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    Gluten Triggers 1 in 4 Cases of Sporadic Ataxia


    Jefferson Adams
    • What is the role of gluten in cerebellar ataxia?

    Gluten Triggers 1 in 4 Cases of Sporadic Ataxia
    Image Caption: Image: CC-- Dierk Schaefer

    Celiac.com 01/16/2017 - Cerebellar ataxias can be caused by a wide range of disease processes, either genetic or acquired. Establishing a clear diagnosis requires a methodical approach with expert clinical evaluation and investigation.

    A team of researchers recently published a description of the causes of ataxia in 1500 patients with cerebellar ataxia.  The research team included M Hadjivassiliou, J Martindale, P Shanmugarajah, R A Grünewald, P G Sarrigiannis, N Beauchamp, K Garrard, R Warburton, D S Sanders, D Friend, S Duty, J Taylor, and N Hoggard.

    They are variously affiliated with the Academic Department of Neurosciences, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Trust, Sheffield, UK; Sheffield Diagnostic Genetics Service, Sheffield Children's NHS Foundation Trust, Sheffield, UK; the Department of Gastroenterology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Trust, Sheffield, UK; and the Department of Neuroradiology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Trust, Sheffield, UK.

    All patients in the study were referred to the Sheffield Ataxia Centre, UK, and underwent extensive examination, including, where appropriate genetic testing using next-generation sequencing (NGS).

    The team followed-up patients on a 6-month basis for reassessment and further investigations, as needed.

    The team assessed a total of 1500 patients over 20 years. Twenty per cent of those patients had a family history of ataxia, with the remaining having sporadic ataxia.

    The most common cause of sporadic ataxia was gluten ataxia at 25%. They found a genetic cause in 156, or 13% of sporadic cases, with alcohol excess causing 12% and a cerebellar variant of multiple system atrophy causing 11% of sporadic cases.

    Using NGS, they obtained positive results in 32% of 146 patients tested. The most common ataxia they found was EA2. A total of 57% of all familial ataxias were supported by genetic diagnosis. The most common genetic ataxias were Friedreich's ataxia (22%), SCA6 (14%), EA2 (13%), SPG7 (10%) and mitochondrial disease (10%).

    The diagnostic yield following attendance at the Sheffield Ataxia Centre was 63%. Immune-mediated ataxias are common. Advances in genetic testing have significantly improved the diagnostic yield of patients suspected of having a genetic ataxia.

    Making a diagnosis of the cause of ataxia is essential due to potential therapeutic interventions for immune and some genetic ataxias.

    Gluten is a culprit is 25% of sporadic ataxia cases, and clinicians should keep this in mind when diagnosing patients, as many of these cases can be reversed with a gluten-free diet.

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    Guest rosalyn

    Posted

    Terms were not defined, so I didn't understand what the article was talking about other than it had to do something with the brain.

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    Guest Jefferson Adams

    Posted

    Terms were not defined, so I didn't understand what the article was talking about other than it had to do something with the brain.

    Key terms are highlighted with definitions. For the rest, maybe try Google.

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    Guest Julie Hahn

    Posted

    I have gluten ataxia and am part of a very small worldwide support group of those with Ataxia caused by celiac disease/gluten sensitivity. Three of the members of the group see Prof. Marios Hadjivassiliou. One is a good friend and was one of his earliest patients to be diagnosed decades ago. A gluten-free diet did the trick for me. However, because I was already over 50 when diagnosed I've not and won't recover 100% of my previous function. My gastro (Dr. Scot Lewey) consulted with Prof. Hadjivassiliou regarding my prognosis when I was first diagnosed. I was featured in an article about the neurological manifestations of Celiac Disease in the formerly named "Living Without"magazine in their April/May 2014 issue.

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  • About Me

    Jefferson Adams earned his B.A. and M.F.A. at Arizona State University, and has authored more than 2,000 articles on celiac disease. His coursework includes studies in biology, anatomy, medicine, and science. He previously served as Health News Examiner for Examiner.com, and provided health and medical content for Sharecare.com.

    Jefferson has spoken about celiac disease to the media, including an appearance on the KQED radio show Forum, and is the editor of the book Dangerous Grains by James Braly, MD and Ron Hoggan, MA.

  • Related Articles

    Jefferson Adams
    Celiac.com 08/13/2009 - In the latest issue of the journal Medical Hypotheses, Dr. Rodney Philip Kinvig Ford of the Children’s Gastroenterology and Allergy Clinic in Christchurch, New Zealand, offers up a compelling hypothesis regarding celiac disease and gluten sensitivity, which asserts that the broad array of associated symptoms are more fully explained using a neurological perspective, than using a digestive/nutritional perspective.
    For Dr. Ford, the idea that celiac disease is exclusively an auto-immune condition, and that nutritional mal-absorption is the main cause of related problems, is simply not borne out by the body of clinical data.
    Dr. Ford accepts that celiac disease may itself be largely an auto-immune disorder. However, he believes that the broad array of problems associated with gluten intolerance are best explained by looking at the neurological aspects of intolerance to gluten, indeed, treating it as a neurological condition.
    That's because gluten intolerance can affect up to up to 10% of the population, and that intolerance to gluten has largely neurological manifestations. That is, up to 10% of the population tests positive for elevated antibodies for gluten, even with no bowel damage.
    Under Dr. Ford's hypothesis, neurological causes, rather than gut damage and nutritional deficiency, best explain
    the myriad symptoms experienced by sufferers of celiac disease and gluten-sensitivity.
    Under Dr. Ford hypothesis, if gluten is the assumed cause of harm, then exposure to gluten in sensitive individuals may cause neurological harm through a combination of cross-reacting antibodies, immune complex disease and direct toxicity.
    It's certainly true that a number of celiac patients experience neurological symptoms, often associated with autonomic nervous system malfunction.
    Such neurological symptoms can even show up in celiac patients who are otherwise well nourished. Moreover, gluten-sensitivity can be associated with neurological symptoms in patients who have no mucosal gut damage--that is, patients who are clinically free of celiac disease.
    Dr. Ford argues that gluten exposure can cause neurological harm through a combination of cross-reacting antibodies, immune complex disease and direct toxicity. These nervous system affects include: dis-regulation of the autonomic nervous system, cerebella ataxia, hypotonia, developmental delay, learning disorders, depression, migraine, and headache. He calls such neurologically-driven sensitivity to gluten ‘‘The Gluten Syndrome."
    Hypothesis: Gluten causes symptoms, in both celiac disease and non-celiac gluten-sensitivity, by its adverse actions on the nervous system.
    Many celiac patients experience neurological symptoms, frequently associated with malfunction of the autonomic nervous system. These neurological symptoms can present in celiac patients who are well nourished. The crucial point, however, is that gluten-sensitivity can also be associated with neurological symptoms in patients who do not have any mucosal gut damage (that is, without celiac disease).
    Gluten can cause neurological harm through a combination of cross-reacting antibodies, immune complex disease and direct toxicity. These nervous system affects include: dis-regulation of the autonomic nervous system, cerebella ataxia, hypotonia, developmental delay, learning disorders, depression, migraine, and headache.
    If gluten is the putative harmful agent, then there is no requirement to invoke gut damage and nutritional deficiency to explain the myriad symptoms experienced by sufferers of celiac disease and
    gluten-sensitivity. This he calls: ‘‘The Gluten Syndrome."
    To support his hypothesis, Dr. Ford cites a study of 921 children carried out at his gastroenterology and allergy clinic. All children were screened for celiac disease via IgG-gliadinantibody (InovaDiagnostics) and tissue trans-glutaminase (tTG); and 190 had a small bowel biopsy. Results showed 724 with high IgG-gliadin levels (>14 units): mean age 5.3 years, s.d. 3.8.
    In a key part of the, all children, whatever the biopsy results, were offered a gluten-free diet.
    Results fell into three distinct categories:
    (a) Deï¬nite celiac disease was revealed in 31 patients (4.3%), via histologic diagnosis. 94% of these patients reported improvement on a gluten-free diet.
    ( Possible celiac was revealed in 48 patients (6.6%), who had elevated tTG antibodies, but normal gut histology: 75% of these patients reported improvement on a gluten-free diet.
    © Not-celiacs, n=644 (89.1%), with normal tTG antibodies and no evidence of gut damage: 53% reported improvement gluten-free.
    Note that last category: More than half of people without celiac disease reported improvement on a gluten-free diet. What's up with that? Well, those are the people Dr. Ford suspects suffer from "gluten syndrome."
    The parents of apparently ‘‘asymptomatic” children were interviewed as part of a population study to identify those with celiac disease. They found many children who had positive tests for gliadin antibodies also had irritability, lethargy, abdominal distension, gas, and poor weight gains.  A high proportion of children with gastro-intestinal, allergy, and neurological conditions have elevated IgG-gliadin antibodies.
    The three groups all shared similar clinical features. In the respective groups, 71%, 65%, and 51% of patients reported behavior issues, such as tiredness, lethargy, irritability, sleep disturbance, while 16%, 15%, and 24% reported gastric reflux. Dr. Ford believes these symptoms are likely to be neurologically driven by gluten-sensitivity.
    Celiac patients completed a questionnaire regarding the presence of neurological symptoms. Those reporting any neurological manifestations were compared with a control group: celiac patients had more neurological disorders (51.4%) in comparison with controls (19.9%). These conditions included: hypotonia, developmental delay, learning disorders, attention deï¬cit hyperactivity disorder, migraine, headache, and cerebella ataxia.
    For Dr. Ford, not only is it significant that such high numbers of people with celiac disease report neurological issues, but it is also significant that the majority of 'non-celiac' patients report improvement on a gluten-free diet.
    These patients are likely candidates for what he calls 'gluten syndrome.' These children can likely be spotted via screening for high IgG-gliadin levels.
    Dr. Ford believes the next step is to test this hypothesis in a double-blind study.
    Certainly, the idea that a whole category of non-celiac gluten-sensitivity exists is intriguing, as is the idea that a neurological take on celiac-disease and gluten-sensitivty might might provide a better or improved understanding of those who suffer from these conditions.
    Medical Hypotheses 73 (2009) 438–440


    Jefferson Adams
    Higher Rates of Peripheral Neuropathy in People with Celiac Disease and Inflammatory Bowel Disease
    Celiac.com 07/17/2012 - To follow up on reported associations between celiac disease and peripheral neuropathy, a research team recently conducted a study of peripheral neuropathic symptoms in celiac disease and inflammatory bowel disease.
    T.C. Shen, B. Lebwohl, H. Verma, N. Kumta, C. Tennyson, S. Lewis, E. Scherl, A. Swaminath, K.M. Capiak, D. DiGiacomo, B.P. Bosworth, T.H. Brannagan 3rd, and P.H. Green. They are affiliated with the Department of Medicine, Division of Digestive and Liver Diseases at Columbia University Medical Center in New York, NY.
    For their study, the team recruited patients celiac disease and/or inflammatory bowel disease from the gastroenterology clinics at a medical center and local support groups. The team recruited control subjects without celiac disease or inflammatory bowel disease from the staff of the medical center, and from relatives and attendees at support groups.
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    The team found that 38.9% of participants with celiac disease and 38.7% in the inflammatory bowel disease group (P = 0.97) met criteria for peripheral neuropathy compared with 20.5% in the control group (P < 0.001).
    Using multiple logistic regression, the researchers found that those with celiac disease had higher odds of peripheral neuropathy (odds ratio, 2.51; 95% confidence interval, 1.82-3.47), adjusted for age, gender, diabetes, vitamin B12 deficiency, and cancer history; as did those with inflammatory bowel disease (odds ratio, 2.78; 95% confidence interval, 1.85-4.18).
    The results showed that people with celiac disease and/or inflammatory bowel disease
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    Source:
    J Clin Neuromuscul Dis. 2012 Mar;13(3):137-45.

    Jefferson Adams
    Celiac.com 10/27/2014 - There have been a few reports tying cortical myoclonus with ataxia to celiac disease. Such reports also suggest that the former is unresponsive to a gluten-free diet.
    A team of researchers recently set out to determine if there is any significant connection between the two conditions. The research team included Ptolemaios G. Sarrigiannis, Nigel Hoggard, Daniel Aeschlimann, David S. Sanders, Richard A. Grünewald, Zoe C. Unwin, and Marios Hadjivassiliou.
    They are variously associated with the Departments of Gastroenterology, Neurology, Neurophysiology and Neuroradiology at Royal Hallamshire Hospital, in Sheffield, UK, and with the College of Biomedical and Life Sciences at Cardiff University in Cardiff, UK.
    The team presented detailed electro-clinical characteristics of a new syndrome of progressive cortical hyperexcitability with ataxia and refractory celiac disease. Regular follow ups of over 600 patients with neurological manifestations due to gluten sensitivity revealed 9 patients with this syndrome.
    They found that all nine patients, six men and three women, experienced asymmetrical irregular myoclonus involving one or more limbs and sometimes face. This was often stimulus sensitive and became more widespread over time. Three patients had a history of Jacksonian march, and five had at least one secondarily generalized seizure. Electrophysiology showed evidence of cortical myoclonus. Three showed a phenotype of epilepsia partialis continua at onset.
    All patients showed clinical, imaging and/or pathological evidence of cerebellar involvement. All patients followed a strict gluten-free diet, and most successfully eliminated gluten-related antibodies. However, all patients still showed evidence of enteropathy, suggests that refractory celiac disease is to blame.
    During the study, two patients died from enteropathy-associated lymphoma and one from status epilepticus. Five patients were treated with mycophenolate and one in addition with rituximab and IV immunoglobulins. These patients showed improvement of ataxia and enteropathy, but continued to suffer the effects of myoclonus.
    These results indicate that myoclonus ataxia might be the most common neurological manifestation of refractory celiac disease.
    The clinical involvement, apart from ataxia, covers the whole clinical spectrum of cortical myoclonus.
    Source:
    Cerebellum & Ataxias 2014, 1:11. doi:10.1186/2053-8871-1-11

    Jefferson Adams
    What's the Best Way to Treat Immune-mediated Cerebellar Ataxias?
    Celiac.com 12/28/2015 - Immune-mediated cerebellar ataxias include gluten ataxia, paraneoplastic cerebellar degeneration, GAD antibody associated cerebellar ataxia, and Hashimoto's encephalopathy.
    Despite the identification of an increasing number of immune-mediated cerebellar ataxias, there is no proposed standardized therapy.
    Recently, a research team set out to develop guidelines for treatment of immune-mediated cerebellar ataxias.
    The research team included H. Mitoma, M. Hadjivassiliou, and J. Honnorat. They are variously associated with the Department of Medical Education at Tokyo Medical University in Tokyo, Japan; the Academic Department of Neurosciences at Royal Hallamshire Hospital, Sheffield, UK; the University Lyon 1; INSERM, UMR-S1028, CNRS, UMR-5292, Lyon Neuroscience Research Center, Neuro-Oncology and Neuro-Inflammation Team, 7; and the National Reference Centre for Paraneoplastic Neurological Diseases, Hospices Civils de Lyon, Hôpital neurologique in Bron, France.
    For their study, the team evaluated the efficacies of immunotherapies in reported cases using a common scale of daily activity.
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    Source:
    Cerebellum Ataxias. 2015 Nov 10;2:14. doi: 10.1186/s40673-015-0034-y. eCollection 2015.

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