Primary Sjögren’s syndrome (pSS)-related cerebellar ataxia: a systematic review and meta-analysis

Liampas, Andreas; Nteveros, Antonios; Parperis, Konstantinos; Akil, Mohammed; Dardiotis, Efthymios; Andreadou, Elizabeth; Hadjivassiliou, Marios; Zis, Panagiotis

doi:10.1007/s13760-021-01784-1

Cited by 7 publications

(5 citation statements)

References 36 publications

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“…This cerebellar syndrome is typically characterized by prominent gait dysfunction, with a smaller number of patients experiencing dysarthria. 36 Although this syndrome mimics that of a degenerative ataxia, it is important to note that it may be responsive to immunotherapy. 37,38 In addition to the inflammatory CNS manifestations of Sjögren syndrome, it has also been noted that many patients with Sjögren syndrome have cognitive dysfunction.…”

Section: Key Pointsmentioning

confidence: 99%

“…Also described is Sjögren syndrome–related cerebellar ataxia not caused by underlying vasculitis or demyelinating disease. This cerebellar syndrome is typically characterized by prominent gait dysfunction, with a smaller number of patients experiencing dysarthria 36 . Although this syndrome mimics that of a degenerative ataxia, it is important to note that it may be responsive to immunotherapy 37,38 …”

Section: Neurologic Manifestations Of Select Systemic Autoimmune Dise...mentioning

confidence: 99%

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Neurologic Manifestations of Rheumatologic Disorders

McCombe

2024

CONTINUUM: Lifelong Learning in Neurology

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OBJECTIVE This article provides an overview of the neurologic manifestations of sarcoidosis and select rheumatologic disorders. An approach to the assessment and differential diagnosis of characteristic clinical presentations, including meningitis and vasculitis, is also reviewed. A review of treatment options is included as well as discussion of distinct areas of overlap, including rheumatologic disease in the setting of neuromyelitis spectrum disorder and demyelinating disease in the setting of tumor necrosis factor-α inhibitors. LATEST DEVELOPMENTS An increased understanding of the immune mechanisms involved in sarcoidosis and rheumatologic diseases has resulted in a greater diversity of therapeutic options for their treatment. Evidence directing the treatment of the central nervous system (CNS) manifestations of these same diseases is lacking, with a paucity of controlled trials. ESSENTIAL POINTS It is important to have a basic knowledge of the common CNS manifestations of rheumatologic diseases and sarcoidosis so that they can be recognized when encountered. In the context of many systemic inflammatory diseases, including systemic lupus erythematosus, IgG4-related disease, and sarcoidosis, CNS disease may be a presenting feature or occur without systemic manifestations of the disease, making familiarity with these diseases even more important.

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Section: Key Pointsmentioning

confidence: 99%

Section: Neurologic Manifestations Of Select Systemic Autoimmune Dise...mentioning

confidence: 99%

Neurologic Manifestations of Rheumatologic Disorders

McCombe

2024

CONTINUUM: Lifelong Learning in Neurology

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“…Recognition of alternative causes of subacute ataxia is vital as many are reversible if identified early in course. Important diseases on the differential include autoimmune processes related to thyroid disease, diabetes, gluten intolerance (celiac disease), Sjogren's syndrome, toxic/metabolic syndromes such as vitamin deficiencies (B and E) or metronidazole toxicity, infectious processes such as varicella cerebellitis, and prionopathies [32][33][34][35][36][37]. Depending on presentation, a thorough evaluation of the aforementioned entities should be considered in patients with subacute ataxia.…”

Section: Rapidly Progressive Cerebellar Syndromementioning

confidence: 99%

Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment

et al. 2023

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Paraneoplastic neurological syndromes (PNS) include any symptomatic and non-metastatic neurological manifestations associated with a neoplasm. PNS associated with antibodies against intracellular antigens, known as “high-risk” antibodies, show frequent association with underlying cancer. PNS associated with antibodies against neural surface antigens, known as “intermediate- or low-risk” antibodies, are less frequently associated with cancer. In this narrative review, we will focus on PNS of the central nervous system (CNS). Clinicians should have a high index of suspicion with acute/subacute encephalopathies to achieve a prompt diagnosis and treatment. PNS of the CNS exhibit a range of overlapping “high-risk” clinical syndromes, including but not limited to latent and overt rapidly progressive cerebellar syndrome, opsoclonus-myoclonus-ataxia syndrome, paraneoplastic (and limbic) encephalitis/encephalomyelitis, and stiff-person spectrum disorders. Some of these phenotypes may also arise from recent anti-cancer treatments, namely immune-checkpoint inhibitors and CAR T-cell therapies, as a consequence of boosting of the immune system against cancer cells. Here, we highlight the clinical features of PNS of the CNS, their associated tumors and antibodies, and the diagnostic and therapeutic strategies. The potential and the advance of this review consists on a broad description on how the field of PNS of the CNS is constantly expanding with newly discovered antibodies and syndromes. Standardized diagnostic criteria and disease biomarkers are fundamental to quickly recognize PNS to allow prompt treatment initiation, thus improving the long-term outcome of these conditions.

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“…In a small series of patients with SS presenting with sensory ataxia, cerebellar signs were found in nearly 40%, most of whom showed cerebellar atrophy on neuroimaging studies (48). Therefore, the diagnostic approach to a patient with cerebellar ataxia of unknown etiology should rule out an underlying SS, mainly by testing for anti-Ro antibodies and, if necessary, with a salivary gland biopsy (49). A recent study has identified a novel autoantibody against Purkinje cells in a patient with primary autoimmune cerebellar ataxia associated with SS (50).…”

Section: Pearl Test For Anti-ro Antibodies In Patients Presenting Wit...mentioning

confidence: 99%

Practical diagnostic tips for the Sjögren Clinic: pearls, myths and mistakes

Brito-Zerón

Retamozo

Flores-Chávez³

et al. 2022

Clinical and Experimental Rheumatology

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More than 90 years have passed since Hendrik Sjögren began to consider that behind the dryness that several of his patients presented, there could be a systemic disease potentially linked to abnormal immune responses. For many years, the disease was mostly considered a minor syndrome compared with other systemic autoimmune diseases such as systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and vasculitis, and advances in its understanding were slow and little recognised. The irruption of new technologies at the end of the 20th century rapidly promoted the development of international projects with a wide impact and diffusion. In the last 20 years, a significant improvement has been achieved in epidemiological determinants, pathogenic mechanisms, diagnostic accuracy, and a standardised therapeutic approach for patients with Sjögren's syndrome (SS). These developments have provided the tools for an early diagnosis and personalised management for most patients. However, a significant number of early myths and ongoing controversies are still making the appropriate management of SS difficult in daily clinical practice. This review provides a selection of pearls, myths, and mistakes that may serve as practical diagnostic tips for the Sjögren Clinic in four specific scenarios: defining the appropriate epidemiological background, enabling the earliest diagnostic suspicion as possible, improving the systemic characterisation of the disease, and designing an optimal follow-up of patients.

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Primary Sjögren’s syndrome (pSS)-related cerebellar ataxia: a systematic review and meta-analysis

Cited by 7 publications

References 36 publications

Neurologic Manifestations of Rheumatologic Disorders

Neurologic Manifestations of Rheumatologic Disorders

Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment

Practical diagnostic tips for the Sjögren Clinic: pearls, myths and mistakes

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