venous thrombosis with lupus anticoagulants. Report of two cases. , 1987, Stroke.

[105]  S. Levine,et al.  Migraine and the Lupus Anticoagulant: Case Reports and Review of the Literature , 1987, Cephalalgia : an international journal of headache.

[106]  G. Hughes,et al.  Chorea in systemic lupus erythematosus and "lupus-like" disease: association with antiphospholipid antibodies. , 1987, Seminars in arthritis and rheumatism.

[107]  G. Hughes,et al.  Lupoid sclerosis: a possible pathogenetic role for antiphospholipid antibodies. , 1985, Annals of the rheumatic diseases.

[108]  J. Chiras,et al.  Cerebral venous thrombosis--a review of 38 cases. , 1985, Stroke.

[109]  G. Hughes,et al.  Epilepsy: an early symptom of systemic lupus erythematosus. , 1985, Journal of neurology, neurosurgery, and psychiatry.

[110]  G. Hughes Connective tissue disease and the skin , 1984 .

[111]  J. Berciano,et al.  Cerebral arterial occlusion and intracranial venous thrombosis in a woman taking oral contraceptives. , 1984, Postgraduate medical journal.

[112]  A. Gharavi,et al.  ANTIPHOSPHOLIPID ANTIBODIES IN ACUTE GUILLAIN-BARRÉ SYNDROME , 1983, The Lancet.

[113]  C. Mackworth-Young,et al.  ANTICARDIOLIPIN ANTIBODIES: DETECTION BY RADIOIMMUNOASSAY AND ASSOCIATION WITH THROMBOSIS IN SYSTEMIC LUPUS ERYTHEMATOSUS , 1983, The Lancet.

[114]  G. Hughes Thrombosis, abortion, cerebral disease, and the lupus anticoagulant. , 1983, British medical journal.

[115]  L Cocito,et al.  Epileptic seizures in cerebral arterial occlusive disease. , 1982, Stroke.

[116]  R. Foà,et al.  Collagen vascular disease appearing as chorea gravidarum. , 1982, Archives of neurology.

[117]  B. Estañol,et al.  Intracraniai Venous Thrombosis in Young Women , 1979, Stroke.

[118]  V. Saldivar,et al.  Chorea associated with systemic lupus erythematosus: report of two cases and review of the literature. , 1978, Pediatrics.

[119]  W. Weiner,et al.  The pharmacology of choreatic movement disorders , 1976, Progress in Neurobiology.

[120]  F. Sj An attack of chorea complicating oral contraceptive therapy. , 1966 .

[121]  S. Levine,et al.  Short Communications Cerebral Venous Thrombosis with Lupus Anticoagulants , 2005 .

[122]  G. Hughes,et al.  The central nervous system in systemic lupus erythematosus , 2004, Rheumatology International.

[123]  Johan Smith,et al.  Case reports and review of the literature , 2003 .

[124]  V. Adhiyaman,et al.  Antiphospholipid syndrome and dystonia-parkinsonism , 2002 .

[125]  F. Chiarelli,et al.  Lack of association between antiphospholipid antibodies and migraine in children , 2000, International journal of clinical & laboratory research.

[126]  G. Hughes,et al.  Prevalence of headache and relationship with the presence of antiphospholipid antibodies in systemic lupus erythematosus , 2000 .

[127]  D. Alarcón-Segovia,et al.  Serum anti-beta2-glycoprotein-I and anticardiolipin antibodies during thrombosis in systemic lupus erythematosus patients. , 1999, The American journal of medicine.

[128]  L. Bonomo,et al.  The clinico-ophthalmological spectrum of antiphospholipid syndrome. , 1998, Ocular immunology and inflammation.

[129]  A. Camaiti,et al.  Venous dural sinus thrombosis in a middle-aged woman with anticardiolipin antibodies. , 1995, Acta neurologica Belgica.

[130]  C. R. Wang,et al.  Anticardiolipin antisera from lupus patients with seizures reduce a GABA receptor-mediated chloride current in snail neurons. , 1994, Life sciences.

[131]  J. Hogg Magnetic resonance imaging. , 1994, Journal of the Royal Naval Medical Service.

[132]  D. Tanné,et al.  Autoantibodies to phospholipids and brain extract in patients with the Guillain-Barre syndrome: cross-reactive or pathogenic? , 1993, Autoimmunity.

[133]  J. Fisk,et al.  Cognitive impairment and autoantibodies in systemic lupus erythematosus. , 1993, British journal of rheumatology.

[134]  J. Sánchez-Guerrero,et al.  Transverse myelitis: a manifestation of systemic lupus erythematosus strongly associated with antiphospholipid antibodies. , 1990, The Journal of rheumatology.

[135]  G. Hughes,et al.  Transient global amnesia and antiphospholipid antibodies. , 1989, Clinical and experimental rheumatology.

[136]  G. Hughes,et al.  Antiphospholipid antibodies and chorea. , 1988, The Journal of rheumatology.

[137]  G. Hughes The Prosser-White oration 1983. Connective tissue disease and the skin. , 1984, Clinical and experimental dermatology.

[138]  G. Hughes,et al.  Cerebral infarction in systemic lupus: association with anticardiolipin antibodies. , 1984, Clinical and experimental rheumatology.

[139]  S. Fernando An attack of chorea complicating oral contraceptive therapy. , 1966, The Practitioner.

[140]  a presentation of two cases with , 2022 .