title: Myopathy associated with gluten sensitivity.
abstract: Ataxia and peripheral neuropathy are the most common neurological manifestations of gluten sensitivity. Myopathy is a less common and poorly characterized additional neurological manifestation of gluten sensitivity. We present our experience with 13 patients who presented with symptoms and signs suggestive of a myopathy and in whom investigation led to the diagnosis of gluten sensitivity. Three of these patients had a neuropathy with or without ataxia in addition to the myopathy. The mean age at onset of the myopathic symptoms was 54 years. Ten patients had neurophysiological evidence of myopathy. Inflammatory myopathy was the most common finding on neuropathological examination. One patient had basophilic rimmed vacuoles suggestive of inclusion-body myositis. Six patients received immunosuppressive treatment in addition to starting on a gluten-free diet; five improved and one remained unchanged. Among seven patients not on immunosuppressive treatment, four showed clinical improvement of the myopathy with a gluten-free diet. The improvement was also associated with reduction or normalization of serum creatine kinase level. The myopathy progressed in one patient who refused the gluten-free diet. Myopathy may be another manifestation of gluten sensitivity and is likely to have an immune-mediated pathogenesis. A gluten-free diet may be a useful therapeutic intervention.
authors.affiliation: Department of Neurology, The Royal Hallamshire Hospital, Glossop Road, Sheffield S10 2JF, UK. m.hadjivassiliou@sheffield.ac.uk
authors.forename: Marios
authors.initials: M
authors.lastname: Hadjivassiliou
authors.forename: Arup K
authors.initials: AK
authors.lastname: Chattopadhyay
authors.forename: Richard A
authors.initials: RA
authors.lastname: Grünewald
authors.forename: John A
authors.initials: JA
authors.lastname: Jarratt
authors.forename: Rosalind H
authors.initials: RH
authors.lastname: Kandler
authors.forename: D G
authors.initials: DG
authors.lastname: Rao
authors.forename: D S
authors.initials: DS
authors.lastname: Sanders
authors.forename: S B
authors.initials: SB
authors.lastname: Wharton
authors.forename: G A B
authors.initials: GA
authors.lastname: Davies-Jones
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D001259:Ataxia / Q000209:etiology / Q000503:physiopathology; D002446:Celiac Disease / Q000150:complications* / Q000503:physiopathology; D015331:Cohort Studies; D003402:Creatine Kinase / Q000097:blood; D004576:Electromyography; D005260:Female; D005526:Food, Formulated; D005983:Glutens / Q000009:adverse effects; D006801:Humans; D007167:Immunotherapy; D008297:Male; D008875:Middle Aged; D018485:Muscle Fibers, Skeletal / Q000473:pathology; D018482:Muscle, Skeletal / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology*; D009135:Muscular Diseases / Q000209:etiology* / Q000503:physiopathology* / Q000628:therapy; D018979:Myositis, Inclusion Body / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D009431:Neural Conduction / Q000502:physiology; D010523:Peripheral Nervous System Diseases / Q000209:etiology / Q000503:physiopathology; D016896:Treatment Outcome
chemical_list: D005983:Glutens; D003402:Creatine Kinase