Neurological Involvement in Primary Sjögren Syndrome: A Focus on Central Nervous System

Morreale, Manuela; Marchione, Pasquale; Giacomini, Patrizia; Pontecorvo, Simona; Marianetti, Massimo; Vento, Claudio; Tinelli, Emanuele; Francia, Ada

doi:10.1371/journal.pone.0084605

Cited by 103 publications

(117 citation statements)

References 33 publications

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“…Neurologic complications occur in nearly 20% of all patients with primary SS (2,3). Although peripheral nervous system (PNS) involvement has been widely described in several previous studies (1)(2)(3)(4)(5), recent studies have implicated the central nervous system (CNS) involvement as a frequent manifestation of SS (6,7). After the introduction of the current diagnostic criteria for the SS in 2002 (8), an increased frequency of CNS involvement has been documented in SS patients (7).…”

Section: Introductionmentioning

confidence: 99%

Limbic Encephalitis Associated with Sjögren's Syndrome: Report of Three Cases

et al. 2016

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Sjögren's syndrome (SS) may be complicated by neurological manifestations. We herein report three women (age range 26-60 years old) who all presented with limbic encephalitis (LE) as the predominant clinical feature 3 months to 15 years after the diagnosis of SS. The 26-year-old patient also developed acute motor axonal neuropathy one week after autoimmune encephalitis. All three patients showed contrast-enhanced MRI lesions and inflammatory cerebrospinal fluid findings, while not displaying any anti-neuronal antibodies and showing a remarkable response to immunotherapy. SS is often overlooked when the symptoms are mild. Therefore, in LE cases with no identifiable cause, serological screening for rheumatologic disorders is recommended.

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Section: Introductionmentioning

confidence: 99%

Limbic Encephalitis Associated with Sjögren's Syndrome: Report of Three Cases

et al. 2016

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“…10 Among them, the most frequent are headache, movement disorders, seizures, myelopathy, aseptic meningitis, and cognitive dysfunction. 4,10,11 The pathological processes causing the CNS involvement include mononuclear lymphocytic infiltration in the meninges or parenchyma, microinfarcts, or microhaemorrhages in brain parenchyma.…”

Section: Discussionmentioning

confidence: 99%

Bilateral Internuclear Ophthalmoplegia as a Presenting Manifestation of Primary Sjögren’s Syndrome

Natsis

Boura

Kyriazis

et al. 2016

Neuro-Ophthalmology

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Bilateral internuclear ophthalmoplegia has been linked with various pathological conditions of the central nervous system (CNS), such as multiple sclerosis, stroke, tumours, and brainstem inflammatory processes. Herein the authors report a case of a 45-year-old female patient who presented with diplopia due to bilateral internuclear ophthalmoplegia, with no evidence of brainstem lesion in brain magnetic resonance imaging (MRI) and was diagnosed with primary Sjögren's syndrome. ARTICLE HISTORY

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“…It may appear in different forms: sensorymotor polyneuropathy, pure sensory neuropathy (peculiar to pSS), neuronopathy (related to lymphocytic infiltration of the dorsal root ganglia), autonomic neuropathy, cranial nerve involvement (mainly the cochleovestibular nerve but also the trigeminal and facial nerves), mononeuritis multiplex, chronic polyradiculoneuropathy, motor neuron disease or small fiber neuropathy (diagnosed on skin biopsy). Symmetrical axonal sensorimotor polyneuropathy is the most common type of polyneuropathy and is associated with higher monoclonal B-cell proliferation markers (hypergammaglobulinemia cryoglobulinemia, monoclonal gammopathy, serum free light chain ratio, and B-cell lymphoma) (35 (38). Headache, cognitive disorders and psychiatric symptoms appear to be associated with anti-SSA antibodies and Raynaud's phenomenon, the latter suggesting a possible underlying endothelial dysfunction (autoimmune endothelitis) of the cerebral microcirculation or a potential inflammation-mediated shift of the neurovascular coupling (38).…”

Section: N Peripheral and Central Nervous System Involvementmentioning

confidence: 99%

“…Symmetrical axonal sensorimotor polyneuropathy is the most common type of polyneuropathy and is associated with higher monoclonal B-cell proliferation markers (hypergammaglobulinemia cryoglobulinemia, monoclonal gammopathy, serum free light chain ratio, and B-cell lymphoma) (35 (38). Headache, cognitive disorders and psychiatric symptoms appear to be associated with anti-SSA antibodies and Raynaud's phenomenon, the latter suggesting a possible underlying endothelial dysfunction (autoimmune endothelitis) of the cerebral microcirculation or a potential inflammation-mediated shift of the neurovascular coupling (38). Interestingly, abnormalities in the electrophysiological parameters can also be detected in asymptomatic patients with longer disease duration, salivary gland ultrasonography abnormalities and elevated inflammatory markers in blood tests (39).…”

Section: N Peripheral and Central Nervous System Involvementmentioning

confidence: 99%

The clinical spectrum of primary Sjögren’s syndrome: beyond exocrine glands

et al. 2017

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SUMMARYAlthough primary Sjögren's syndrome (pSS) is a mild indolent chronic disease mainly characterized by mucosal dryness in the majority of cases, a consistent subgroup of patients display extra-glandular manifestations. Virtually any organs and systems can be affected, leading to a more serious disease prognosis. Therefore, the prompt identification of patients at higher risk of extra-glandular manifestations is necessary to start a thorough follow up and an aggressive treatment. The aim of this review article is to provide an overview of epidemiological, clinical and serological features of extra-glandular manifestations in pSS as well as current knowledge about putative biomarkers useful in clinical practice. Key words:Sjögren's syndrome; Extra-glandular manifestations; Cardiovascular risk. Reumatismo, 2017; 69 (3): 93-100 n INTRODUCION P rimary Sjögren's syndrome (pSS) is a complex and heterogeneous chronic autoimmune disease primarily characterized by a focal lymphocytic chronic inflammation of exocrine glands leading to progressive loss of secretory function (1). Therefore, the specific case-history of pSS is dominated by signs and symptoms of mucosal dryness. However, although salivary and lachrymal glands represent the main target of pSS, several other organs and systems may be affected during the disease course in at least one third of patients, resulting in a plethora of systemic clinical manifestations, serological abnormalities and complications (2). The most severe complication of pSS is non-Hodgkin lymphoma occurring in about 5% of patients (3) and dramatically worsening disease prognosis. In this regard, the prompt identification of pSS patients with higher risk of developing extraglandular manifestations is mandatory for correct management. Low C3 and C4 levels, cryoglobulins, monoclonal component, anti-Ro/ SSA, anti-La/SSB, rheumatoid factor and hypergammaglobulinaemia represent poor prognostic serological factors in pSS associated with lymphoma and severe extraglandular features (2). Furthermore, histological features such as the presence of germinal centre-like structures in the minor salivary glands have been associated with higher risk of developing lymphoma (4). The purpose of this review article is to discuss current knowledge of extra-glandular manifestations in pSS not secondary to mucosal dryness, excluding pathogenesis and clinical manifestations of lymphoproliferative disorders. n MUSCULOSKELETAL INVOLVEMENTMusculoskeletal manifestations such as myalgia, arthralgia and morning stiffness are present in as many as 90% of patients, while clinically evident arthritis is found in up to 17%. In a recent study conducted on 17 female patients with pSS and 18 patients with secondary SS (sSS), ultrasonography (US) was used to confirm the non-erosive nature of joint involvement in pSS and to discriminate between pSS and RA-associated SS. Synovitis was commonly detected in pSS, mainly in metacarpophalangeal joints (MCP), wrists, and knees. Bone erosions also may occur. Power Doppler signal ...

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Neurological Involvement in Primary Sjögren Syndrome: A Focus on Central Nervous System

Cited by 103 publications

References 33 publications

Limbic Encephalitis Associated with Sjögren's Syndrome: Report of Three Cases

Limbic Encephalitis Associated with Sjögren's Syndrome: Report of Three Cases

Bilateral Internuclear Ophthalmoplegia as a Presenting Manifestation of Primary Sjögren’s Syndrome

The clinical spectrum of primary Sjögren’s syndrome: beyond exocrine glands

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