Coeliac disease presenting with chorea

Chorea can be genetic or acquired, and often leads to a challenging diagnostic conundrum. In a significant proportion, there is no specific identifiable cause. Chorea is a rare but potentially reversible neurological manifestation of coeliac disease, usually presenting insidiously and often presumed to be associated with typical gastrointestinal symptoms. We report a patient with rapidly progressive generalised chorea, but without preceding gastrointestinal symptoms, who was subsequently diagnosed with coeliac disease. A gluten-free diet resulted in complete resolution of the chorea.