Gluten-Related Disorders: Gluten Ataxia

Abstract

The term gluten-related disorders (GRD) refers to a spectrum of diverse clinical manifestations triggered by the ingestion of gluten in genetically susceptible individuals. They include both intestinal and extraintestinal manifestations. Gluten ataxia (GA) is one of the commonest neurological manifestations of GRD. It was originally defined as otherwise idiopathic sporadic ataxia in the presence of circulating antigliadin antibodies of IgA and/or IgG type. Newer more specific serological markers have been identified but are not as yet readily available. GA has a prevalence of 15% amongst all ataxias and 40% of all idiopathic sporadic ataxias. It usually presents with gait and lower limb ataxia. It is of insidious onset with a mean age at onset of 53 years. Up to 40% of patients have evidence of enteropathy on duodenal biopsy. Gastrointestinal symptoms are seldom prominent and are not a reliable indicator for the presence of enteropathy. Furthermore, the presence of enteropathy does not influence the response to a gluten-free diet. Most patients will stabilise or improve with strict adherence to gluten-free diet depending on the duration of the ataxia prior to the treatment. Up to 60% of patients with GA have evidence of cerebellar atrophy on MR imaging, but all patients have spectroscopic abnormalities primarily affecting the vermis. Recent evidence suggests that patients with newly diagnosed coeliac disease presenting to the gastroenterologists have abnormal MR spectroscopy at presentation associated with clinical evidence of subtle cerebellar dysfunction. The advantage of early diagnosis and treatment (mean age 42 years in patients presenting with gastrointestinal symptoms vs. 53 years in patients presenting with ataxia) may protect the first group from the development and/or progression of neurological dysfunction.

Clinical Features in Border Terrier Dogs with Paroxysmal Involuntary Movements

“…It is also possible that the new diets improved the Border Terriers’ general health status and reduced their stress; in fact, 28% of the dogs in this study presented recurrent skin and ear problems and 16% of the dogs had recurrent gastrointestinal problems, which may be associated with an underlying food hypersensitivity. However, there is mounting evidence of an influence of the brain‐gut‐microbiome axis on central nervous system neurotransmission, and enteric glial cells have been involved in the pathophysiology of gastrointestinal disease, such inflammatory bowel disease, gluten ataxia, but also Parkinson's disease . An association between diet and severity of Border Terrier attacks, as suggested by our findings and Black et al's study, needs to be verified with a prospective study; however, if this hypothesis was to be confirmed, the Border Terrier dogs could represent an ideal spontaneous model to investigate the effects of diet on paroxysmal dyskinesias.…”

Ataxia Triggered by Gluten

“…It is considered as one of the commonest neurological manifestations of gluten-related disorders: its prevalence was estimated at 15% amongst all ataxias and 40% of all idiopathic sporadic ataxias . [6]. Its diagnosis should be confirmed by the presence of anti-gliadin antibodies, with or without other serological, autoimmune markers of gluten intolerance (i.e.…”

“…Its diagnosis should be confirmed by the presence of anti-gliadin antibodies, with or without other serological, autoimmune markers of gluten intolerance (i.e. anti-tissue trnasglutaminase) [6,7]. Early diagnosis and treatment may protect the first group from the development and/or progression of neurological dysfunction.…”

“…[8] Gluten free diet remains the only therapy that has proven real efficacy, and the response to diet seems depending on the prior duration of the ataxia before diagnosis. [6,7,9].…”

Serum antigliadin antibodies in cerebellar ataxias: a systematic review and meta-analysis

“…AGA has been reported in 43% of otherwise idiopathic cerebellar ataxia (IDCA) cases; however, AGA is also found in up to 12% controls and in 13% hereditary ataxia (HA) cases 9 10. When comparing with controls, IDCA cases are thus more commonly reported to have AGA seropositivity 10. However, these results have been inconsistently replicated 3 11–14.…”