Clinical, radiological, neurophysiological, and neuropathological characteristics of gluten ataxia

摘要: Summary Background Ataxia is the commonest neurological manifestation of coeliac disease. Some individuals with genetic susceptibility to disease have serological evidence gluten sensitivity without overt gastrointestinal symptoms or small-bowel inflammation. The sole in such patients may be ataxia. We describe clinical, radiological, and neurophysiological features this disorder. Methods Patients ataxia attending neurology outpatient clinics at Royal Hallamshire Hospital, Sheffield, UK, were screened for as shown by titre antibody gliadin. Those other causes excluded. carried out neurophysiological, neuroradiological, and, two cases, neuropathological examinations. Findings 28 identified. All had gait most limb more severe longer duration. No patient tremor extrapyramidal features. 19 showed some form peripheral neuropathy on examination. 16 no symptoms. Distal duodenal biopsy lymphocytic infiltration patients, changes compatible 11. Six cerebellar atrophy magnetic-resonance imaging. Necropsy was done who died; there cerebellum, damage posterior columns spinal cord, sparse nerves. Interpretation Gluten an important cause apparently idiopathic progressive. a result immunological propose term