Characterisation of ataxia in Sjogren’s syndrome

Jaques, Cristina Saade; Moraes, Marianna Pinheiro Moraes de; Silva, Eduardo Antonio Roquim; Coimbra-Neto, Antônio R.; Martinez, Alberto Rolim Muro; Camargos, Sarah Teixeira; Cardoso, Francisco; França, Marcondes C.; Nucci, Anamarli; Pedroso, José Luiz; Barsottini, Orlando Graziani Póvoas

doi:10.1136/jnnp-2019-322373

Cited by 8 publications

(4 citation statements)

References 6 publications

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“…In a study with 14 SS patients, Jacques et al 27 observed that most ataxia cases were of sensory type. However, cerebellar atrophy and cerebellar ataxia may also be observed in SS, mimicking a neurodegenerative disease.…”

Section: Central Nervous System Manifestationsmentioning

confidence: 97%

Sjogren's syndrome: a neurological perspective

Barsottini,

Moraes,

Fraiman

et al. 2023

Arq Neuropsiquiatr

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Sjogren's syndrome (SS) is a complex autoimmune disease characterized by lymphocytic infiltration of salivary and lacrimal glands, resulting in sicca symptoms. Additionally, SS presents with neurological manifestations that significantly impact the nervous system. This review aims to provide a comprehensive overview of the neurological aspects of SSj, covering both the peripheral and central nervous system involvement, while emphasizing diagnosis, treatment, and prognosis.

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Section: Central Nervous System Manifestationsmentioning

confidence: 97%

Sjogren's syndrome: a neurological perspective

Barsottini,

Moraes,

Fraiman

et al. 2023

Arq Neuropsiquiatr

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“…50 A recent study showed that greater superior and middle cerebellar peduncle volume is associated with better motor and cognitive function, further highlighting the importance of the cerebellar status on overall functioning in MS. 51 Ataxia associated with primary Sjogren syndrome (pSS) presents as a sensory ataxia due to a sensory neuronopathy affecting large sensory fibers. 52 Cerebellar ataxia in pSS, although rare, can occasionally occur-both acute and chronic cerebellar ataxia have been reported. [53][54][55] Therefore, we still recommend testing the antinuclear antibody with reflex testing, together with tests of other autoimmune disorders such as gluten ataxia and Hashimoto encephalopathy (details addressed below), as part of the initial workup for cerebellar ataxia.…”

Section: Immune-mediated Ataxiamentioning

confidence: 99%

Ataxias: Hereditary, Acquired, and Reversible Etiologies

Lin

Kuo

2023

Semin Neurol

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A variety of etiologies can cause cerebellar dysfunction, leading to ataxia symptoms. Therefore, the accurate diagnosis of the cause for cerebellar ataxia can be challenging. A step-wise investigation will reveal underlying causes, including nutritional, toxin, immune-mediated, genetic, and degenerative disorders. Recent advances in genetics have identified new genes for both autosomal dominant and autosomal recessive ataxias, and new therapies are on the horizon for targeting specific biological pathways. New diagnostic criteria for degenerative ataxias have been proposed, specifically for multiple system atrophy, which will have a broad impact on the future clinical research in ataxia. In this article, we aim to provide a review focus on symptoms, laboratory testing, neuroimaging, and genetic testing for the diagnosis of cerebellar ataxia causes, with a special emphasis on recent advances. Strategies for the management of cerebellar ataxia is also discussed.

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“…In these patients, typical cerebellar ataxia manifests with vermian dysfunction, namely gait ataxia and/or cerebellar speech. 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).…”

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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Characterisation of ataxia in Sjogren’s syndrome

Cited by 8 publications

References 6 publications

Sjogren's syndrome: a neurological perspective

Sjogren's syndrome: a neurological perspective

Ataxias: Hereditary, Acquired, and Reversible Etiologies

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

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