title: Monomelic muscle atrophy.
abstract: Two patients with slowly progressive muscle atrophy limited to only one leg are reported. They had pes equinovarus deformity and muscle weakness in the affected leg but no symptom in the other limbs. Muscle biopsies from the affected leg showed dystrophic changes consisting of variation in muscle fiber size, endomysial fibrosis, and necrotic and regenerating fibers. Dystrophin was normally expressed at the surface membrane of the muscle fibers. These two patients possibly had a variant of distal muscular dystrophy, though a neural influence could not be completely excluded.
authors.affiliation: National Institute of Neuroscience, NCNP, Tokyo, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Takemitsu
authors.forename: K
authors.initials: K
authors.lastname: Murayama
authors.forename: T
authors.initials: T
authors.lastname: Saga
authors.forename: N
authors.initials: N
authors.lastname: Michihiro
authors.forename: H
authors.initials: H
authors.lastname: Shiihara
authors.forename: M
authors.initials: M
authors.lastname: Kimizuka
authors.forename: I
authors.initials: I
authors.lastname: Nonaka
mesh_terms: D001706:Biopsy; D002462:Cell Membrane / Q000473:pathology; D002648:Child; D016189:Dystrophin / Q000032:analysis; D005260:Female; D005355:Fibrosis; D006801:Humans; D015151:Immunoblotting; D008297:Male; D009132:Muscles / Q000473:pathology*; D009133:Muscular Atrophy / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology; D009336:Necrosis; D012038:Regeneration; D014057:Tomography, X-Ray Computed
chemical_list: D016189:Dystrophin