Abstract
Primary Sjögren’s syndrome (pSS) is a chronic autoimmune disorder associated with a variety of systemic presentations. Varied neurological dysfunctions of newly diagnosed adult patients with pSS have been observed in recent years. We aimed to describe a rare case of acute bilateral facial paralysis diagnosed with pSS for the first time and review the previous studies including similar cases. A 69-year-old female, who had experienced ocular and oral dryness for more than 10 years, presented with bilateral facial palsy. Her laboratory test results showed positive anti-Ro (SSA) and anti-Ro-52 antibodies. Ophthalmic examination and test of saliva secretion verified xerophthalmia and xerostomia, respectively. Other possibilities of Lyme disease, Möbius syndrome, tumor, bilateral temporal bone fracture, Guillain–Barré syndrome, central nervous system lymphoma and HIV infection were ruled out. A diagnosis of pSS associated with bilateral facial paralysis was made. The literature review revealed one article describing a similar patient. Our case was the only one suffering from acute bilateral facial palsy without other nerve involvement. The presence of such patients reveals that pSS is an underlying cause of acute bilateral facial paralysis.
Similar content being viewed by others
References
Bowman SJ (2018) Primary Sjogren’s syndrome. Lupus 27(1_suppl):32–35. https://doi.org/10.1177/0961203318801673
Vallat JM, Rabin M, Magy L (2012) Peripheral neuropathies in rheumatic disease—a guide to diagnosis. Nat Rev Rheumatol 8(10):599–609. https://doi.org/10.1038/nrrheum.2012.138
Margaretten M (2017) Neurologic Manifestations of Primary Sjogren Syndrome. Rheum Dis Clin North Am 43(4):519–529. https://doi.org/10.1016/j.rdc.2017.06.002
Reda H, Chin RL (2014) Peripheral neuropathies of rheumatologic disease and gluten-related disorders. Semin Neurol 34(4):413–424. https://doi.org/10.1055/s-0034-1390390
Mori K, Iijima M, Koike H, Hattori N, Tanaka F, Watanabe H, Katsuno M, Fujita A, Aiba I, Ogata A, Saito T, Asakura K, Yoshida M, Hirayama M, Sobue G (2005) The wide spectrum of clinical manifestations in Sjogren’s syndrome-associated neuropathy. Brain J Neurol 128(Pt 11):2518–2534. https://doi.org/10.1093/brain/awh605
Gilchrist JM (2009) Seventh cranial neuropathy. Semin Neurol 29(1):5–13. https://doi.org/10.1055/s-0028-1124018
Mavrikakis I (2008) Facial nerve palsy: anatomy, etiology, evaluation, and management. Orbit 27(6):466–474. https://doi.org/10.1080/01676830802352543
Hadithi M, Stam F, Donker AJ, Dijkmans BA (2001) Sjogren’s syndrome: an unusual cause of Bell’s palsy. Ann Rheum Dis 60(7):724–725
Birnbaum J (2015) Facial Weakness, otalgia, and hemifacial spasm: a novel neurological syndrome in a case-series of 3 patients with rheumatic disease. Medicine 94(40):e1445. https://doi.org/10.1097/MD.0000000000001445
Ashraf VV, Bhasi R, Kumar RP, Girija AS (2009) Primary Sjogren’s syndrome manifesting as multiple cranial neuropathies: mri findings. Ann Indian Acad Neurol 12(2):124–126. https://doi.org/10.4103/0972-2327.53083
Colaci M, Cassone G, Manfredi A, Sebastiani M, Giuggioli D, Ferri C (2014) Neurologic complications associated with Sjogren’s disease: case reports and modern pathogenic dilemma. Case Rep Neurol Med. https://doi.org/10.1155/2014/590292
Uchihara T, Yoshida S, Tsukagoshi H (1989) Bilateral facial paresis with Sjogren’s syndrome. J Neurol 236(3):186
Gasparyan AY, Ayvazyan L, Blackmore H, Kitas GD (2011) Writing a narrative biomedical review: considerations for authors, peer reviewers, and editors. Rheumatol Int 31(11):1409–1417. https://doi.org/10.1007/s00296-011-1999-3
Teixeira F, Moreira I, Silva AM, Vasconcelos C, Farinha F, Santos E (2013) Neurological involvement in primary Sjogren Syndrome. Acta Reumatol Port 38(1):29–36
Christensen LB, Petersen PE, Thorn JJ, Schiodt M (2001) Dental caries and dental health behavior of patients with primary Sjogren syndrome. Acta Odontol Scand 59(3):116–120
Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, Rasmussen A, Scofield H, Vitali C, Bowman SJ, Mariette X (2017) 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjogren’s syndrome: a consensus and data-driven methodology involving three international patient cohorts. Ann Rheum Dis 76(1):9–16. https://doi.org/10.1136/annrheumdis-2016-210571
Gaudin RA, Jowett N, Banks CA, Knox CJ, Hadlock TA (2016) Bilateral facial paralysis: a 13-year experience. Plast Reconstr Surg 138(4):879–887. https://doi.org/10.1097/PRS.0000000000002599
Mellgren SI, Goransson LG, Omdal R (2007) Primary Sjogren’s syndrome associated neuropathy. Can J Neurol Sci Le journal canadien des sciences neurologiques 34(3):280–287
Gemignani F, Marbini A, Pavesi G, Di Vittorio S, Manganelli P, Cenacchi G, Mancia D (1994) Peripheral neuropathy associated with primary Sjogren’s syndrome. J Neurol Neurosurg Psychiatry 57(8):983–986
Mellgren SI, Conn DL, Stevens JC, Dyck PJ (1989) Peripheral neuropathy in primary Sjogren’s syndrome. Neurology 39(3):390–394
Berkowitz AL, Samuels MA (2014) The neurology of Sjogren’s syndrome and the rheumatology of peripheral neuropathy and myelitis. Pract Neurol 14(1):14–22. https://doi.org/10.1136/practneurol-2013-000651
Delalande S, de Seze J, Fauchais AL, Hachulla E, Stojkovic T, Ferriby D, Dubucquoi S, Pruvo JP, Vermersch P, Hatron PY (2004) Neurologic manifestations in primary Sjogren syndrome: a study of 82 patients. Medicine 83(5):280–291
Alexander EL, Malinow K, Lejewski JE, Jerdan MS, Provost TT, Alexander GE (1986) Primary Sjogren’s syndrome with central nervous system disease mimicking multiple sclerosis. Ann Intern Med 104(3):323–330
Alhomoud IA, Bohlega SA, Alkawi MZ, Alsemari AM, Omer SM, Alsenani FM (2009) Primary Sjogren’s syndrome with central nervous system involvement. Saudi Med J 30(8):1067–1072
Holland NJ, Bernstein JM (2014) Bell’s palsy. BMJ Clin Evid 2014:1–19
Balachandran C, McCluskey PJ, Champion GD, Halmagyi GM (2002) Methotrexate-induced optic neuropathy. Clin Exp Ophthalmol 30(6):440–441
Park JE, Lee KM, Choi HY, Ahn SE, You MW (2019) Methotrexate-associated primary hepatic lymphoma and cranial neuropathy in a patient with rheumatoid arthritis: a case report with clinical follow-up over a 7-year period. Medicine 98(17):e14997. https://doi.org/10.1097/MD.0000000000014997
Funding
None.
Author information
Authors and Affiliations
Contributions
ZW: writing of manuscript including editing and revision and the literature review at all stages of its production, and clinical management of patient. SJ: writing of manuscript including editing and revision and the literature review at all stages of its production, and clinical management of patient. GJ: manuscript editing and revision at all stages of production, final approval of manuscript, and clinical management of patient.
Corresponding author
Ethics declarations
Conflict of interest
All the authors declare no conflict of interest.
Ethical approval
All the procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.
Informed consent
Written informed consent was obtained from this patient prior to submission of this article for consideration as a case-based review.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Wei, Z., Jiaying, S. & Junhong, G. Bilateral facial paralysis as a rare neurological manifestation of primary Sjögren’s syndrome: case-based review. Rheumatol Int 39, 1651–1654 (2019). https://doi.org/10.1007/s00296-019-04339-z
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00296-019-04339-z