title: Muscle magnetic resonance imaging in spinal muscular atrophy type 3: Selective and progressive involvement.
abstract: In this study we sought to identify magnetic resonance imaging (MRI) signs of selective muscle involvement and disease progression in patients with spinal muscular atrophy type 3b (SMA3b).
  
  
  
  Twenty-five patients with genetically confirmed SMA3b underwent MRI on a 1.5-Tesla MR scanner.
  
  
  
  MRI showed significantly more severe involvement of the iliopsoas than of the gluteus maximus muscles, and more severe involvement of the triceps brachii than of the biceps brachii muscles. The quadriceps femoris muscles were severely involved. The deltoid, adductor longus, portions of the hamstrings, gracilis, sartorius, and rectus abdominis muscles were well preserved. We found a significant positive correlation between MRI changes and disease duration for gluteus maximus and triceps brachii. Follow-up MRIs of 4 patients showed disease progression.
  
  
  
  This study confirms the pattern of selective muscle involvement suggested by previous studies and further refines muscle MRI changes in SMA3b. Progressive muscle involvement is implicated. Muscle Nerve 55: 651-656, 2017.
authors.affiliation: Department of Neurology, Istanbul Faculty of Medicine, Istanbul University, Millet Caddesi, Capa, 34390, Istanbul, Turkey.
authors.forename: Hacer
authors.initials: H
authors.lastname: Durmus
authors.affiliation: Department of Radiology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
authors.forename: Ravza
authors.initials: R
authors.lastname: Yilmaz
authors.affiliation: Department of Neurology, Istanbul Faculty of Medicine, Istanbul University, Millet Caddesi, Capa, 34390, Istanbul, Turkey.
authors.forename: Yesim
authors.initials: Y
authors.lastname: Gulsen-Parman
authors.affiliation: Department of Neurology, Istanbul Faculty of Medicine, Istanbul University, Millet Caddesi, Capa, 34390, Istanbul, Turkey.
authors.forename: Piraye
authors.initials: P
authors.lastname: Oflazer-Serdaroglu
authors.affiliation: Research Unit of Perinatal Epidemiology, Pediatric Hospital Bambino Gesù, Rome, Italy.
authors.forename: Marina
authors.initials: M
authors.lastname: Cuttini
authors.affiliation: Department of Radiology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
authors.forename: Memduh
authors.initials: M
authors.lastname: Dursun
authors.affiliation: Department of Neurology, Istanbul Faculty of Medicine, Istanbul University, Millet Caddesi, Capa, 34390, Istanbul, Turkey.
authors.forename: Feza
authors.initials: F
authors.lastname: Deymeer
mesh_terms: D000293:Adolescent; D000328:Adult; D018450:Disease Progression; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000000981:diagnostic imaging* / Q000473:pathology; D009133:Muscular Atrophy / Q000000981:diagnostic imaging* / Q000473:pathology; D014897:Spinal Muscular Atrophies of Childhood / Q000000981:diagnostic imaging* / Q000473:pathology; D013848:Thigh / Q000000981:diagnostic imaging / Q000473:pathology; D055815:Young Adult
keywords: MRI; SMA; magnetic resonance imaging; muscle; natural history; spinal muscular atrophy