Recognition and management of rapid-onset gluten ataxias: case series

Abstract

Background Most immune-mediated cerebellar ataxias, including those associated with gluten sensitivity (Gluten Ataxia), tend to present subacutely and usually progress gradually. Acute presentations with rapid progression outside the context of paraneoplastic cerebellar degeneration require prompt diagnosis and early access to disease-modifying immunotherapy in order to avert severe and permanent neurological disability. Case presentations We describe three cases of rapid-onset Gluten Ataxia, an immune-mediated cerebellar ataxia due to gluten sensitivity. We detail their presentation, clinical and neuroimaging findings, and our treatment strategy with immunotherapy. Conclusions Our cases highlight the potential for immune-mediated cerebellar ataxias to present acutely, with rapid-onset symptoms and devastating neurological consequences. We caution against the diagnosis of ‘post-infective cerebellitis’ in adults, and advocate early consideration of an immune-mediated cerebellar ataxia and initiation of immunotherapy to prevent irreversible cerebellar damage.

Cerebellar degeneration in gluten ataxia is linked to microglial activation

“… 2 Interestingly, an unusual pattern of immunoreactivity to Iba-1 was present in one case in which Iba-1 + and MHC-II + staining was present towards the surface of the spinal cord, potentially indicating a diffusion gradient driving inflammation from the CSF. CSF-restricted oligoclonal bands and pleocytosis have been reported in rare cases of rapidly progressive forms of GA. 67 However, further research is needed to provide an in-depth characterization of CSF in patients with GA, including inflammatory markers and the presence of anti-TG6 and anti-gliadin antibodies to better understand the mechanisms of autoimmunity and cerebellar involvement in GA.…”

Ataxias: Hereditary, Acquired, and Reversible Etiologies

“…56 Gluten ataxia refers to an immune-mediated cerebellar ataxia resulting from heightened gluten sensitivity, 57,58 which can present as acute, subacute, or chronic ataxia. 59 Anti-transglutaminase 6 antibody appears to be most specific to gluten ataxia; however, this test has not been commercially available. Therefore, the commercially available anti-gliadin antibody test should be considered to aid in the diagnosis of gluten ataxia.…”