The trusted source for
healthcare information and
Neurology of Sjögren's Syndrome
Abstract & Commentary
By Michael Rubin, MD, Professor of Clinical Neurology, Weill Cornell Medical College. Dr. Rubin reports no financial relationships relevant to this field of study.
Synopsis: Peripheral and central nervous system involvement is common in Sjögren's syndrome, and may mimic multiple sclerosis or neuromyelitis optica.
Source: Gono T, et al. Clinical manifestations of neurological involvement in primary Sjögren's syndrome. Clin Rheumatol DOI 10.1007/s10067-010-1458-7.
Initially described by Swedish ophthalmologist Henrik Sjögren (1899-1986), and his wife, also an ophthalmologist, Sjögren's syndrome (SS) is a rare autoimmune disease, with a prevalence of 0.09 to 3.5%, characterized by dry eyes and dry mouth, xeropthalmia, and xerostomia, due to chronic lymphocytic and plasma cellular infiltration of exocrine glands. It exists as a primary disorder, but may co-exist as a secondary disorder in association with other autoimmune conditions such as systemic lupus erythematosus (SLE), dermatomyositis, scleroderma, or rheumatoid arthritis (RA). Primary SS (pSS) may be associated with peripheral or central nervous system involvement, and this retrospective study evaluated pSS patients to determine the variety of these complications in pSS.
Between August 1, 1992 and October 31, 2008, 32 patients with pSS were admitted to Tokyo Women's Medical University, Institute of Rheumatology. Diagnosis of pSS was based on the revised European criteria of the American-European Consensus Group. Patients with other collagen vascular diseases, including RA, SLE, or scleroderma, were excluded. All patient's charts were reviewed for findings on neurologic examination, magnetic resonance imaging, electroencephalography, and spinal fluid analysis. Fisher's exact test provided statistical analysis and P<0.05 was considered significant.
Among the 32 pSS patients, 20 demonstrated neurological involvement. Mean age at diagnosis was 44.2 years for those with, and 46.2 years for those without, neurologic findings, and females predominated in both groups, 95% and 100%, respectively. Neurologic involvement preceded pSS diagnosis in 25%. Other than the presence of fever in those with neurological involvement, no other factor differentiated the groups, including skin rash, lymphadenopathy, antibody positivity (ANA > 160, anti-SS-A, anti-SS-B), or serum immunoglobulin G (IgG) values. Peripheral nervous system (PNS) findings encompassed cranial neuropathy in 41% (n = 7), including optic neuritis (n =3), trigeminal neuralgia (n =2), and one each with facial neuropathy and combined glossopharyngeal and vagus neuropathy. Peripheral neuropathy was seen in 53% (n = 9), purely sensory in eight, with one patient demonstrating sensorimotor neuropathy. Mononeuritis multiplex was seen in 18% (n =3). Central nervous system (CNS) involvement included encephalopathy (n = 3), aseptic meningitis (n = 2), and subclinical white matter changes in the brain and spinal cord (n = 1). Neuromyelitis optica, associated with pSS, was diagnosed in one optic neuritis patient, based on anti-aquaporin 4-antibody positivity. Both CNS and PNS findings occur in pSS but their etiology remains uncertain.
Peripheral nervous system involvement occurs in approximately 20% of patients with Sjögren's Syndrome. CNS disease is less common. Among 424 pSS patients, diagnosed by the revised European criteria of the American-European Consensus Group, CNS involvement was detected in 25 (5.8%), including 24 females and 1 male, a mean of 7 years following initial diagnosis (Rheumatology doi:10.1093/rheumatology/keq111). Diffuse CNS disease was seen in 10 patients (40%), usually manifested by recurrent subacute encephalopathy (n = 6), characterized by impaired concentration, attention, and cognition, memory loss, visual disturbances, and dizziness. Focal or multifocal disease was seen in 9 patients (36%), and encompassed focal motor deficits, aphasia, seizures, parkinsonism, and cerebellar ataxia. Multiple-sclerosis-like disease was seen in five patients (20%) and isolated optic neuritis, seen in a single patient (4%), rounded out the most common CNS presentations. Whereas articular manifestations were more common in pSS patients without neurological complications, significant risk factors for CNS disease included pulmonary involvement, decreased complement (C4) level, and longer pSS duration, with lung disease emerging as the strongest association.