Gluten-Related Disorders: Gluten Ataxia

2015 ◽  
Vol 33 (2) ◽  
pp. 264-268 ◽  
Author(s):  
Marios Hadjivassiliou ◽  
David D. Sanders ◽  
Daniel P. Aeschlimann
Keyword(s):  
Age At Onset ◽  
Clinical Manifestations ◽  
Gastrointestinal Symptoms ◽  
Cerebellar Atrophy ◽  
Gluten Free Diet ◽  
Neurological Dysfunction ◽  
Gluten Free ◽  
Strict Adherence ◽  
Gluten Ataxia ◽  
Antigliadin Antibodies

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.

Nutritional Management of Celiac Disease

2018 ◽  
Author(s):  
Ciarán P Kelly ◽  
Satya Kurada ◽  
Mariana Urquiaga
Keyword(s):  
Immune Response ◽  
Celiac Disease ◽  
Tight Junction ◽  
Villous Atrophy ◽  
Gastrointestinal Symptoms ◽  
Gluten Free Diet ◽  
Intestinal Biopsy ◽  
Nutritional Management ◽  
Gluten Free ◽  
Antigliadin Antibodies

Celiac disease (CD) is an autoimmune disorder characterized by an immune response to gluten peptides in wheat, barley, and rye. The diagnosis of celiac disease is confirmed by three important characteristics: consistent symptoms, positive celiac-specific serology, and small intestinal biopsy findings of inflammation, crypt hyperplasia, and villous atrophy. CD may present with overt gastrointestinal symptoms, including diarrhea (or constipation), weight loss, and abdominal bloating and discomfort, or covertly with micronutrient deficiencies such as iron deficiency with anemia. A gluten-free diet (GFD) remains the mainstay of treatment. The aim of this review is to highlight the pathogenesis of CD, concepts and challenges associated with a GFD, and nutritional management of CD applicable in clinical practice to internists, gastroenterologists, and dietitians. Patients should be referred to an expert celiac dietitian for education on adherence to a GFD to address gluten contamination in the diet, the psychosocial implications of following a GFD, and macro- and micronutrient disequilibria arising from celiac disease and the GFD. Several novel therapeutics are on the horizon in various stages of development, including glutenases, antigliadin antibodies, tight junction regulators, modulation of the immune response to gliadin, and efforts to engineer less toxic gluten-containing foodstuffs. This review contains 3 figures, 5 tables, and 61 references. Key words: celiac disease, genetic engineering, food engineering, gluten, glutenases, gluten-free diet, oats, IgY, nutrition, tight junction regulators, wheat

Nutritional Management of Celiac Disease

2017 ◽  
Author(s):  
Ciarán P Kelly ◽  
Satya Kurada ◽  
Mariana Urquiaga
Keyword(s):  
Immune Response ◽  
Celiac Disease ◽  
Tight Junction ◽  
Villous Atrophy ◽  
Gastrointestinal Symptoms ◽  
Gluten Free Diet ◽  
Intestinal Biopsy ◽  
Nutritional Management ◽  
Gluten Free ◽  
Antigliadin Antibodies

Celiac disease (CD) is an autoimmune disorder characterized by an immune response to gluten peptides in wheat, barley, and rye. The diagnosis of celiac disease is confirmed by three important characteristics: consistent symptoms, positive celiac-specific serology, and small intestinal biopsy findings of inflammation, crypt hyperplasia, and villous atrophy. CD may present with overt gastrointestinal symptoms, including diarrhea (or constipation), weight loss, and abdominal bloating and discomfort, or covertly with micronutrient deficiencies such as iron deficiency with anemia. A gluten-free diet (GFD) remains the mainstay of treatment. The aim of this review is to highlight the pathogenesis of CD, concepts and challenges associated with a GFD, and nutritional management of CD applicable in clinical practice to internists, gastroenterologists, and dietitians. Patients should be referred to an expert celiac dietitian for education on adherence to a GFD to address gluten contamination in the diet, the psychosocial implications of following a GFD, and macro- and micronutrient disequilibria arising from celiac disease and the GFD. Several novel therapeutics are on the horizon in various stages of development, including glutenases, antigliadin antibodies, tight junction regulators, modulation of the immune response to gliadin, and efforts to engineer less toxic gluten-containing foodstuffs. This review contains 3 figures, 5 tables, and 61 references. Key words: celiac disease, genetic engineering, food engineering, gluten, glutenases, gluten-free diet, oats, IgY, nutrition, tight junction regulators, wheat

Nutritional Management of Celiac Disease

2017 ◽  
Author(s):  
Ciarán P Kelly ◽  
Satya Kurada ◽  
Mariana Urquiaga
Keyword(s):  
Immune Response ◽  
Celiac Disease ◽  
Tight Junction ◽  
Villous Atrophy ◽  
Gastrointestinal Symptoms ◽  
Gluten Free Diet ◽  
Intestinal Biopsy ◽  
Nutritional Management ◽  
Gluten Free ◽  
Antigliadin Antibodies

Celiac disease (CD) is an autoimmune disorder characterized by an immune response to gluten peptides in wheat, barley, and rye. The diagnosis of celiac disease is confirmed by three important characteristics: consistent symptoms, positive celiac-specific serology, and small intestinal biopsy findings of inflammation, crypt hyperplasia, and villous atrophy. CD may present with overt gastrointestinal symptoms, including diarrhea (or constipation), weight loss, and abdominal bloating and discomfort, or covertly with micronutrient deficiencies such as iron deficiency with anemia. A gluten-free diet (GFD) remains the mainstay of treatment. The aim of this review is to highlight the pathogenesis of CD, concepts and challenges associated with a GFD, and nutritional management of CD applicable in clinical practice to internists, gastroenterologists, and dietitians. Patients should be referred to an expert celiac dietitian for education on adherence to a GFD to address gluten contamination in the diet, the psychosocial implications of following a GFD, and macro- and micronutrient disequilibria arising from celiac disease and the GFD. Several novel therapeutics are on the horizon in various stages of development, including glutenases, antigliadin antibodies, tight junction regulators, modulation of the immune response to gliadin, and efforts to engineer less toxic gluten-containing foodstuffs. This review contains 3 figures, 5 tables, and 61 references. Key words: celiac disease, genetic engineering, food engineering, gluten, glutenases, gluten-free diet, oats, IgY, nutrition, tight junction regulators, wheat

A Case of Cognitive Impairment and Ataxia

2018 ◽  
pp. 65-68
Author(s):  
Aaron E. Miller ◽  
Tracy M. DeAngelis ◽  
Michelle Fabian ◽  
Ilana Katz Sand
Keyword(s):  
Celiac Disease ◽  
Gastrointestinal Symptoms ◽  
Cerebellar Atrophy ◽  
Adaptive Immune System ◽  
Cell Loss ◽  
Mucosal Injury ◽  
Inflammatory Infiltration ◽  
Gluten Ataxia ◽  
Antigliadin Antibodies ◽  
Intestinal Mucosal Injury

Celiac disease is an autoimmune enteropathy characterized by the presence of antigliadin antibodies and small intestinal mucosal injury, mediated by the adaptive immune system in response to gluten. Gastrointestinal symptoms typically include diarrhea, malabsorption/weight loss, and bloating/abdominal pain. Extra-intestinal symptoms often occur. The most commonly reported neurological syndromes are peripheral neuropathy, headache, and “gluten ataxia.” Gluten ataxia may occur in the setting of celiac disease or with non-celiac gluten sensitivity. Consistent with this being a cerebellar phenomenon, autopsy studies demonstrate inflammatory infiltration and Purkinje cell loss. Cerebellar atrophy as well as white matter abnormalities may be evident on MRI. Treatment is institution of a gluten-free diet. Intravenous immune globulin may also be helpful in select patients.

Effect of gluten-free diet on cerebellar MR spectroscopy in gluten ataxia

Neurology ◽  
2017 ◽  
Vol 89 (7) ◽  
pp. 705-709 ◽  
Author(s):  
Marios Hadjivassiliou ◽  
Richard A. Grünewald ◽  
David S. Sanders ◽  
Priya Shanmugarajah ◽  
Nigel Hoggard
Keyword(s):  
Clinical Care ◽  
Area Ratio ◽  
Gluten Free Diet ◽  
Resonance Spectroscopy ◽  
Gluten Free ◽  
Positive Serology ◽  
Gluten Ataxia ◽  
Antigliadin Antibodies ◽  
Sporadic Ataxia ◽  
Repeat Scanning

Objective:To evaluate the effect of gluten free diet (GFD) on magnetic resonance spectroscopy (MRS) of the cerebellum in patients with gluten ataxia (GA).Methods:Patients with GA, defined as sporadic ataxia with positive antigliadin antibodies in the absence of an alternative cause, routinely undergo MRS at baseline and after the introduction of GFD as part of their clinical care. We present our experience of the effect of GFD on MRS of the cerebellum.Results:A total of 117 consecutive patients with GA were included in this report. Sixty-three were on strict GFD with elimination of antigliadin antibodies, 35 were on GFD but were still positive for antigliadin antibodies, and 19 patients opted not to go on GFD. The N-acetylaspartate (NAA)/creatine (Cr) area ratio from the cerebellar vermis increased in 62 out of 63 (98%) patients on strict GFD, in 9 of 35 (26%) patients on GFD but positive antibodies, and in only 1 of 19 (5%) patients not on GFD. The NAA/Cr ratio decreased in all 14 ataxia control patients (cerebellar variant of multisystem atrophy). There were no differences in the MRS results between those patients who had and those who did not have enteropathy (celiac disease) within each group.Conclusions:The demonstration of increased NAA/Cr ratio on repeat scanning following strict GFD strengthens previous findings of clinical improvement of the ataxia in patients with GA. The presence of enteropathy is not a prerequisite for such improvement; therefore patients with positive serology and negative duodenal biopsy should still be treated with strict GFD.

scholarly journals Gluten Degrading Enzymes for Treatment of Celiac Disease

Nutrients ◽  
2020 ◽  
Vol 12 (7) ◽  
pp. 2095 ◽  
Author(s):  
Guoxian Wei ◽  
Eva J. Helmerhorst ◽  
Ghassan Darwish ◽  
Gabriel Blumenkranz ◽  
Detlef Schuppan
Keyword(s):  
Celiac Disease ◽  
Enzyme Stability ◽  
Clinical Manifestations ◽  
Cysteine Proteases ◽  
Gluten Free Diet ◽  
Gluten Free ◽  
Strict Adherence ◽  
Degrading Enzymes ◽  
Gluten Peptides ◽  
Promising Therapeutic Approach

Celiac disease (CeD) affects about 1% of most world populations. It presents a wide spectrum of clinical manifestations, ranging from minor symptoms to mild or severe malabsorption, and it may be associated with a wide variety of autoimmune diseases. CeD is triggered and maintained by the ingestion of gluten proteins from wheat and related grains. Gluten peptides that resist gastrointestinal digestion are antigenically presented to gluten specific T cells in the intestinal mucosa via HLA-DQ2 or HLA-DQ8, the necessary genetic predisposition for CeD. To date, there is no effective or approved treatment for CeD other than a strict adherence to a gluten-free diet, which is difficult to maintain in professional or social environments. Moreover, many patients with CeD have active disease despite diet adherence due to a high sensitivity to traces of gluten. Therefore, safe pharmacological treatments that complement the gluten-free diet are urgently needed. Oral enzyme therapy, employing gluten-degrading enzymes, is a promising therapeutic approach. A prerequisite is that such enzymes are active under gastro-duodenal conditions, quickly neutralize the T cell activating gluten peptides and are safe for human consumption. Several enzymes including prolyl endopeptidases, cysteine proteases and subtilisins can cleave the human digestion-resistant gluten peptides in vitro and in vivo. Examples are several prolyl endopeptidases from bacterial sources, subtilisins from Rothia bacteria that are natural oral colonizers and synthetic enzymes with optimized gluten-degrading activities. Without exception, these enzymes must cleave the otherwise unusual glutamine and proline-rich domains characteristic of antigenic gluten peptides. Moreover, they should be stable and active in both the acidic environment of the stomach and under near neutral pH in the duodenum. This review focuses on those enzymes that have been characterized and evaluated for the treatment of CeD, discussing their origin and activities, their clinical evaluation and challenges for therapeutic application. Novel developments include strategies like enteric coating and genetic modification to increase enzyme stability in the digestive tract.

scholarly journals Neurological disorders associated with gluten sensitivity

2017 ◽  
Vol 89 (2) ◽  
pp. 99-102
Author(s):  
D A Degterev ◽  
I V Damulin ◽  
A I Parfenov
Keyword(s):  
Clinical Features ◽  
Neurological Disorders ◽  
Gluten Free Diet ◽  
Gluten Sensitivity ◽  
Gluten Free ◽  
Gluten Ataxia

The review considers the pathogenetic, clinical, and therapeutic aspects of neurological disorders associated with gluten sensitivity. Gluten ataxia and polyneuropathy are most common. The clinical features of neurological disorders in patients with gluten sensitivity and the effects of a gluten-free diet are described.

Sa1268 The Relationship Between Self-Reported Strict Adherence to a Gluten-Free Diet and the Ability to Identify Gluten-Containing Foods

2013 ◽  
Vol 144 (5) ◽  
pp. S-247
Author(s):  
Jocelyn A. Silvester ◽  
Dayna Weiten ◽  
Lesley A. Graff ◽  
John R. Walker ◽  
Donald R. Duerksen
Keyword(s):  
Gluten Free Diet ◽  
Gluten Free ◽  
Strict Adherence ◽  
The Relationship

scholarly journals Clinical Characteristics and Management of 50 Patients with Anti-GAD Ataxia: Gluten-Free Diet Has a Major Impact

2020 ◽  
Author(s):  
M. Hadjivassiliou ◽  
P. G. Sarrigiannis ◽  
P. D. Shanmugarajah ◽  
D. S. Sanders ◽  
R. A. Grünewald ◽  
...  
Keyword(s):  
Autoimmune Diseases ◽  
Clinical Characteristics ◽  
Age At Onset ◽  
Gluten Free Diet ◽  
Gluten Sensitivity ◽  
Gluten Free ◽  
Progressive Ataxia ◽  
Progressive Cerebellar Ataxia ◽  
Limb Ataxia ◽  
History Of

Abstract The objective of this study is to report the clinical characteristics and treatment of patients with progressive cerebellar ataxia associated with anti-GAD antibodies. We performed a retrospective review of all patients with anti-GAD ataxia managed at the Sheffield Ataxia Centre over the last 25 years. We identified 50 patients (62% females) with anti-GAD ataxia. The prevalence was 2.5% amongst 2000 patients with progressive ataxia of various causes. Mean age at onset was 55 and mean duration 8 years. Gaze-evoked nystagmus was present in 26%, cerebellar dysarthria in 26%, limb ataxia in 44% and gait ataxia in 100%. Nine patients (18%) had severe, 12 (24%) moderate and 29 (58%) mild ataxia. Ninety percent of patients had a history of additional autoimmune diseases. Family history of autoimmune diseases was seen in 52%. Baseline MR spectroscopy of the vermis was abnormal at presentation in 72%. Thirty-five patients (70%) had serological evidence of gluten sensitivity. All 35 went on gluten-free diet (GFD). Eighteen (51%) improved, 13 (37%) stabilised, 3 have started the GFD too recently to draw conclusions and one deteriorated. Mycophenolate was used in 16 patients, 7 (44%) improved, 2 stabilised, 6 have started the medication too recently to draw conclusions and one did not tolerate the drug. There is considerable overlap between anti-GAD ataxia and gluten ataxia. For those patients with both, strict GFD alone can be an effective treatment. Patients with anti-GAD ataxia and no gluten sensitivity respond well to immunosuppression.

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