Research Project

Principal researchers: Prof Marios Hadjivassiliou, Department of Neuroscience, Sheffield Teaching Hospitals NHS Trusts (UK) 

Ataxia UK, Coeliac UK and Neurocare (part of Sheffield Hospitals Charity) and the Greaves and Withey Foundation have awarded funding to Professor Marios Hadjivassiliou, Director of the Sheffield Ataxia Centre, Sheffield Teaching Hospitals NHS Trust, and colleagues, to help improve the diagnosis and management of gluten ataxia. This is a collaborative project involving other Neurology Centres with an interest in Ataxia across the UK, including the London and Oxford Ataxia Centres. 

Lay Summary  

Gluten ataxia is caused by sensitivity to gluten, which is found in wheat, barley and rye products. This sensitivity means that when people with gluten ataxia eat gluten, the body’s immune system produces antibodies. These antibodies can then attack the balance centre of the brain and cause the symptoms of ataxia. Gluten ataxia can be treated by a strict gluten-free diet, which has been shown to stop the progression and potentially improve ataxia symptoms if started early enough. Therefore, early diagnosis of gluten ataxia is extremely important.  

While some people with gluten ataxia will also have coeliac disease (inflammation of the small bowel causing abdominal pain, altered bowel habit, bloating and malabsorption), the majority do not, and will test negative in the widely available blood tests used to diagnose coeliac disease. Previous research by Professor Hadjivassiliou and colleagues showed that people with gluten ataxia have antibodies called tissue transglutaminase 6 (tTG6) and antigliadin antibodies present in their blood, and these can be tested in addition to the widely available coeliac blood tests to diagnose gluten ataxia. This testing is currently available in Sheffield under the NHS but not in other laboratories in the UK.  

In this study, the Sheffield Ataxia Centre will work with other neurologists throughout the UK who have clinics for people with ataxia (London, Oxford, Manchester, Romford). People who have had tests to rule out other types of ataxia and who still do not have a diagnosis of the cause of their ataxia will be referred to the Sheffield Ataxia Centre to be tested for gluten ataxia. This will include tTG6 and antigliadin antibody tests, a brain scan, and if positive for these antibodies a gut biopsy. Those diagnosed with gluten ataxia will be advised to follow a strict gluten-free diet. They will then be followed up after one year to assess the impact of the gluten free diet. If you are interested in being involved in the project (and you are attending one of the centres involved), speak to your neurologist about taking part.   

Scientific Summary  

Professor Hadjivassiliou and colleagues have identified a novel biomarker for gluten ataxia (TG6 antibodies). Testing for TG6 antibodies is currently available in Sheffield under the NHS but not in other laboratories in the UK. Most immunology labs in the UK offer only the widely available serological markers for classic coeliac disease (anti-transglutaminase 2 and/or endomysium antibodies). These antibodies alone will be negative in the majority of people with gluten ataxia. 

In this study, the Sheffield Ataxia Centre will work with other neurologists throughout the UK who have clinics for people with ataxia (London, Oxford, Manchester, Romford).  

They aim to test all those with idiopathic sporadic ataxia referred to the Sheffield Ataxia Centre by other centres, using a battery of serological tests for gluten sensitivity. Those who test positive in one or more of these tests will be offered duodenal biopsy and MR spectroscopy of the cerebellum, following which they will be referred to the local dietetic services for advice regarding a gluten free diet. If they are willing to be followed up, then a further appointment will be made for re-evaluation after a year on a gluten free diet.  

Aside from the development of a diagnostic pathway for gluten ataxia, they envisage that at the end of the study they will also produce best practice guidelines for the management and follow up of people with gluten ataxia.  

For further information on this project see the Coeliac UK website.

For more support or information please contact:  
Ataxia UK, 12 Broadbent Close, London, N6 5JW 
Website: www.ataxia.org.uk.   
Helpline: 0800 995 6037 Tel: +44 (0)20 7582 1444   
Email: helpline@ataxia.org.uk.