Coeliac disease is the prototypical gluten-sensitive disease. Clinico-pathological features heal on a gluten-free diet and relapse when gluten is reintroduced. An immunopathology is suspected. A number of neurological syndromes may be associated with coeliac disease but it is unclear whether these are directly or indirectly caused by gluten ingestion. It has been proposed that idiopathic ataxias and central nervous system white matter disease are gluten-sensitive syndromes. This is an exciting hypothesis because it offers new therapeutic possibilities including simple exclusion diets. However, interpretation is difficult because occult sub-clinical coeliac disease occurs commonly and background prevalence needs to be accounted for in population-based studies. This review will attempt to summarize the pertinent literature on this fascinating topic.