Skip to main content

Advertisement

SpringerLink
Log in

Reversible cerebral vasoconstriction syndrome (RCVS) in antiphospholipid antibody syndrome (APLA): the role of centrally acting vasodilators. Case series and review of literature

  • Case Based Review
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is Raynaud's phenomenon of the brain. Changes in neurological function are dependent upon which areas of the brain are deprived of normal blood flow. Antiphospholipid antibody syndrome (APLA) is a common cause of Raynaud's phenomenon that can occur anywhere in the body, including the brain. Management of CNS vasospasm generally involves the use of centrally acting calcium channel blockers, which have been shown to relieve the associated headaches and transient neurological symptoms associated with it. Three patients with APLA and RCVS from our clinics are illustrated. It is demonstrated that the use of centrally acting calcium channel-blocking drugs, such as nimodipine, which prevent and reverse CNS vasospasm, led to clinical improvement in our patients over the course of 5–9 years. All of them had MRIs done at the initiation of therapy and 5–9 years after being on therapy. MRI measures of T2 lesion volumes (LVs) and number were obtained. All three patients had a good response in controlling clinical symptoms related to CNS vasospasm, Raynaud's phenomenon, visual disturbances, confusion, headaches, and hearing loss. There was also a resolution in the MRI findings of these patients. This case series of three patients shows a clinical improvement and decrease in T2 LV and number in patients with APLA and Raynaud's syndrome on centrally acting calcium channel blockers. RCVS is much more common than that currently appreciated. APLA is the common cause of RCVS. Further studies are needed to determine the optimal methods to diagnose RCVS and optimal therapies to treat it.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB (2007) Narrative review: reversible cerebral vasoconstriction syndromes. Ann Intern Med 146(1):34–44

    Article  PubMed  Google Scholar 

  2. Chen SP, Fuh JL, Wang SJ (2011) Reversible cerebral vasoconstriction syndrome: current and future perspectives. Expert Rev Neurother 11(9):1265–1276. doi:10.1586/ern.11.112

    Article  PubMed  Google Scholar 

  3. Ducros A, Boukobza M, Porcher R, Sarov M, Valade D, Bousser MG (2007) The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain: J Neurol 130(Pt 12):3091–3101. doi:10.1093/brain/awm256

    Article  Google Scholar 

  4. Garcin B, Clouston J, Saines N (2009) Reversible cerebral vasoconstriction syndrome. J Clin Neurosci 16(1):147–150. doi:10.1016/j.jocn.2008.03.014

    Article  PubMed  Google Scholar 

  5. Hajj-Ali RA, Calabrese LH (2009) Central nervous system vasculitis. Curr Opin Rheumatol 21(1):10–18. doi:10.1097/BOR.0b013e32831cf5e6

    Article  PubMed  Google Scholar 

  6. Singhal AB, Hajj-Ali RA, Topcuoglu MA, Fok J, Bena J, Yang DS, Calabrese LH (2011) Reversible cerebral vasoconstriction syndromes analysis of 139 cases. Arch Neurol 68(8):1005–1012. doi:10.1001/archneurol.2011.68

    Article  PubMed  Google Scholar 

  7. Chen SP, Fuh JL, Wang SJ, Tsai SJ, Hong CJ, Yang AC (2011) Brain-derived neurotrophic factor gene Val66Met polymorphism modulates reversible cerebral vasoconstriction syndromes. PloS One 6(3):e18024. doi:10.1371/journal.pone.0018024

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  8. Boey ML, Colaco CB, Gharavi AE, Elkon KB, Loizou S, Hughes GR (1983) Thrombosis in systemic lupus erythematosus: striking association with the presence of circulating lupus anticoagulant. Br Med J 287(6398):1021–1023

    Article  CAS  Google Scholar 

  9. Wilson WA, Gharavi AE, Koike T, Lockshin MD, Branch DW, Piette JC, Brey R, Derksen R, Harris EN, Hughes GR, Triplett DA, Khamashta MA (1999) International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. Arthritis Rheum 42(7):1309–1311. doi:10.1002/1529-0131(199907)42:7<1309::AID-ANR1>3.0.CO;2-F

    Article  CAS  PubMed  Google Scholar 

  10. Utz VM, Tang J (2011) Ocular manifestations of the antiphospholipid syndrome. Br J Ophthalmol 95(4):454–459. doi:10.1136/bjo.2010.182857

    Article  PubMed  Google Scholar 

  11. Uthman I, Godeau B, Taher A, Khamashta M (2008) The hematologic manifestations of the antiphospholipid syndrome. Blood Rev 22(4):187–194. doi:10.1016/j.blre.2008.03.005

    Article  PubMed  Google Scholar 

  12. Urbanus RT, Siegerink B, Roest M, Rosendaal FR, de Groot PG, Algra A (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):998–1005. doi:10.1016/s1474-4422(09)70239-x

    Article  CAS  PubMed  Google Scholar 

  13. Tincani A, Balestrieri G, Danieli E, Faden D, Lojacono A, Acaia B, Trespidi L, Ventura D, Meroni PL (2003) Pregnancy complications of the antiphospholipid syndrome. Autoimmunity 36(1):27–32

    Article  CAS  PubMed  Google Scholar 

  14. Tektonidou MG, Malagari K, Vlachoyiannopoulos PG, Kelekis DA, Moutsopoulos HM (2003) Asymptomatic avascular necrosis in patients with primary antiphospholipid syndrome in the absence of corticosteroid use—a prospective study by magnetic resonance imaging. Arthritis Rheum 48(3):732–736

    Article  PubMed  Google Scholar 

  15. Sinico RA, Cavazzana I, Nuzzo M, Vianelli M, Napodano P, Scaini P, Tincani A (2010) Renal involvement in primary antiphospholipid syndrome: retrospective analysis of 160 patients. Clin J Am Soc Nephrol 5(7):1211–1217. doi:10.2215/cjn.00460110

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  16. Palomo I, Segovia F, Ortega C, Pierangeli S (2009) Antiphospholipid syndrome: a comprehensive review of a complex and multisystemic disease. Clin Exp Rheumatol 27(4):668–677

    CAS  PubMed  Google Scholar 

  17. Miesbach W (2008) Neurologic symptoms as a feature of the antiphospholipid syndrome. Semin Thromb Hemost 34(3):286–289. doi:10.1055/s-0028-1082273

    Article  CAS  PubMed  Google Scholar 

  18. Levine JS, Branch DW, Rauch J (2002) Medical progress: the antiphospholipid syndrome. N Engl J Med 346(10):752–763

    Article  CAS  PubMed  Google Scholar 

  19. Cervera R, Piette JC, Font J, Khamashta MA, Shoenfeld Y, Camps MT, Jacobsen S, Lakos G, Tincani A, Kontopoulou-Griva I, Galeazzi M, Meroni PL, Derksen RH, de Groot PG, Gromnica-Ihle E, Baleva M, Mosca M, Bombardieri S, Houssiau F, Gris JC, Quere I, Hachulla E, Vasconcelos C, Roch B, Fernandez-Nebro A, Boffa MC, Hughes GR, Ingelmo M (2002) Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum 46(4):1019–1027

    Article  PubMed  Google Scholar 

  20. Hughes GRV (2008) Hughes syndrome (the antiphospholipid syndrome) ten clinical lessons. Autoimmun Rev 7(3):262–266

    Article  PubMed  Google Scholar 

  21. Mayer M, Cerovec M, Rados M, Cikes N (2010) Antiphospholipid syndrome and central nervous system. Clin Neurol Neurosurg 112(7):602–608. doi:10.1016/j.clineuro.2010.03.023

    Article  PubMed  Google Scholar 

  22. Meroni PL (2008) Pathogenesis of the antiphospholipid syndrome: an additional example of the mosaic of autoimmunity. J Autoimmun 30(1–2):99–103

    Article  CAS  PubMed  Google Scholar 

  23. Girardi G, Redecha P, Salmon JE (2004) Heparin prevents antiphospholipid antibody-induced fetal loss by inhibiting complement activation. Nat Med 10(11):1222–1226

    Article  CAS  PubMed  Google Scholar 

  24. Lim W (2011) Complement and the antiphospholipid syndrome. Curr Opin Hematol 18(5):361–365. doi:10.1097/MOH.0b013e3283497f3e

    Article  CAS  PubMed  Google Scholar 

  25. Atsumi T, Khamashta MA, Haworth RS, Brooks G, Amengual O, Ichikawa K, Koike T, Hughes GR (1998) Arterial disease and thrombosis in the antiphospholipid syndrome: a pathogenic role for endothelin 1. Arthritis Rheum 41(5):800–807. doi:10.1002/1529-0131(199805)41:5<800::AID-ART5>3.0.CO;2-J

    Article  CAS  PubMed  Google Scholar 

  26. Flammer J, Pache M, Resink T (2001) Vasospasm, its role in the pathogenesis of diseases with particular reference to the eye. Prog Retin Eye Res 20(3):319–349

    Article  CAS  PubMed  Google Scholar 

  27. Bakst R, Merola JF, Franks AG Jr, Sanchez M (2008) Raynaud's phenomenon: pathogenesis and management. J Am Acad Dermatol 59(4):633–653. doi:10.1016/j.jaad.2008.06.004

    Article  PubMed  Google Scholar 

  28. Rovaris M, Viti B, Ciboddo G, Gerevini S, Capra R, Iannucci G, Comi G, Filippi M (2000) Brain involvement in systemic immune mediated diseases: magnetic resonance and magnetisation transfer imaging study. J Neurol Neurosurg Psychiatry 68(2):170–177

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  29. Stosic M, Ambrus J, Garg N, Weinstock-Guttman B, Ramanathan M, Kalman B, Minagar A, Munschauer FE, Galey TM, Hussein S, Bakshi R, Zivadinov R (2010) MRI characteristics of patients with antiphospholipid syndrome and multiple sclerosis. J Neurol 257(1):63–71. doi:10.1007/s00415-009-5264-6

    Article  PubMed  Google Scholar 

  30. Mandell DM, Matouk CC, Farb RI, Krings T, Agid R, Terbrugge K, Willinsky RA, Swartz RH, Silver FL, Mikulis DJ (2011) Vessel wall MRI to differentiate between reversible cerebral vasoconstriction syndrome and central nervous system vasculitis: preliminary results. Stroke J Cereb Circ. doi:10.1161/STROKEAHA.111.626184

    Google Scholar 

  31. Chen SP, Fuh JL, Lirng JF, Chang FC, Wang SJ (2006) Recurrent primary thunderclap headache and benign CNS angiopathy: spectra of the same disorder? Neurology 67(12):2164–2169. doi:10.1212/01.wnl.0000249115.63436.6d

    Article  PubMed  Google Scholar 

  32. Feng L, Fitzsimmons BF, Young WL, Berman MF, Lin E, Aagaard BDL, Duong H, PileSpellman J (2002) Intraarterially administered verapamil as adjunct therapy for cerebral vasospasm: safety and 2-year experience. Am J Neuroradiol 23(8):1284–1290

    PubMed  Google Scholar 

Download references

Disclosures

None.

Author information

Author notes

  1. Sarthak Gupta

    Present address: National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA

Authors and Affiliations

  1. School of Medicine and Biomedical Sciences, State University of New York at Buffalo, Buffalo, NY, USA

    Sarthak Gupta, Robert Zivadinov, Deepa Ramasamy & Julian L. Ambrus Jr.

  2. Department of Medicine, State University of New York at Buffalo, Buffalo, NY, USA

    Sarthak Gupta & Julian L. Ambrus Jr.

  3. Department of Neurology, State University of New York at Buffalo, Buffalo, NY, USA

    Robert Zivadinov & Deepa Ramasamy

  4. Buffalo General Hospital, Room C281, 100 High Street, Buffalo, NY, 14203, USA

    Julian L. Ambrus Jr.

Authors
  1. Sarthak Gupta
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Robert Zivadinov
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Deepa Ramasamy
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Julian L. Ambrus Jr.
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Julian L. Ambrus Jr..

Additional information

All authors participated in the writing of this manuscript.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Gupta, S., Zivadinov, R., Ramasamy, D. et al. Reversible cerebral vasoconstriction syndrome (RCVS) in antiphospholipid antibody syndrome (APLA): the role of centrally acting vasodilators. Case series and review of literature. Clin Rheumatol 33, 1829–1833 (2014). https://doi.org/10.1007/s10067-013-2434-9

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-013-2434-9

Keywords

Search

Navigation