CLINICAL, RADIOLOGICAL, NEUROPHYSIOLOGICAL, AND NEUROPATHOLOGICAL CHARACTERISTICS OF GLUTEN ATAXIA

Background Ataxia is the commonest neurological manifestation of coeli ac disease. Some individuals with genetic susceptibility to the diseas e have serological evidence of gluten sensitivity without overt gastro intestinal symptoms or evidence of small-bower inflammation. The sole manifestation of disease in such patients may be ataxia. We describe t he clinical, radiological, and neurophysiological features of this dis order. Methods Patients with ataxia attending the neurology outpatient clinics at the royal Hallamshire Hospital, Sheffield, UK, were screen ed for gluten sensitivity as shown by the titre of antibody to gliadin . Those with other causes of ataxia were excluded. we carried out clin ical, neurophysiological, neuroradiological, and, in two cases, neurop athological examinations. Findings 28 patients with gluten ataxia were identified. All had gait ataxia and most had limb ataxia. Those with more severe gait ataxia had longer disease duration. No patient had tr emor or other extrapyramidal features. 19 patients showed some form of peripheral neuropathy on neurophysiological examination. 16 patients had no gastrointestinal symptoms. Distal duodenal biopsy showed lympho cytic infiltration in two patients, and changes compatible with coelia c disease in 11. Six patients had evidence of cerebellar atrophy on ma gnetic-resonance imaging. Necropsy was done on two patients who died; there was lymphocytic infiltration of the cerebellum, damage to the po sterior columns of the spinal cord, and sparse infiltration of the per ipheral nerves. Interpretation Gluten sensitivity is an important caus e of apparently idiopathic ataxia and may be progressive. The ataxia i s a result of immunological damage to the cerebellum, to the posterior columns of the spinal cord, and to peripheral nerves. We propose the term gluten ataxia to describe this disorder.