ArticleCerebellum & ataxias2017
Cerebellar ataxia with sensory ganglionopathy; does autoimmunity have a role to play?
Article in Cerebellum & ataxias, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers, 2 of them syntheses that pooled it.
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Who cites it
6 citing papers in PubMed, 2 syntheses or guidelines pooled it.
- Primary Sjögren syndrome-related peripheral neuropathy: A systematic review and meta-analysis.European journal of neurology · 2023Pooled it
- Primary Sjögren's syndrome (pSS)-related cerebellar ataxia: a systematic review and meta-analysis.Acta neurologica Belgica · 2022Pooled it
- Can CANVAS due toJournal of neurology, neurosurgery, and psychiatry · 2024Article
- The Intersection Between Cerebellar Ataxia and Neuropathy: a Proposed Classification and a Diagnostic Approach.Cerebellum (London, England) · 2022Review
- Neurological manifestation of coeliac disease with particular emphasis on gluten ataxia and immunological injury: a review article.Gastroenterology and hepatology from bed to bench · 2021Review
- Treatment of Neurological Manifestations of Gluten Sensitivity and Coeliac Disease.Current treatment options in neurology · 2019Review
Corrections and comments
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Authors and funding
6 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
background and purposeCerebellar ataxia with sensory ganglionopathy (SG) is a disabling combination of neurological dysfunction usually seen as part of some hereditary ataxias. However, patients may present with this combination without a genetic cause.
methodsWe reviewed records of all patients that have been referred to the Sheffield Ataxia Centre who had neurophysiological and imaging data suggestive of SG and cerebellar ataxia respectively. We excluded patients with Friedreich's ataxia, a common cause of this combination. All patients were screened for genetic causes and underwent extensive investigations.
resultsWe identified 40 patients (45% males, mean age at symptom onset 53.7 ± 14.7 years) with combined cerebellar ataxia and SG. The majority of patients (40%) were initially diagnosed with cerebellar dysfunction and 30% were initially diagnosed with SG. For 30% the two diagnoses were made at the same time. The mean latency between the two diagnoses was 6.5 ± 8.9 years (range 0-44). The commonest initial manifestation was unsteadiness (77.5%) followed by patchy sensory loss (17.5%) and peripheral neuropathic pain (5%).Nineteen patients (47.5%) had gluten sensitivity, of whom 3 patients (7.5%) had biopsy proven coeliac disease. Other abnormal immunological tests were present in another 15 patients. Six patients had malignancy, which was diagnosed within 5 years of the neurological symptoms. Only 3 patients (7.5%) were classified as having a truly idiopathic combination of cerebellar ataxia with SG.
conclusionOur case series highlights that amongst patients with the unusual combination of cerebellar ataxia and SG, immune pathogenesis plays a significant role.
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