ALS in association or component of the clinical picture of sjögren's syndrome: a case report
DOI:
https://doi.org/10.11606/issn.1679-9836.v103i2e-219326Keywords:
Sjogren's Syndrome, Motor Neurons, Amyotrophic Lateral Sclerosis, Case Reports, ElectromyographyAbstract
Introduction: Sjögren's Syndrome (SS) is an immune-mediated systemic inflammatory disease related to the involvement of the exocrine glands. However, its heterogeneity also allows extraglandular involvement, and changes of neurological origin may occur, such as motor neuron involvement, which mimics the symptoms of Amyotrophic Lateral Sclerosis (ALS). Objectives: Describe and discuss a case of a patient with SS with neurological impairment, indicating systemic impairment with possible association with Aminotrophic Lateral Sclerosis. Methodology: A retrospective study of medical records and exams was carried out, after approval by the Research Ethics Committee. A systematic literature search was carried out in the databases PUBMED/MEDLINE, LILACS and Cochrane Central using the search strategy “(Sjogren's syndrome) AND (motor neuron disease)” and “(amyotrophic lateral sclerosis) AND (Sjogren's syndrome) Sjogren)” with “full text” filter. Case Report and Discussion: Patient reported loss of strength in the right hand for a year, xerophthalmia and xerostomia, and presented positive Anti-SSB/La and ANA with positive glandular biopsy for SS. Initial nerve conduction examination showed a decreased action potential amplitude and increased F waves in the right ulnar nerve. It evolved with impairment of the lower limbs, without significant complaints, just a progressive loss of strength. In the literature consulted, we found that only 11 articles with a clinical profile compatible with isolated motor impairment in SS and only 2 other cases showed the possibility of an association between ALS and the syndrome, describing very compatible conditions, but with an unfavorable evolution. Conclusion: The patient presents a rare condition, with few reports in the literature, which, given the symptomatic progression, considered ALS as a component of the clinical picture or associated pathology. The scarcity of information raises doubts about the most appropriate diagnosis and treatment. The patient remains unresponsive to the treatments used and the condition has been evolving progressively.
Downloads
References
Carsons SE, Patel BC. Sjogren Syndrome. StatPearls Publishing. 2021. https://www.ncbi.nlm.nih.gov/books/NBK431049/
Parisis D, Chivasso C, Perret J, Soyfoo MS, Delporte C. Current State of Knowledge on Primary Sjögren's Syndrome, an Autoimmune Exocrinopathy. J Clin Med. 2020;9(7):2299-360. Doi: 10.3390/jcm9072299.
van Ginkel MS, Glaudemans AWJM, van der Vegt B, Mossel E, Kroese FGM, Bootsma H, et al. Imaging in Primary Sjögren’s Syndrome. J Clin Med. 2020;9(8):2492. Doi: 10.3390/jcm9082492.
Delalande S, de Seze J, Fauchais AL, Hachulla E, Stojkovic T, Ferriby D, et al. Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine (Baltimore). 2004; 83(5): 280-91. Doi: 10.1097/01.md.0000141099.53742.16.
Fauchais AL, Magy L, Vidal E. Central and peripheral neurological complications of primary Sjögren's syndrome. Presse Med, 2012;41(9): e485-e493. Doi: 10.1016/j.lpm.2012.06.002.
Pavlakis PP, Alexopoulos H, Kosmidis ML, Stamboulis E, Routsias JG, Tzartos SJ, et al. Peripheral neuropathies in Sjogren syndrome: a new reappraisal. J Neurol Neurosurg Psychiatry 2011; 82: 798–802. Doi: 10.1136/jnnp.2010.222109.
Colaci M, Cassone G, Manfredi A, Sebastiani M, Giuggioli D, Ferri C. Neurologic complications associated with Sjögren's disease: case reports and modern pathogenic dilemma. Case Rep Neurol Med, 2014;2014:590292-303. Doi: 10.1155/2014/590292.
Li JA, Meng HM, Cui ZT, Wang X, Miao J. Recurrent cerebral infarctions in primary Sjogren syndrome: a case report and literature review. Front Neurol, 2018;9:865. Doi: 10.3389/fneur.2018.00865.
Yang H, Jing X, Yan J, Ma D. Sjögren's syndrome with rapidly progressive motor neuron disease: a case report. J Int Med Res. 2020;48(11): 300060520974465. Doi: 10.1177/0300060520974465.
Alencar MA, Silva IMM, Hilário SM, Rangel MFA, Abdo JS, Araújo CM, et al. Quality of life, disability, and clinical variables in amyotrophic lateral sclerosis. Arq Neuropsiquiatr. 2022;80(3): 255-61. Doi: 10.1590/0004-282X-ANP-2021-0201.
Wendebourg MJ, Kuhle J, Hardmeier M. Case Report: A 72-Year-Old Woman With Progressive Motor Weakness, Dry Eyes and High Levels of Serum Neurofilament Light Chain. Front Neurol. 2022;13:889894. Doi:10.3389/fneur.2022.889894.
Wijesekera LC, Leigh PN. Amyotrophic lateral sclerosis. Orphanet Journal of Rare Diseases, 2009;4(1):3. Doi: 10.1186/1750-1172-4-3.
Ozgocmen S, Gur A. Treatment of central nervous system involvement associated with primary Sjögren's syndrome. Curr Pharm Des. 2008;14(13):1270-3. Doi: 10.2174/138161208799316366.
Attout H, Rahmeh F, Ziegler F. Syndrome de Gougerot-Sjögren simulant une sclérose latérale amyotrophique. Rev Med Interne. 2000; 21(8):708-10. Doi: 10.1016/s0248-8663(00)80030-2.
Yamamoto A, Imai K, Hamanaka M, Yamada T, Yamazaki H, Tsuto K, et al. A case of motor dominant neuropathy and focal segmental glomerulosclerosis associated with Sjögren's syndrome. Rinsho Shinkeigaku. 2015; 55(10):732-6. Doi: 10.5692/clinicalneurol.cn-000739.
Hagiwara K, Murai H, Ochi H, Osoegawa M, Shigeto H, Ohyagi Y, et al. Upper motor neuron syndrome associated with subclinical Sjögren's syndrome. Intern Med. 2008; 47(11):1047-51. Doi: 10.2169/internalmedicine.47.0846.
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.
Suk-Won A, Byung-Nam Y. Motor dominant polyradiculopathy with Primary Sjögren’s syndrome mimicking motor neuron disease. Annals of Clinical Neurophysiology. 2019; 21(1):61-5. Doi: 10.14253/acn.2019.21.1.61.
Published
Issue
Section
License
Copyright (c) 2024 Vanessa Furtado do Vale Bento, Fernanda Aparecida Mateus Vieira, Maria Fernanda Reis Nunes da Silva, Virginia Fernandes Moça Trevisani
This work is licensed under a Creative Commons Attribution-ShareAlike 4.0 International License.
