Myoclonus ataxia and refractory coeliac disease.

ABSTRACT: BACKGROUND: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. RESULTS: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

SUBMITTER: Sarrigiannis PG 

PROVIDER: S-EPMC4552176 | biostudies-literature | 2014

REPOSITORIES: biostudies-literature