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Myoclonus ataxia and refractory coeliac disease

Overview of attention for article published in Cerebellum & Ataxias, September 2014
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Myoclonus ataxia and refractory coeliac disease
Published in
Cerebellum & Ataxias, September 2014
DOI 10.1186/2053-8871-1-11
Pubmed ID

Ptolemaios G Sarrigiannis, Nigel Hoggard, Daniel Aeschlimann, David S Sanders, Richard A Grünewald, Zoe C Unwin, Marios Hadjivassiliou


Cortical myoclonus with ataxia has only rarely been reported in association with Coeliac Disease (CD). Such reports also suggested that it is unresponsive to gluten-free diet. We present detailed electro-clinical characteristics of a new syndrome of progressive cortical hyperexcitability with ataxia and refractory CD. At our gluten/neurology clinic we have assessed and regularly follow up over 600 patients with neurological manifestations due to gluten sensitivity. We have identified 9 patients with this syndrome. All 9 patients (6 male, 3 female) 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 generalised seizure. Electrophysiology showed evidence of cortical myoclonus. Three had a phenotype of epilepsia partialis continua at onset. There was clinical, imaging and/or pathological evidence of cerebellar involvement in all cases. All patients adhered to a strict gluten-free diet with elimination of gluten-related antibodies in most. However, there was still evidence of enteropathy in all, suggestive of refractory celiac disease. Two 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. Their ataxia and enteropathy improved but myoclonus remained the most disabling feature of their illness. This syndrome may well be the commonest neurological manifestation of refractory CD. The clinical involvement, apart from ataxia, covers the whole clinical spectrum of cortical myoclonus.

Mendeley readers

The data shown below were compiled from readership statistics for 43 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Indonesia 1 2%
Unknown 42 98%

Demographic breakdown

Readers by professional status Count As %
Researcher 9 21%
Student > Master 6 14%
Student > Ph. D. Student 6 14%
Student > Postgraduate 4 9%
Student > Doctoral Student 3 7%
Other 4 9%
Unknown 11 26%
Readers by discipline Count As %
Medicine and Dentistry 16 37%
Agricultural and Biological Sciences 3 7%
Neuroscience 3 7%
Nursing and Health Professions 2 5%
Business, Management and Accounting 1 2%
Other 4 9%
Unknown 14 33%

Attention Score in Context

This research output has an Altmetric Attention Score of 1. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 21 June 2016.
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