CNS Involvement in Primary Sj??gren??s Syndrome

Abstract

Among the systemic manifestations of primary Sjögren's syndrome, neurological involvement is still an intriguing and debated issue. Although peripheral nervous system abnormalities are a well documented occurrence with a reported prevalence ranging from 10 to 20%, opinions differ as to the prevalence of CNS disease, with suggestions from 'nonexistent' to 'very common'. The lack of agreement probably reflects the different populations selected, different inclusion criteria and lack of rigorous epidemiological studies. In our experience, CNS involvement was detected in 7 of 87 (8%) unselected consecutive patients observed over a period of 5 years. The spectrum of CNS involvement is wide, including focal, diffuse, neuropsychiatric and spinal cord symptoms, frequently characterised by insidious onset, remitting course and, sometimes, progressive evolution. The diagnostic approach enabling early recognition of this complication relies on careful clinical assessment using history and physical examination combined with neuropsychological testing and instrumental, laboratory and imaging investigations such as magnetic resonance imaging, single photon emission computed tomography, electrophysiological testing and CSF analysis. The clinical picture often shows spontaneous remission, but when overt neurological symptoms occur or become progressive, therapeutic interventions with high dose corticosteroids and cytotoxic agents, such as intravenous cyclophosphamide pulse therapy, may be indicated.

Primary Sjogren’s Syndrome Associated With Treatment-Resistant Obsessive–Compulsive Disorder

“…Women with pSS rated themselves higher on symptoms of somatization, depression, anxiety, and paranoid ideation than healthy controls (8). Other studies have reported cognitive impairment (9), psychosis (9, 10), mood disorders (11–13), anxiety disorders (12, 13), somatization disorders (12), and dissociative states (12) or a combination of these in pSS patients. Despite this, very few published studies comprehensively phenotype the neuropsychiatric manifestations of pSS, disease course, and response to treatment.…”

Limbic Encephalitis as a Heralding Manifestation of Primary Sjogren’s Syndrome

“…Anti-Ro/SSA and anti-La/SSB antibodies are positive in only 50% of patients. 3 Thus, the neurologic symptoms of SS are present in the absence of oral/ocular dryness. In such cases, the clinicians should have a high index of suspicion for pSS and consider autoantibody testing.…”

“…Among the neurological presentations, the involvement of the peripheral nervous system is seen more commonly with a frequency of around 10 to 20%. 2 3 The central nervous system (CNS) involvement is not well established, with a rate varying from 1.5 to 25%. 4 5 The CNS manifestations can be in a diverse spectrum and may manifest at onset or later during the disease.…”

Progressive cerebellar degeneration revealing Primary Sjögren Syndrome: a case report

“…Movement disorders have been rarely described in association with pSS, and are less classic that in systemic lupus erythematous. There are few reports of chorea, dystonia, athetosis and tremor [ 20 ]. Extrapyramidal syndrome has been more frequently reported [ 21 – 24 ].…”