Peripheral and central nervous system involvement in a patient with primary Sjögren’s syndrome: a case report
Abstract Background Primary Sjögren’s syndrome is the second most common rheumatological disorder after rheumatoid arthritis. It typically presents as xerophthalmia and xerostomia in postmenopausal women. Involvement of the central nervous system has been recognized, although its pathogenesis and ch...
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| Format: | Article |
| Language: | English |
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BMC
2019-05-01
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| Series: | Journal of Medical Case Reports |
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| Online Access: | http://link.springer.com/article/10.1186/s13256-019-2086-8 |
| _version_ | 1818243334310723584 |
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| author | Kathi Ging Marie-Luise Mono Mathias Sturzenegger Martin Zbinden Sabine Adler Vera Genitsch Franca Wagner |
| author_facet | Kathi Ging Marie-Luise Mono Mathias Sturzenegger Martin Zbinden Sabine Adler Vera Genitsch Franca Wagner |
| author_sort | Kathi Ging |
| collection | DOAJ |
| description | Abstract Background Primary Sjögren’s syndrome is the second most common rheumatological disorder after rheumatoid arthritis. It typically presents as xerophthalmia and xerostomia in postmenopausal women. Involvement of the central nervous system has been recognized, although its pathogenesis and characteristics are poorly understood. Central nervous system complications are a diagnostic challenge and emphasize the need for systematic screening of patients with new peripheral and central neurological symptoms. Case report We report a case of a 58-year-old Swiss woman presenting with rapidly progressive sensorimotor distal polyneuropathy together with new-onset generalized seizures. Initial magnetic resonance imaging (MRI) of the brain performed after the first seizure showed multiple, bihemispheric, confluent white matter hyperintensities with contrast enhancement. Follow-up imaging 3 days after the initial magnetic resonance imaging demonstrated a fulminant disease progression associated with the serious clinical deterioration of the patient. In light of the results of a minor salivary gland biopsy, autoantibody testing, nerve conduction studies, and cranial magnetic resonance imaging, primary Sjögren’s syndrome with cryoglobulinemia type II was diagnosed. Response to plasmapheresis and subsequent administration of cyclophosphamide was favorable. Conclusion Even though exocrinopathy is the hallmark of Sjögren’s syndrome, systemic symptoms are observed in one-third of patients. There is an urgent need to better characterize the mechanisms underlying different disease phenotypes and to perform randomized controlled trials in order to provide tailored and evidence-based treatment for primary Sjögren’s syndrome. |
| first_indexed | 2024-12-12T13:59:28Z |
| format | Article |
| id | doaj.art-2a4f8a95dba242419cd219013e25bb71 |
| institution | Directory Open Access Journal |
| issn | 1752-1947 |
| language | English |
| last_indexed | 2024-12-12T13:59:28Z |
| publishDate | 2019-05-01 |
| publisher | BMC |
| record_format | Article |
| series | Journal of Medical Case Reports |
| spelling | doaj.art-2a4f8a95dba242419cd219013e25bb712022-12-22T00:22:23ZengBMCJournal of Medical Case Reports1752-19472019-05-011311710.1186/s13256-019-2086-8Peripheral and central nervous system involvement in a patient with primary Sjögren’s syndrome: a case reportKathi Ging0Marie-Luise Mono1Mathias Sturzenegger2Martin Zbinden3Sabine Adler4Vera Genitsch5Franca Wagner6Institute of Anatomy, University of ZurichDepartment of Neurology, Stadtspital TriemliDepartment of Neurology, Inselspital, Bern University HospitalUniversity Institute of Diagnostic and Interventional Neuroradiology, Inselspital, Bern University HospitalDepartment of Rheumatology, Inselspital, Bern University HospitalDepartment of Pathology, Inselspital, Bern University HospitalUniversity Institute of Diagnostic and Interventional Neuroradiology, Inselspital, Bern University HospitalAbstract Background Primary Sjögren’s syndrome is the second most common rheumatological disorder after rheumatoid arthritis. It typically presents as xerophthalmia and xerostomia in postmenopausal women. Involvement of the central nervous system has been recognized, although its pathogenesis and characteristics are poorly understood. Central nervous system complications are a diagnostic challenge and emphasize the need for systematic screening of patients with new peripheral and central neurological symptoms. Case report We report a case of a 58-year-old Swiss woman presenting with rapidly progressive sensorimotor distal polyneuropathy together with new-onset generalized seizures. Initial magnetic resonance imaging (MRI) of the brain performed after the first seizure showed multiple, bihemispheric, confluent white matter hyperintensities with contrast enhancement. Follow-up imaging 3 days after the initial magnetic resonance imaging demonstrated a fulminant disease progression associated with the serious clinical deterioration of the patient. In light of the results of a minor salivary gland biopsy, autoantibody testing, nerve conduction studies, and cranial magnetic resonance imaging, primary Sjögren’s syndrome with cryoglobulinemia type II was diagnosed. Response to plasmapheresis and subsequent administration of cyclophosphamide was favorable. Conclusion Even though exocrinopathy is the hallmark of Sjögren’s syndrome, systemic symptoms are observed in one-third of patients. There is an urgent need to better characterize the mechanisms underlying different disease phenotypes and to perform randomized controlled trials in order to provide tailored and evidence-based treatment for primary Sjögren’s syndrome.http://link.springer.com/article/10.1186/s13256-019-2086-8Sjögren’s syndromeCentral nervous systemPolyneuropathyMagnetic resonance imaging |
| spellingShingle | Kathi Ging Marie-Luise Mono Mathias Sturzenegger Martin Zbinden Sabine Adler Vera Genitsch Franca Wagner Peripheral and central nervous system involvement in a patient with primary Sjögren’s syndrome: a case report Journal of Medical Case Reports Sjögren’s syndrome Central nervous system Polyneuropathy Magnetic resonance imaging |
| title | Peripheral and central nervous system involvement in a patient with primary Sjögren’s syndrome: a case report |
| title_full | Peripheral and central nervous system involvement in a patient with primary Sjögren’s syndrome: a case report |
| title_fullStr | Peripheral and central nervous system involvement in a patient with primary Sjögren’s syndrome: a case report |
| title_full_unstemmed | Peripheral and central nervous system involvement in a patient with primary Sjögren’s syndrome: a case report |
| title_short | Peripheral and central nervous system involvement in a patient with primary Sjögren’s syndrome: a case report |
| title_sort | peripheral and central nervous system involvement in a patient with primary sjogren s syndrome a case report |
| topic | Sjögren’s syndrome Central nervous system Polyneuropathy Magnetic resonance imaging |
| url | http://link.springer.com/article/10.1186/s13256-019-2086-8 |
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