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Cerebellar Disorders due to Gluten sensitivity

Timothy C. Hain, MD Page last modified: July 15, 2019

Related page: cerebellar

Autoantibodies to gluten and ataxia:

The cerebellum may be injured by antibodies or other immune system disturbances such as in the paraneoplastic cerebellar degenerations and in celiac disease (Gluten enteropathy).

Hadivassiliou (Hadjivassiliou et al. 2003) described a large number of patients with gluten sensitivity presenting with ataxia. The mean age of onset of ataxia in these patients was 48. Few of these patients had gastrointestinal symptoms. Atrophy of the cerebellum was noted in 79%. According to Hadjivassiliou there is some evidence for the existence of gluten related neurological syndromes (Hadjivassiliou, Grunewald et al. 2002; Hadjivassiliou, Grunewald et al. 2003). Hadjivassiliou suggests that gluten intolerance related ataxia is the single most common cause of a sporadic ataxia, accounting for about 41% of cases. There are a number of recent related publications supporting gluten ataxia (Rodrigo et al. 2016) (Nanri et al. 2014; Guan et al. 2013; Hadjivassiliou et al. 2013; Rashtak et al. 2011)Looking at the donut hole rather than the donut, Mittelbronn, claimed that up to 8% of patients with gluten sensitivity (GS) develop neurological symptoms such as ataxia, dementia, seizures or peripheral neuropathy. Of course, these are common symptoms in the general population too, and there may not be a causal relationship.

Others are not so sure that gliaden sensitivity is a significant source of neurological disability. Willis and Unsworth observed that 10% of the otherwise normal population has anti-gliaden antibodies, which would suggest a very high rate of false-positives would be expected. (Willis and Unsworth, 2002).

The underlying immunological mechanisms for gluten ataxia still remain to be elucidated.

Testing for gluten ataxia:

This is largely a "wastebasket syndrome", although the recent development of more specific antibodies (i.e. anti-transglutamase-6) is promising. One should combine both evidence for gluten antibodies as well as reasonable evidence for cerebellar ataxia. The latter might reasonably include cerebellar findings on exam and cerebellar atrophy on MRI.

Treatment:

The treatment of Gluten ataxia is dietary. A gluten-free diet should reasonably be tried in persons who have no other cause for ataxia. Hadjivassiliou, in an unblinded trial, but using somewhat objective measures, reported improvement after a year of diet (2003). It seems to us that without a placebo control, which is of course impossible for a diet like this, the value of this observation is unclear.

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Copyright July 15, 2019 , Timothy C. Hain, M.D. All rights reserved. Last saved on July 15, 2019