Gluten Ataxia

The Gluten File

 PubMed Abstracts 

Cerebellar signs in celiac disease.
PMID: 19506229  June 2009

RESULTS: Whereas the development of anti-transglutaminase 2 IgA is linked with gastrointestinal disease, an anti-transglutaminase 6 IgG and IgA response is prevalent in gluten ataxia, independent of intestinal involvement. Such antibodies are absent in ataxia of defined genetic origin or in healthy individuals. Inhibition studies showed that in those patients with ataxia and enteropathy, separate antibody populations react with the two different transglutaminase isozymes. Furthermore, postmortem analysis of brain tissue showed cerebellar IgA deposits that contained transglutaminase 6. INTERPRETATION: Antibodies against transglutaminase 6 can serve as a marker in addition to human leukocyte antigen type and detection of anti-gliadin and anti-transglutaminase 2 antibodies to identify a subgroup of patients with gluten sensitivity who may be at risk for development of neurological disease.
Autoantibodies in gluten ataxia recognize a novel neuronal transglutaminase.
PMID: 18825674  Sept 2008

Gluten ataxia is an immune-mediated disease triggered by the ingestion of gluten in genetically susceptible individuals. It should be considered in the differential diagnosis of all patients with idiopathic sporadic ataxia. Early diagnosis and treatment with a gluten free diet can improve ataxia and prevent its progression. Readily available and sensitive markers of gluten ataxia include antigliadin antibodies. IgA deposits against TG2 in the small bowel and at extraintestinal sites are proving to be additional reliable and perhaps more specific markers of the whole spectrum of gluten sensitivity. They may also hold the key to its pathogenesis.
Gluten ataxia.
PMID: 18787912  Sept 2008

Dermatitis herpetiformis and coeliac disease are gluten-sensitive diseases that share immunopathological mechanisms. Neurological disorders are reported in both diseases, being more frequent in coeliac disease. Dermatitis herpetiformis is rare in paediatric populations and only sporadic cases with neurological dysfunction are reported. Uncertainty exists as to whether early treatment may stop or reverse neurological symptoms. We describe here the case of a child presenting with a rash and ataxia, diagnosed with dermatitis herpetiformis, in whom neurological symptoms and signs regressed after treatment.

Dermatitis herpetiformis presenting as ataxia in a child.
PMID: 17340026 2007

Myoclonus in a context of progressive ataxia suggests one clinical form of the Ramsay-Hunt syndrome (progressive myoclonic ataxia, PMA), whose most frequent causes are: coeliac disease, mitochondriopathies, some spino-cerebellar degenerations, and some late metabolic disorders.
Symptomatic myoclonus.  PMID: 17336775  Sep-Dec 2006

Gluten sensitivity in Japanese patients with adult-onset cerebellar ataxia.
PMID: 16508226  Mar 2006

CONCLUSIONS: Anti-tissue transglutaminase IgA antibodies are present in the gut and brain of patients with gluten ataxia with or without an enteropathy in a similar fashion to patients with celiac disease, latent celiac disease, and dermatitis herpetiformis but not in ataxia control subjects. This finding strengthens the contention that gluten ataxia is immune mediated and belongs to the same spectrum of gluten sensitivity as celiac disease and dermatitis herpetiformis.

Autoantibody targeting of brain and intestinal transglutaminase in gluten ataxia.

PMID: 16476935  Feb 2006

CONCLUSION: Screening for AGA presence should be systematically performed at presentation of patients with unknown etiology ataxia; in the event AGA are present without any other etiology, treatment with gluten-free diet must be discussed. ....more

[Ataxia associated with gluten sensitivity, myth or reality?]

PMID: 16518262 Feb 2006

[Progressive myoclonic ataxia associated with antibodies against Purkinje cells in a celiac patient]
PMID: 16454613 Dec 2005

Cerebellar abnormalities on proton MR spectroscopy in gluten ataxia.
PMID: 15965215 July 2005 

We report an unusual case of celiac disease with cerebellar ataxia. Gastrointestinal signs and malabsorption were not found in this patient. We suggested that celiac disease should be taken into consideration in differential diagnosis of patients with cerebellar ataxia with unknown etiology.

Subclinical celiac disease with cerebellar ataxia.
PMID: 15508272  June 2004

Gluten ataxia responds to a strict gluten-free diet even in the absence of an enteropathy. The diagnosis of gluten ataxia is vital as it is one of the very few treatable causes of sporadic ataxia.
Dietary treatment of gluten ataxia.
PMID: 12933922  Sept 2003

Gluten ataxia is therefore the single most common cause of sporadic idiopathic ataxia. Antigliadin antibody testing is essential at first presentation of patients with sporadic ataxia.
Gluten ataxia in perspective: epidemiology, genetic susceptibility and clinical characteristics
PMID: 12566288  March 2003

Patients with gluten ataxia have antibodies against Purkinje cells. Antigliadin antibodies cross-react with epitopes on Purkinje cells.
The humoral response in the pathogenesis of gluten ataxia.

PMID: 11971090  April 2002

The fact that the disease is strongly associated with the same HLA haplotypes found in coeliac disease not only demonstrates coeliac disease and ataxia with gluten sensitivity to be part of the same disease entity but supports the hypothesis of an immunological pathogenesis of cerebellar degeneration.
Sporadic cerebellar ataxia associated with gluten sensitivity.
PMID: 11335703  May 2001