Myoclonus ataxia and XXXX .

@entity1 @entity410 @entity66 @entity1593 @entity706 @entity2045 @entity16 @entity10895 @entity7017 @entity6051 @entity159 @entity2055 @entity71255
BACKGROUND: Cortical @entity2045 with @entity2055 has only rarely been reported in association with @entity66 ( @entity66 ). Such reports also suggested that it is unresponsive to gluten-free diet. We present detailed electro-clinical characteristics of a new @entity71255 with @entity2055 . At our gluten/neurology clinic we have assessed and regularly follow up over 600 @entity1 with @entity16 due to @entity1593 . We have identified 9 @entity1 with this syndrome. RESULTS: All 9 @entity1 (6 male, 3 female) experienced asymmetrical irregular @entity2045 involving one or more limbs and sometimes face. This was often stimulus sensitive and became more widespread over time. Three @entity1 had a history of Jacksonian march and five had at least one secondarily generalised @entity410 . Electrophysiology showed evidence of cortical @entity2045 . Three had a phenotype of @entity10895 partialis @entity10895 at onset. There was clinical, imaging and/or pathological evidence of cerebellar involvement in all cases. All @entity1 adhered to a strict gluten-free diet with elimination of gluten-related antibodies in most. However, there was still evidence of @entity7017 in all, suggestive of @entity1593 . Two died from @entity706 and one from @entity159 . Five @entity1 were treated with @entity6051 and one in addition with rituximab and IV immunoglobulins. Their @entity2055 and @entity7017 improved but @entity2045 remained the most disabling feature of their illness. CONCLUSIONS: This syndrome may well be the commonest neurological manifestation of refractory @entity66 . The clinical involvement, apart from @entity2055 , covers the whole clinical spectrum of cortical @entity2045 .
