GLUTEN ATAXIA - A COMMON, COMMONLY MISDIAGNOSED DISEASE

The objective of our research was to raise awareness of neurologists and family physicians about gluten ataxia and improve the diagnostic approaches. Methods. PubMed and Google Scholar resources were used to write the review article. Discussion. It is incredibly important not to overlook treatable courses of ataxia. One of these diseases is gluten ataxia. Early diagnosis and timely treatment (gluten-free diet) of gluten ataxia leads to a significant improvement of clinical symptoms and prevents their progression. Gluten ataxia is one of the most common and commonly misdiagnosed ataxia. Its prevalence among all ataxias is 15% and reaches 40% among all idiopathic sporadic ataxias (Hadjivassiliou M., Sanders D.D., Aeschlimann D.P., 2015). Keywords: gluten ataxia, celiac disease, gluten-related disorders.

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

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.

Gluten-related disorders

Gluten-related disorders are a heterogeneous group of clinical entities caused by intolerance of wheat, rye, and barley flour components. They occur in 3-5% of genetically predisposed persons and based on pathogenic and clinical features are classified into celiac disease, non-celiac gluten sensitivity, and wheat allergy. There are also specific entities such as dermatitis herpetiformis or gluten ataxia, which can occur either within the celiac disease or independently. This article based on the current knowledge shows the basic details of the pathogenesis, clinical expression, diagnosis, and treatment of these disorders.

Gluten Ataxia Associated with Dietary Protein Cross-Reactivity with GAD-65

Cross-reactivity occurs when antibodies formed against an antigen have amino acid sequence homology with another target protein. This allows antibodies formed against the antigen to also bind to similar proteins that share structural similarity. Autoimmune reactions to gluten can lead to sporadic ataxia in susceptible genotypes due to cross-reactivity. With gluten ataxia, dietary consumption of gluten proteins induce immunological cross-reactivity with glutamic-acid decarboxylase-65 (GAD-65) target proteins found in the cerebellum. Implementation of a strict gluten-free diet has been shown to improve the expression of this form of ataxia with most patients in this subgroup. However, there are some subjects that have limited clinical responses to only a strict gluten-free diet. Dietary protein cross-reactivity to other food proteins, besides gluten, that also share structural similarity to GAD-65 may also play a role in this reaction. In this case report, we report of a patient suffering from gluten-ataxia in which a gluten-free diet alone did not generate significant clinical outcomes until other foods that cross-react with GAD-65 were also removed from their diet.

Prevalence of gluten-related disorders in Asia-Pacific region: a systematic review

Background & Aims: The epidemiology of gluten-related disorders (GRDs) is still an open field to be explored. We conducted this systematic review based on the current epidemiology knowledge of GRDs, focusing on the changing prevalence of GRDs reported in the Asia-Pacific region.Methods: We searched Medline, PubMed, Scopus, Web of Science and Cochrane database with the following MeSH terms and keywords: celiac disease (CD), wheat allergy (WA), non-celiac gluten sensitivity (NCGS), dermatitis herpetiformis (DH) and gluten ataxia (GA) and the prevalence studies published from January 1991 to January 2018. Each article was cross-referenced with “Asia-Pacific region” and countries in this regionsuch as Australia, New Zealand, India, Pakistan, Turkey, Iran and others.Results: We included 66 studies, which reported the prevalence of GRDs in the Asia-Pacific region. Prevalence of celiac disease was 0.32%-1.41% in healthy children and 0.05%-1.22% in the adult population, while the prevalence in the high risk population was higher (0.6%-11.8%). Previous studies have shown a very low incidence of dermatitis herpetiformis (DH) (<0.001%) and gluten ataxia (GA) in this area. Few studies on NCGS outbreaks have been found in this area due to the lack of specific diagnostic biomarkers. Wheat allergy (WA), although uncommon in most Asian-Pacific countries, is the most common cause of anaphylaxis in this region.Conclusion: The results of this systematic review suggest the need to plan further proper epidemiological studies in order to understand the natural history of GRDs and to assess its burden on health systems.

Neurological Manifestations of Neuropathy and Ataxia in Celiac Disease: A Systematic Review

Celiac disease (CD) is an immune-mediated gastrointestinal disorder driven by innate and adaptive immune responses to gluten. Patients with CD are at an increased risk of several neurological manifestations, frequently peripheral neuropathy and gluten ataxia. A systematic literature review of the most commonly reported neurological manifestations (neuropathy and ataxia) associated with CD was performed. MEDLINE, Embase, the Cochrane Library, and conference proceedings were systematically searched from January 2007 through September 2018. Included studies evaluated patients with CD with at least one neurological manifestation of interest and reported prevalence, and/or incidence, and/or clinical outcomes. Sixteen studies were included describing the risk of gluten neuropathy and/or gluten ataxia in patients with CD. Gluten neuropathy was a neurological manifestation in CD (up to 39%) in 13 studies. Nine studies reported a lower risk and/or prevalence of gluten ataxia with a range of 0%–6%. Adherence to a gluten-free diet appeared to improve symptoms of both neuropathy and ataxia. The prevalence of gluten neuropathy and gluten ataxia in patients with CD varied in reported studies, but the increased risk supports the need for physicians to consider CD in patients with ataxia and neurological manifestations of unknown etiology.

Commentary: “Gluten Ataxia and their relationships with Celiac Disease and Non-Celiac Gluten Sensitivity”

The word "ataxia" means etymologically, incoordination or clumsiness. It is a symptom, not a diagnosis in itself, or a clinical entity. The diseases that present it, have as a common denominator, the permanent presence of a progressive alteration of the balance, more evident when standing and walking, together with a lack of coordination of the extremities with movement, all accompanied by disorders of the language, consisting of a difficulty in pronouncing well, having to work much harder than usual, to achieve an understanding, especially with difficult words, or in prolonged conversations. The underlying lesion is located mainly at the level of the cerebellum, which is the part of the brain responsible for coordinating movements and the center of postural balance, as well as the language.