Cerebral Large Vessels Vasculitis Following Guillain-Barré Syndrome As First Clinical Manifestations of Primary Sjogren's Syndrome: A Case Based - Review

Overview

Journal Heliyon

Specialty Social Sciences

Date 2024 May 3

PMID 38698975

Authors

Zakaria Saied

Rania Zouari

Amine Rachdi

Fatma Nabli

Dina Ben Mohamed

Samia Ben Sassi

Affiliations

Soon will be listed here.

Abstract

Background: Primary Sjogren's syndrome (pSS) is an autoimmune exocrinopathy in which extraglandular signs of pSS are determinant for the prognosis. Involvement of both peripheral and central nervous system (CNS) are known to be among the sites of high systemic activity in pSS.

Case Presentation: We, herein, report a case of a 57-year-old female patient with pSS presenting with typical (GBS), shortly followed by acute headaches accompanied by cortical blindness. Cerebral magnetic resonance imaging (MRI) demonstrated T2 signal abnormalities on the occipital region with narrowing and irregularities of the cerebral arteries, suggestive of CNS vasculitis.Subtle sicca symptoms occurring prior to neurological symptoms by 8 months together with immunological disturbances (anti-SSA, anti-SSB antibodies positivity, type II cryoglobulins positivity, and C4 hypocomplementemia) allowed us to retain the diagnosis of pSS. Recovery of motor symptoms was possible under the combined use of immunoglobulins and corticotherapy during the initial phase. A three-years follow-up confirmed progressive motor recovery and stabilization under 6-months cyclophosphamide cycles relayed by azathioprine therapy.

Conclusions: Neurological complications can be inaugural in lead to urgent investigations and treatment. Peripheral and central neurological manifestations can coexist. The approach should integrate careful clinical assessment, as well as radiological and immunological findings.

References

Unnikrishnan G, Hiremath N, Chandrasekharan K, Sreedharan S, Sylaja P . Cerebral Large-Vessel Vasculitis in Sjogren's Syndrome: Utility of High-Resolution Magnetic Resonance Vessel Wall Imaging. J Clin Neurol. 2018; 14(4):588-590. PMC: 6172513. DOI: 10.3988/jcn.2018.14.4.588. View

Bejot Y, Osseby G, Salem D, Beynat J, Muller G, Moreau T . Bilateral optic neuropathy revealing Sjögren's syndrome. Rev Neurol (Paris). 2008; 164(12):1044-7. DOI: 10.1016/j.neurol.2008.04.017. View

Delalande S, De Seze J, Fauchais A, Hachulla E, Stojkovic T, Ferriby D . Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine (Baltimore). 2004; 83(5):280-291. DOI: 10.1097/01.md.0000141099.53742.16. View

Flores-Chavez A, Kostov B, Solans R, Fraile G, Maure B, Feijoo-Masso C . Severe, life-threatening phenotype of primary Sjögren's syndrome: clinical characterisation and outcomes in 1580 patients (GEAS-SS Registry). Clin Exp Rheumatol. 2018; 36 Suppl 112(3):121-129. View

Mori K, Iijima M, Koike H, Hattori N, Tanaka F, Watanabe H . The wide spectrum of clinical manifestations in Sjögren's syndrome-associated neuropathy. Brain. 2005; 128(Pt 11):2518-34. DOI: 10.1093/brain/awh605. View

Baldini C, Pepe P, Quartuccio L, Priori R, Bartoloni E, Alunno A . Primary Sjogren's syndrome as a multi-organ disease: impact of the serological profile on the clinical presentation of the disease in a large cohort of Italian patients. Rheumatology (Oxford). 2013; 53(5):839-44. DOI: 10.1093/rheumatology/ket427. View

Zippel C, Beider S, Kramer E, Konen F, Seeliger T, Skripuletz T . Premature stroke and cardiovascular risk in primary Sjögren's syndrome. Front Cardiovasc Med. 2023; 9:1048684. PMC: 9794609. DOI: 10.3389/fcvm.2022.1048684. View

Harley J, Alexander E, Bias W, Fox O, Provost T, Reichlin M . Anti-Ro (SS-A) and anti-La (SS-B) in patients with Sjögren's syndrome. Arthritis Rheum. 1986; 29(2):196-206. DOI: 10.1002/art.1780290207. View

Argyropoulou O, Tzioufas A . Common and rare forms of vasculitis associated with Sjögren's syndrome. Curr Opin Rheumatol. 2019; 32(1):21-28. DOI: 10.1097/BOR.0000000000000668. View

10.

Goransson L, Herigstad A, Tjensvoll A, Harboe E, Mellgren S, Omdal R . Peripheral neuropathy in primary sjogren syndrome: a population-based study. Arch Neurol. 2006; 63(11):1612-5. DOI: 10.1001/archneur.63.11.1612. View

11.

Massara A, Bonazza S, Castellino G, Caniatti L, Trotta F, Borrelli M . Central nervous system involvement in Sjögren's syndrome: unusual, but not unremarkable--clinical, serological characteristics and outcomes in a large cohort of Italian patients. Rheumatology (Oxford). 2010; 49(8):1540-9. DOI: 10.1093/rheumatology/keq111. View

12.

Shiboski C, Shiboski S, Seror R, Criswell L, Labetoulle M, Lietman T . 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren's Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts. Arthritis Rheumatol. 2016; 69(1):35-45. PMC: 5650478. DOI: 10.1002/art.39859. View

13.

Ging K, Mono M, Sturzenegger M, Zbinden M, Adler S, Genitsch V . Peripheral and central nervous system involvement in a patient with primary Sjögren's syndrome: a case report. J Med Case Rep. 2019; 13(1):165. PMC: 6534842. DOI: 10.1186/s13256-019-2086-8. View

14.

Scofield R, Fayyaz A, Kurien B, Koelsch K . Prognostic value of Sjögren's syndrome autoantibodies. J Lab Precis Med. 2020; 3. PMC: 7034933. DOI: 10.21037/jlpm.2018.08.05. View

15.

Carvajal Alegria G, Guellec D, Mariette X, Gottenberg J, Dernis E, Dubost J . Epidemiology of neurological manifestations in Sjögren's syndrome: data from the French ASSESS Cohort. RMD Open. 2016; 2(1):e000179. PMC: 4838763. DOI: 10.1136/rmdopen-2015-000179. View

16.

Jeong H, Suh B, Kim Y, Chung P, Moon H, Yoon W . Posterior reversible encephalopathy syndrome as an initial neurological manifestation of primary Sjögren's syndrome. Clin Auton Res. 2015; 25(4):259-62. DOI: 10.1007/s10286-015-0305-y. View

17.

Awad A, Mathew S, Katirji B . Acute motor axonal neuropathy in association with Sjögren syndrome. Muscle Nerve. 2010; 42(5):828-30. DOI: 10.1002/mus.21830. View

18.

Tobon G, Pers J, Devauchelle-Pensec V, Youinou P . Neurological Disorders in Primary Sjögren's Syndrome. Autoimmune Dis. 2012; 2012:645967. PMC: 3303537. DOI: 10.1155/2012/645967. View

19.

Mochizuki H, Kamakura K, Masaki T, Hirata A, Nakamura R, Motoyoshi K . Motor dominant neuropathy in Sjögren's syndrome: report of two cases. Intern Med. 2002; 41(2):142-6. DOI: 10.2169/internalmedicine.41.142. View

20.

Alexander G, Provost T, STEVENS M, Alexander E . Sjögren syndrome: central nervous system manifestations. Neurology. 1981; 31(11):1391-6. DOI: 10.1212/wnl.31.11.1391. View