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Sjögren’s Syndrome pp 281–291Cite as

Central Nervous System Involvement

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Abstract

Neurologic signs and symptoms have been recognized in the setting of Sjögren’s syndrome (SS) since the syndrome was first described by Sjögren in 1935 [1]. In particular, peripheral nervous system involvement in primary SS (pSS) has been well characterized and is believed to affect approximately 10–20% of SS patients [2]. The frequency of central nervous system (CNS) involvement is less clear, however, as controversy exists regarding the frequency and type of CNS manifestations in pSS.

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References

  1. Delalande S, de Seze J, Fauchais AL, et al. Neurologic manifestations in primary Sjogren’s syndrome: a study of 82 patients. Medicine (Baltimore). 2004;83:280–91.

    Article  Google Scholar 

  2. Govoni M, Padovan M, Rizzo N, Trotta F. CNS involvement in primary Sjogren’s syndrome: prevalence, clinical aspects, diagnostic assessment and therapeutic approach. CNS Drugs. 2001;15:597–607.

    Article  PubMed  CAS  Google Scholar 

  3. Morgen K, McFarland HF, Pillemer SR. Central nervous system disease in primary Sjogren’s syndrome: the role of magnetic resonance imaging. Semin Arthritis Rheum. 2004;34:623–30.

    Article  PubMed  Google Scholar 

  4. Ozgocmen S, Gur A. Treatment of central nervous system involvement associated with primary Sjogren’s syndrome. Curr Pharm Des. 2008;14:1270–3.

    PubMed  CAS  Google Scholar 

  5. Soliotis FC, Mavragani CP, Moutsopoulos HM. Central nervous system involvement in Sjogren’s syndrome. Ann Rheum Dis. 2004;63:616–20.

    Article  PubMed  CAS  Google Scholar 

  6. Lafitte C, Amoura Z, Cacoub P, et al. Neurological complications of primary Sjogren’s syndrome. J Neurol. 2001;248:577–84.

    Article  PubMed  CAS  Google Scholar 

  7. Belin C, Moroni C, Caillat-Vigneron N, et al. Central nervous system involvement in Sjogren’s syndrome: evidence from neuropsychological testing and HMPAO-SPECT. Ann Med Interne (Paris). 1999;150:598–604.

    CAS  Google Scholar 

  8. Escudero D, Latorre P, Codina M, et al. Central nervous system disease in Sjogren’s syndrome. Ann Med Interne (Parris). 1995;146:239–42.

    CAS  Google Scholar 

  9. Mauch E, Volk C, Kratzsch G, et al. Neurological and neuropsychiatric dysfunction in primary Sjogren’s syndrome. Acta Neurol Scand. 1994;89:31–5.

    Article  PubMed  CAS  Google Scholar 

  10. Hietaharju A, Jannti V, Korpela M, Frey H. Nervous system involvement in systemic lupus erythematosus, Sjogren syndrome’s and scleroderma. Acta Neurol Scand. 1993;88:299–308.

    Article  PubMed  CAS  Google Scholar 

  11. Andonopoulos AP, Lagos G, Drosos AA, Moutsopoulos HM. The spectrum of neurological involvement in Sjogren’s syndrome. Br J Rheumatol. 1990;29:21–3.

    Article  PubMed  CAS  Google Scholar 

  12. Alexander EL, Beall SS, Gordon B, Selnes OA, Yannakakis GD, Patronas N, et al. Magnetic resonance imaging of cerebral lesions in patients with the Sjogren syndrome. Ann Intern Med. 1988;108:815–23.

    PubMed  CAS  Google Scholar 

  13. Coates T, Slavotinek JP, Rischmueller M, Schultz D, Anderson C, Dellamelva M, et al. Cerebral white matter lesions in primary Sjogren’s syndrome: a controlled study. J Rheumatol. 1999;26:1301–5.

    PubMed  CAS  Google Scholar 

  14. Pierot L, Sauve C, Leger JM, Martin N, Koeger AC, Wechsler B, et al. Asymptomatic cerebral involvement in Sjogren’s syndrome: MRI findings of 15 cases. Neuroradiology. 1993;35:378–80.

    Article  PubMed  CAS  Google Scholar 

  15. Alexander EL, Ranzenbach MR, Kumar AJ, Kozachuk WE, Rosenbaum AE, Patronas N, et al. Anti-Ro(SS-A) autoantibodies in central nervous system disease associated with Sjogren’s syndrome (CNS-SS): clinical, neuroimaging, and angiographic correlates. Neurology. 1994;44:899–908.

    PubMed  CAS  Google Scholar 

  16. Michel L, Toulgoat F, Desal H, et al. Atypical neurologic complications in patients with ­primary Sjogren’s syndrome: report of 4 cases. Semin Arthritis Rheum. 2011;40(4):338–42.

    Article  PubMed  Google Scholar 

  17. Min JH, Kim SH, Park MS, Kim BJ, Lee KH. Brain MRI lesions characteristic of neuromyelitis optica and positive anti-aquaporin 4-antibody may predict longitudinal extensive myelitis and optic neuritis in Sjogren’s syndrome. Mult Scler. 2010;16(6):762–4.

    Article  PubMed  Google Scholar 

  18. Alexander EL. Neurologic disease in Sjogren’s syndrome: mononuclear inflammatory vasculopathy affecting central/peripheral nervous system and muscle. A clinical review and update of immunopathogenesis. Rheum Dis Clin North Am. 1993;19(4):869–908.

    PubMed  CAS  Google Scholar 

  19. Mataro M, Escudero D, Ariza M, et al. Magnetic resonance abnormalities associated with cognitive dysfunction in primary Sjogren syndrome. J Neurol. 2003;250(9):1070–6.

    Article  PubMed  Google Scholar 

  20. Morgen KE. Central nervous system disease in primary Sjögren’s syndrome. In: Binder M, Hirokawa N, Windhorst U, editors. Encyclopedia of neuroscience, vol. 3. New York: Springer-Verlag Berlin Heidelberg; 2009. p. 632.

    Google Scholar 

  21. Awad IA, Spetzler RF, Hodak JA, Awad CA, Carey R. Incidental subcortical lesions identified on magnetic resonance imaging in the elderly. I. Correlation with age and cerebrovascular risk factors. Stroke. 1986;17(6):1084–9.

    Article  PubMed  CAS  Google Scholar 

  22. Le Guern V, Belin C, Henegar C, et al. Cognitive function and 99mTc-ECD brain SPECT are significantly correlated in patients with primary Sjogren syndrome: a case-control study. Ann Rheum Dis. 2010;69(1):132–7.

    Article  PubMed  Google Scholar 

  23. Alexander EL, Craft C, Dorsch C, Moser RL, Provost TT, Alexander GE. Necrotizing arteritis and spinal subarachnoid hemorrhage in Sjogren syndrome. Ann Neurol. 1982;11(6):632–5.

    Article  PubMed  CAS  Google Scholar 

  24. Alexander MS, Dias PS, Uttley D. Spontaneous subarachnoid hemorrhage and negative ­cerebral panangiography. Review of 140 cases. J Neurosurg. 1986;64(4):537–42.

    Article  PubMed  CAS  Google Scholar 

  25. Marignier R, Giaudon P, Vukusic S, Confavreux C, Honnorat J. Anti-aquaporin-4 antibodies in Devic’s neuromyelitis optica: therapeutic implications. Ther Adv Neurol Disord. 2010;3:311–21.

    Article  PubMed  CAS  Google Scholar 

  26. Malinow KL, Molina R, Gordon B, Selnes OA, Provost TT, Alexander EL. Neuropsychiatric dysfunction in primary Sjogren’s syndrome. Ann Intern Med. 1985;103(3):344–50.

    PubMed  CAS  Google Scholar 

  27. McFarland HF, Stone LA, Calabresi PA, Maloni H, Bash CN, Frank JA. MRI studies of multiple sclerosis: implications for the natural history of the disease and for monitoring effectiveness of experimental therapies. Mult Scler. 1996;2:198–205.

    PubMed  CAS  Google Scholar 

  28. Simon JH, Jacobs LD, Campion M, Wende K, Simonian N, Cookfair DL, et al. Magnetic resonance studies of intramuscular interferon beta-1a for relapsing multiple sclerosis. The Multiple Sclerosis Collaborative Research Group. Ann Neurol. 1998;43:79–87.

    Article  PubMed  CAS  Google Scholar 

  29. Nomura K, Yamano S, Ikeda Y, Yamada H, Fujimoto T, Minami S, et al. Asymptomatic cerebrovascular lesions detected by magnetic resonance imaging in patients with systemic lupus erythematosus lacking a history of neuropsychiatric events. Intern Med. 1999;38:785–95.

    Article  PubMed  CAS  Google Scholar 

  30. Luks TL, Goodkin DE, Nelson SJ, Majumdar S, Bacchetti P, Portnoy D, et al. A longitudinal study of ventricular volume in early relapsing-remitting multiple sclerosis. Mult Scler. 2000;6:332–7.

    PubMed  CAS  Google Scholar 

  31. de Leeuw FE, de Groot JC, Achten E, Oudkerk M, Ramos LM, Heijboer R, et al. Prevalence of cerebral white matter lesions in elderly people: a population based magnetic resonance imaging study. The Rotterdam Scan Study. J Neurol Neurosurg Psychiatry. 2001;70:9–14.

    Article  PubMed  Google Scholar 

  32. Cortese I, MacFarland HF. Multiple sclerosis. In: Rich RR, Fleisher TA, Shearer WT, Schroeder Jr HW, Frew AJ, Weyand CM, editors. Clinical immunology: principles and practice. 3rd ed. Mosby: Elsevier; 2008.

    Google Scholar 

  33. Van de Merve JP, Yamada T, Sakamoto Y. Systemic aspects of interstitial cystitis, immunology and linkage with autoimmune disorders. Int J Urol. 2003;10(Suppl):S35–8.

    Google Scholar 

  34. Achiron A, Polliack M, Rao SM, Barak Y, Lavie M, Appelboim N, et al. Cognitive patterns and progression in multiple sclerosis: construction and validation of percentile curves. J Neurol Neurosurg Psychiatry. 2005;76:744–9.

    Article  PubMed  CAS  Google Scholar 

  35. Segal B, Carpenter A, Walk D. Involvement of nervous system pathways in primary Sjogren’s syndrome. Rheum Dis Clin North Am. 2008;34:885–906.

    Article  PubMed  Google Scholar 

  36. Polman CH, Reingold SC, Edan G, Filippi M, Hartung HP, Kappos L, et al. Diagnostic criteria for multiple sclerosis: 2005 revisions to the “McDonald Criteria”. Ann Neurol. 2005;58(6):840–6.

    Article  PubMed  Google Scholar 

  37. Napoli SQ, Bakshi R. Magnetic resonance imaging in multiple sclerosis. Rev Neurol Dis. 2005;2(3):109–16; Summer.

    PubMed  Google Scholar 

  38. Wingerchuk MD. Neuromyelitis optica (Devic’s syndrome). Accessed 2006. http://www.myelitis.org/rnds2006/Wingerchuk_NMO_Rare_Neuroimm_062406_final.pdf

  39. Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4:295–306.

    Article  PubMed  CAS  Google Scholar 

  40. Cervera R, Piette J-C, Font J, Mhamashta MA, Shoenfeld Y, Camps MT, et al. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum. 2002;46:1019–27.

    Article  PubMed  Google Scholar 

  41. Cuadrado MJ, Khamashta MA, Ballesteros A, Godfrey T, Simon MJ, Hughes GRV. Can neurologic manifestations of Hughes (antiphospholipid) syndrome be distinguished from multiple sclerosis? Analysis of 27 patients and review of the literature. Medicine (Baltimore). 2000;79:57–68.

    Article  CAS  Google Scholar 

  42. Molloy ES, Calabrese LH. Progressive multifocal leukoencephalopathy. Arthritis Rheum. 2009;60:3761–5.

    Article  PubMed  Google Scholar 

  43. Weiffenbach JM, Fox PC. Odor identification ability among patients with Sjogren’s syndrome. Arthritis Rheum. 1993;36:1752–4.

    Article  PubMed  CAS  Google Scholar 

  44. Boki KA, Ioannidis JP, Segas JV, Maragkoudakis PV, Petrou D, Adamopoulos GK, et al. How significant is sensorineural hearing loss in primary Sjögren’s syndrome? An individually matched case-control study. J Rheumatol. 2001;28(4):798–801.

    PubMed  CAS  Google Scholar 

  45. Tumiati B, Casoli P, Permeggiani A. Hearing loss in the Sjogren syndrome. Ann Intern Med. 1997;126:450–3.

    PubMed  CAS  Google Scholar 

  46. Irani D. Aseptic meningitis and viral myelitis. Neurol Clin. 2008;26:635–55.

    Article  PubMed  Google Scholar 

  47. Schmalstieg WF, Weishenker BG. Approach to acute or subacute myelopathy. Neurol Clin Pract. 2010;75:S2–8.

    Google Scholar 

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Author information

Authors and Affiliations

  1. American Biopharma Corporation, Gaithersburg, MD, USA

    Stanley R. Pillemer

  2. School of Health Sciences, Walden University, Minneapolis, MN, USA

    Aaron B. Mendelsohn

  3. Department of Psychiatry, Central Institute of Mental Health (CIMH), Mannheim, Germany

    Katrin E. Morgen

Authors
  1. Stanley R. Pillemer
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  2. Aaron B. Mendelsohn
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  3. Katrin E. Morgen
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Editor information

Editors and Affiliations

  1. Barcelona, Surgey, Hospital Clinic, Calle Villarroel, 170, Barcelona, 08036, Spain

    Manuel Ramos-Casals

  2. Massachusetts General Hospital, Boston, 02114, Massachusetts, USA

    John H. Stone

  3. National University of Athens, Athens, Greece

    Haralampos M. Moutsopoulos

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Pillemer, S.R., Mendelsohn, A.B., Morgen, K.E. (2011). Central Nervous System Involvement. In: Ramos-Casals, M., Stone, J., Moutsopoulos, H. (eds) Sjögren’s Syndrome. Springer, London. https://doi.org/10.1007/978-0-85729-947-5_20

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  • DOI: https://doi.org/10.1007/978-0-85729-947-5_20

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  • Online ISBN: 978-0-85729-947-5

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