Peripheral Neuropathy

  • Pantelis P. Pavlakis
  • Marinos C. Dalakas


The prevalence of peripheral neuropathy among Sjögren’s syndrome patients varies greatly between different published studies, with numbers ranging between 2% and 60% [1–14]. Such a large disparity is due to: (a) the use of different criteria for the diagnosis of Sjögren’s syndrome, and (b) the varying definition of peripheral neuropathy with inconsistent application of objective clinical or electrodiagnostic criteria. An example of the problem is one recent report, based on a large population of Sjögren’s syndrome patients, which estimates the frequency of peripheral neuropathy at 10% [5], without providing details on how the neuropathy was diagnosed.


Dorsal Root Ganglion Peripheral Neuropathy Nerve Biopsy Small Fiber Neuropathy Sensorimotor Polyneuropathy 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Skopouli FN, Dafni U, Ioannidis JP, et al. Clinical evolution, and morbidity and mortality of primary Sjogren’s syndrome. Semin Arthritis Rheum. 2000;29:296–304.PubMedCrossRefGoogle Scholar
  2. 2.
    Harboe E, Tjensvoll AB, Maroni S, et al. Neuropsychiatric syndromes in patients with systemic lupus erythematosus and primary Sjogren syndrome: a comparative population-based study. Ann Rheum Dis. 2009;68:1541–6.PubMedCrossRefGoogle Scholar
  3. 3.
    Goransson LG, Herigstad A, Tjensvoll AB, et al. Peripheral neuropathy in primary Sjogren syndrome: a population-based study. Arch Neurol. 2006;63:1612–5.PubMedCrossRefGoogle Scholar
  4. 4.
    Govoni M, Bajocchi G, Rizzo N, et al. Neurological involvement in primary Sjogren’s ­syndrome: clinical and instrumental evaluation in a cohort of Italian patients. Clin Rheumatol. 1999;18:299–303.PubMedCrossRefGoogle Scholar
  5. 5.
    Ramos-Casals M, Solans R, Rosas J, et al. Primary Sjogren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine (Baltimore). 2008;87:210–9.CrossRefGoogle Scholar
  6. 6.
    Andonopoulos AP, Lagos G, Drosos AA, et al. The spectrum of neurological involvement in Sjogren’s syndrome. Br J Rheumatol. 1990;29:21–3.PubMedCrossRefGoogle Scholar
  7. 7.
    Vrethem M, Lindvall B, Holmgren H, et al. Neuropathy and myopathy in primary Sjogren’s syndrome: neurophysiological, immunological and muscle biopsy results. Acta Neurol Scand. 1990;82:126–31.PubMedCrossRefGoogle Scholar
  8. 8.
    Gemignani F, Marbini A, Pavesi G, et al. Peripheral neuropathy associated with primary Sjogren’s syndrome. J Neurol Neurosurg Psychiatry. 1994;57:983–6.PubMedCrossRefGoogle Scholar
  9. 9.
    Barendregt PJ, van den Bent MJ, van Raaij-van den Aarssen VJ, et al. Involvement of the peripheral nervous system in primary Sjogren’s syndrome. Ann Rheum Dis. 2001;60:876–81.PubMedGoogle Scholar
  10. 10.
    Andonopoulos AP, Lagos G, Drosos AA, et al. Neurologic involvement in primary Sjogren’s syndrome: a preliminary report. J Autoimmun. 1989;2:485–8.PubMedCrossRefGoogle Scholar
  11. 11.
    Lopate G, Pestronk A, Al-Lozi M, et al. Peripheral neuropathy in an outpatient cohort of patients with Sjogren’s syndrome. Muscle Nerve. 2006;33:672–6.PubMedCrossRefGoogle Scholar
  12. 12.
    Lafitte C, Amoura Z, Cacoub P, et al. Neurological complications of primary Sjogren’s ­syndrome. J Neurol. 2001;248:577–84.PubMedCrossRefGoogle Scholar
  13. 13.
    Mauch E, Volk C, Kratzsch G, et al. Neurological and neuropsychiatric dysfunction in primary Sjogren’s syndrome. Acta Neurol Scand. 1994;89:31–5.PubMedCrossRefGoogle Scholar
  14. 14.
    Binder A, Snaith ML, Isenberg D. Sjogren’s syndrome: a study of its neurological complications. Br J Rheumatol. 1988;27:275–80.PubMedCrossRefGoogle Scholar
  15. 15.
    Grant IA, Hunder GG, Homburger HA, et al. Peripheral neuropathy associated with sicca complex. Neurology. 1997;48:855–62.PubMedGoogle Scholar
  16. 16.
    Delalande S, de Seze J, Fauchais AL, et al. Neurologic manifestations in primary Sjogren syndrome: a study of 82 patients. Medicine (Baltimore). 2004;83:280–91.CrossRefGoogle Scholar
  17. 17.
    Mori K, Iijima M, Koike H, et al. The wide spectrum of clinical manifestations in Sjogren’s syndrome-associated neuropathy. Brain. 2005;128:2518–34.PubMedCrossRefGoogle Scholar
  18. 18.
    Pavlakis PP, Alexopoulos H, Kosmidis M, et al. Sjögren’s syndrome associated polyneuropathy: clinical and immunological profiles. Neurology. 2010;74:491–2.Google Scholar
  19. 19.
    Mochizuki H, Kamakura K, Masaki T, et al. Motor dominant neuropathy in Sjogren’s ­syndrome: report of two cases. Intern Med. 2002;41:142–6.PubMedCrossRefGoogle Scholar
  20. 20.
    Ioannidis JP, Vassiliou VA, Moutsopoulos HM. Long-term risk of mortality and lymphoproliferative disease and predictive classification of primary Sjogren’s syndrome. Arthritis Rheum. 2002;46:741–7.PubMedCrossRefGoogle Scholar
  21. 21.
    Dworkin RH, O’Connor AB, Backonja M, et al. Pharmacologic management of neuropathic pain: evidence-based recommendations. Pain. 2007;132:237–51.PubMedCrossRefGoogle Scholar
  22. 22.
    Hughes RA, Donofrio P, Bril V, et al. Intravenous immune globulin (10% caprylate-chromatography purified) for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (ICE study): a randomised placebo-controlled trial. Lancet Neurol. 2008;7:136–44.PubMedCrossRefGoogle Scholar
  23. 23.
    Mellgren SI, Conn DL, Stevens JC, et al. Peripheral neuropathy in primary Sjogren’s ­syndrome. Neurology. 1989;39:390–4.PubMedGoogle Scholar
  24. 24.
    England JD, Gronseth GS, Franklin G, et al. Evaluation of distal symmetric polyneuropathy: the role of autonomic testing, nerve biopsy, and skin biopsy (an evidence-based review). Muscle Nerve. 2009;39:106–15.PubMedCrossRefGoogle Scholar
  25. 25.
    Collins MP, Mendell JR, Periquet MI, et al. Superficial peroneal nerve/peroneus brevis muscle biopsy in vasculitic neuropathy. Neurology. 2000;55:636–43.PubMedGoogle Scholar
  26. 26.
    Terrier B, Lacroix C, Guillevin L, et al. Diagnostic and prognostic relevance of neuromuscular biopsy in primary Sjogren’s syndrome-related neuropathy. Arthritis Rheum. 2007;57:1520–9.PubMedCrossRefGoogle Scholar
  27. 27.
    Schaublin GA, Michet Jr CJ, Dyck PJ, et al. An update on the classification and treatment of vasculitic neuropathy. Lancet Neurol. 2005;4:853–65.PubMedCrossRefGoogle Scholar
  28. 28.
    Jayne D, Rasmussen N, Andrassy K, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med. 2003;349:36–44.PubMedCrossRefGoogle Scholar
  29. 29.
    Pagnoux C, Mahr A, Hamidou MA, et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med. 2008;359:2790–803.PubMedCrossRefGoogle Scholar
  30. 30.
    Stone JH, Merkel PA, Spiera RF, et al. Rituximab compared with cyclophosphamide for remission induction in ANCA-associated vasculitis. N Engl J Med. 2010;15;363:221–32.Google Scholar
  31. 31.
    Voulgarelis M, Giannouli S, Tzioufas AG, et al. Long term remission of Sjogren’s syndrome associated aggressive B cell non-Hodgkin’s lymphomas following combined B cell depletion therapy and CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone). Ann Rheum Dis. 2006;65:1033–7.PubMedCrossRefGoogle Scholar
  32. 32.
    Souayah N, Chong PS, Cros D. Acute sensory neuronopathy as the presenting symptom of Sjogren’s syndrome. J Clin Neurosci. 2006;13:862–5.PubMedCrossRefGoogle Scholar
  33. 33.
    Dalakas MC. Chronic idiopathic ataxic neuropathy. Ann Neurol. 1986;19:545–54.PubMedCrossRefGoogle Scholar
  34. 34.
    Griffin JW, Cornblath DR, Alexander E, et al. Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjogren’s syndrome. Ann Neurol. 1990;27:304–15.PubMedCrossRefGoogle Scholar
  35. 35.
    Sghirlanzoni A, Pareyson D, Lauria G. Sensory neuron diseases. Lancet Neurol. 2005;4:349–61.PubMedCrossRefGoogle Scholar
  36. 36.
    Molinuevo JL, Graus F, Serrano C, et al. Utility of anti-Hu antibodies in the diagnosis of paraneoplastic sensory neuropathy. Ann Neurol. 1998;44:976–80.PubMedCrossRefGoogle Scholar
  37. 37.
    Malinow K, Yannakakis GD, Glusman SM, et al. Subacute sensory neuronopathy secondary to dorsal root ganglionitis in primary Sjogren’s syndrome. Ann Neurol. 1986;20:535–7.PubMedCrossRefGoogle Scholar
  38. 38.
    Takahashi Y, Takata T, Hoshino M, et al. Benefit of IVIG for long-standing ataxic sensory neuronopathy with Sjogren’s syndrome. IV immunoglobulin. Neurology. 2003;60:503–5.PubMedGoogle Scholar
  39. 39.
    Chen WH, Yeh JH, Chiu HC. Plasmapheresis in the treatment of ataxic sensory neuropathy associated with Sjogren’s syndrome. Eur Neurol. 2001;45:270–4.PubMedCrossRefGoogle Scholar
  40. 40.
    Asahina M, Kuwabara S, Nakajima M, et al. d-penicillamine treatment for chronic sensory ataxic neuropathy associated with Sjogren’s syndrome. Neurology. 1998;51:1451–3.PubMedGoogle Scholar
  41. 41.
    Caroyer JM, Manto MU, Steinfeld SD. Severe sensory neuronopathy responsive to infliximab in primary Sjogren’s syndrome. Neurology. 2002;59:1113–4.PubMedGoogle Scholar
  42. 42.
    Yamada S, Mori K, Matsuo K, et al. Interferon alfa treatment for Sjogren’s syndrome associated neuropathy. J Neurol Neurosurg Psychiatry. 2005;76:576–8.PubMedCrossRefGoogle Scholar
  43. 43.
    Gorson KC, Natarajan N, Ropper AH, et al. Rituximab treatment in patients with IVIg-dependent immune polyneuropathy: a prospective pilot trial. Muscle Nerve. 2007;35:66–9.PubMedCrossRefGoogle Scholar
  44. 44.
    Kosmidis ML, Dalakas MC. Practical considerations on the use of rituximab in autoimmune neurological disorders. Ther Adv Neurol Disord. 2010;3:93–105.PubMedCrossRefGoogle Scholar
  45. 45.
    Lacomis D. Small-fiber neuropathy. Muscle Nerve. 2002;26:173–88.PubMedCrossRefGoogle Scholar
  46. 46.
    Mori K, Iijima M, Sugiura M, et al. Sjogren’s syndrome associated painful sensory neuropathy without sensory ataxia. J Neurol Neurosurg Psychiatry. 2003;74:1320–2.PubMedCrossRefGoogle Scholar
  47. 47.
    Chai J, Herrmann DN, Stanton M, et al. Painful small-fiber neuropathy in Sjogren syndrome. Neurology. 2005;65:925–7.PubMedCrossRefGoogle Scholar
  48. 48.
    Gorson KC, Herrmann DN, Thiagarajan R, et al. Non-length dependent small fibre ­neuropathy/ganglionopathy. J Neurol Neurosurg Psychiatry. 2008;79:163–9.PubMedCrossRefGoogle Scholar
  49. 49.
    Hoitsma E, Reulen JP, de Baets M, et al. Small fiber neuropathy: a common and important clinical disorder. J Neurol Sci. 2004;227:119–30.PubMedCrossRefGoogle Scholar
  50. 50.
    Morozumi S, Kawagashira Y, Iijima M, et al. Intravenous immunoglobulin treatment for ­painful sensory neuropathy associated with Sjogren’s syndrome. J Neurol Sci. 2009;279:57–61.PubMedCrossRefGoogle Scholar
  51. 51.
    Kushida CA. Clinical presentation, diagnosis, and quality of life issues in restless legs ­syndrome. Am J Med. 2007;120:S4–12.PubMedCrossRefGoogle Scholar
  52. 52.
    Jankovic J. Treatment of hyperkinetic movement disorders. Lancet Neurol. 2009;8:844–56.PubMedCrossRefGoogle Scholar
  53. 53.
    Gudbjornsson B, Broman JE, Hetta J, et al. Sleep disturbances in patients with primary Sjogren’s syndrome. Br J Rheumatol. 1993;32:1072–6.PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag London Limited 2011

Authors and Affiliations

  • Pantelis P. Pavlakis
    • 1
  • Marinos C. Dalakas
    • 1
  1. 1.Neuroimmunology Unit, Department of Pathophysiology, Medical SchoolUniversity of AthensAthensGreece

Personalised recommendations