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Neurological manifestations of coeliac disease

The association between progressive myoclonic encephalopathy (Ramsay Hunt syndrome; progressive myoclonic ataxia) was recently the subject of a clinicopathological report in this Journal 1 and of a review by Bhatia et al .2 A notable feature of these cases was the rapidly progressive nature of the encephalopathy. Most patients were unable to walk or died within two years of the onset of their neurological symptoms. The patient that we report is therefore remarkable in that her neurological illness began 13 years ago. Progressive myoclonic encephalopathy associated with coeliac disease may take a more benign course and we suggest that the condition should be considered in all patients with cerebellar ataxia, particularly if there is myoclonus.

The patient, now aged 64, presented …