Muscle magnetic resonance imaging in spinal muscular atrophy type 3: Selective and progressive involvement

ABSTRACT Introduction 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). Methods Twenty‐five patients with genetically confirmed SMA3b underwent MRI on a 1.5‐Tesl...

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Published inMuscle & nerve Vol. 55; no. 5; pp. 651 - 656
Main Authors Durmus, Hacer, Yilmaz, Ravza, Gulsen‐Parman, Yesim, Oflazer‐Serdaroglu, Piraye, Cuttini, Marina, Dursun, Memduh, Deymeer, Feza
Format Journal Article
LanguageEnglish
Published United States Wiley Subscription Services, Inc 01.05.2017
Subjects
Adolescent
Adult
Disease Progression
Female
Humans
magnetic resonance imaging
Magnetic Resonance Imaging - methods
Male
Middle Aged
MRI
muscle
Muscle, Skeletal - diagnostic imaging
Muscle, Skeletal - pathology
Muscular Atrophy - diagnostic imaging
Muscular Atrophy - pathology
natural history
NMR
Nuclear magnetic resonance
Shoulder
SMA
Spinal Muscular Atrophies of Childhood - diagnostic imaging
Spinal Muscular Atrophies of Childhood - pathology
spinal muscular atrophy
Thigh - diagnostic imaging
Thigh - pathology
Young Adult
magnetic resonance imaging
spinal muscular atrophy
MRI
natural history
SMA
muscle
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Summary:ABSTRACT Introduction 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). Methods Twenty‐five patients with genetically confirmed SMA3b underwent MRI on a 1.5‐Tesla MR scanner. Results 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. Conclusions 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
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ISSN:0148-639X
1097-4598
DOI:10.1002/mus.25385