Generic placeholder image

Current Rheumatology Reviews

Editor-in-Chief

ISSN (Print): 1573-3971
ISSN (Online): 1875-6360

Case Report

Neurological Involvement as the Initial Manifestation in Primary Sjögren's Syndrome - A Case Report

Author(s): Iván Posso-Osorio, Juan Naranjo-Escobar, Diana Marcela Loaiza, Marcela Polo, Alex Echeverri and Gabriel J. Tobón*

Volume 15, Issue 3, 2019

Page: [254 - 258] Pages: 5

DOI: 10.2174/1573397114666180731101142

Price: $65

Abstract

Background: Sjögren's syndrome is an autoimmune disease characterised by exocrinopathy mainly involving the salivary and lacrimal glands. In addition, it is a multisystemic condition (i.e., affecting multiple organs and systems). Neurological involvement has been reported in ~20% of cases, with peripheral manifestations being the most frequent.

Methods: We analysed four cases in which neurological manifestations were the first symptoms of Sjögren's syndrome.

Results: In all four cases, neurological symptoms preceded sicca symptoms. In addition, immunosuppressive treatment with steroids and, in some cases, cyclophosphamide showed improvement.

Conclusion: Neurological involvement in Sjögren's syndrome is common and often occurs as the first clinical manifestation. Since evidence is limited, more studies are required in order to determine appropriate diagnostic methods and treatments for each manifestation of Sjögren's syndrome.

Keywords: Neurological involvement, primary Sjögren's syndrome, autoimmune disease, sicca symptoms, exocrinopathy, peripheral neuropathy.

« Previous
[1]
Tobón GJ, Pers J-O, Devauchelle-Pensec V, Youinou P. Neurological Disorders in Primary Sjögren’s Syndrome. Autoimmune Dis 2012.2012645967
[http://dx.doi.org/10.1155/2012/645967] [PMID: 22474573]
[2]
Ramos-Casals M, Tzioufas AG, Font J. Primary Sjögren’s syndrome: new clinical and therapeutic concepts. Ann Rheum Dis 2005; 64(3): 347-54.
[http://dx.doi.org/10.1136/ard.2004.025676] [PMID: 15498797]
[3]
Carvajal Alegria G, Guellec D, Mariette X, et al. Epidemiology of neurological manifestations in Sjögren’s syndrome: data from the French ASSESS Cohort. RMD Open 2016; 2(1)e000179
[http://dx.doi.org/10.1136/rmdopen-2015-000179] [PMID: 27110384]
[4]
Delalande S, de Seze J, Fauchais A-L, et al. Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine (Baltimore) 2004; 83(5): 280-91.
[http://dx.doi.org/10.1097/01.md.0000141099.53742.16] [PMID: 15342972]
[5]
Bakchine S, Duyckaerts C, Hassine L, et al. [Central and peripheral neurologic lesions in primary Gougerot-Sjögren syndrome. Clinicopathological study of a case] Rev Neurol (Paris) 1991; 147(5): 368-75.
[PMID: 1649487]
[6]
Alexander EL, Ranzenbach MR, Kumar AJ, et al. Anti-Ro(SS-A) autoantibodies in central nervous system disease associated with Sjögren’s syndrome (CNS-SS): clinical, neuroimaging, and angiographic correlates. Neurology 1994; 44(5): 899-908.
[http://dx.doi.org/10.1212/WNL.44.5.899] [PMID: 8190294]
[7]
Molina R, Provost TT, Alexander EL. Peripheral inflammatory vascular disease in Sjögren’s syndrome. Association with nervous system complications. Arthritis Rheum 1985; 28(12): 1341-7.
[http://dx.doi.org/10.1002/art.1780281205] [PMID: 3002396]
[8]
Sène D, Jallouli M, Lefaucheur J-P, et al. Peripheral neuropathies associated with primary Sjögren syndrome: immunologic profiles of nonataxic sensory neuropathy and sensorimotor neuropathy. Medicine (Baltimore) 2011; 90(2): 133-8.
[http://dx.doi.org/10.1097/MD.0b013e31820fd2d1] [PMID: 21358442]
[9]
Mellgren SI, Conn DL, Stevens JC, Dyck PJ. Peripheral neuropathy in primary Sjögren’s syndrome. Neurology 1989; 39(3): 390-4.
[http://dx.doi.org/10.1212/WNL.39.3.390] [PMID: 2538774]
[10]
Soliotis FC, Mavragani CP, Moutsopoulos HM. Central nervous system involvement in Sjogren’s syndrome. Ann Rheum Dis 2004; 63(6): 616-20.
[http://dx.doi.org/10.1136/ard.2003.019497] [PMID: 15140765]
[11]
Lafitte C. Neuroradiological manifestations of primary Sjögren’s syndrome. Rev Neurol (Paris) 2002; 158(10 Pt 1): 959-65.
[PMID: 12407304]
[12]
Barendregt PJ, van den Bent MJ, van Raaij-van den Aarssen VJ, et al. Involvement of the peripheral nervous system in primary Sjögren’s syndrome. Ann Rheum Dis 2001; 60(9): 876-81.
[PMID: 11502615]
[13]
Griffin JW, Cornblath DR, Alexander E, et al. Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjögren’s syndrome. Ann Neurol 1990; 27(3): 304-15.
[http://dx.doi.org/10.1002/ana.410270313] [PMID: 2327738]
[14]
Murata Y, Maeda K, Kawai H, et al. Antiganglion neuron antibodies correlate with neuropathy in Sjögren’s syndrome. Neuroreport 2005; 16(7): 677-81.
[http://dx.doi.org/10.1097/00001756-200505120-00005] [PMID: 15858405]
[15]
de Seze J, Delalande S, Fauchais A-L, et al. Myelopathies secondary to Sjögren’s syndrome: treatment with monthly intravenous cyclophosphamide associated with corticosteroids. J Rheumatol 2006; 33(4): 709-11.
[PMID: 16583474]
[16]
Kastrup O, Maschke M, Diener HC. Pulse-cyclophosphamide in the treatment of ataxic sensory and cranial nerve neuropathy associated with Sjogren’s syndrome. Clin Neurol Neurosurg 2005; 107(5): 440-1.
[http://dx.doi.org/10.1016/j.clineuro.2004.10.001] [PMID: 16023544]
[17]
Chen WH, Yeh JH, Chiu HC. Plasmapheresis in the treatment of ataxic sensory neuropathy associated with Sjögren’s syndrome. Eur Neurol 2001; 45(4): 270-4.
[http://dx.doi.org/10.1159/000052142] [PMID: 11385268]
[18]
Pereira PR, Viala K, Maisonobe T, et al. Sjögren sensory neuronopathy (sjögren ganglionopathy): Long-term outcome and treatment response in a series of 13 cases.In: Ilik F, Ed. Medicine (Baltimore) 95. 19. 2016

Rights & Permissions Print Cite
© 2024 Bentham Science Publishers | Privacy Policy