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MRI patterns of muscle involvement in type 2 and 3 spinal muscular atrophy patients.
- Source :
-
Journal of neurology [J Neurol] 2020 Apr; Vol. 267 (4), pp. 898-912. Date of Electronic Publication: 2019 Nov 27. - Publication Year :
- 2020
-
Abstract
- Only few studies have reported muscle involvement in spinal muscular atrophy using muscle MRI but this has not been systematically investigated in a large cohort of both pediatric and adult patients with type 2 and type 3 spinal muscular atrophy. The aim of the present study was to define possible patterns of muscle involvement on MRI, assessing both fatty replacement and muscle atrophy, in a cohort of type 2 and type 3 spinal muscular atrophy children and adults (age range 2-45 years), including both ambulant and non-ambulant patients. Muscle MRI protocol consisted in T1-weighted sequences acquired on axial plane covering the pelvis, the thigh, and the leg with contiguous slices. Each muscle was examined through its whole extension using a grading system that allows a semiquantitative evaluation of fatty infiltration. Thigh muscles were also grouped in anterior, posterior, and medial compartment for classification of global atrophy. The results showed a large variability in both type 2 and type 3 spinal muscular atrophy, with a various degree of proximal to distal gradient. Some muscles, such us the adductor longus and gracilis were always selectively spared. In all patients, the involvement was a combination of muscle atrophy and muscle infiltration. The variability observed may help to better understand both natural history and response to new treatments.
- Subjects :
- Adiposity physiology
Adolescent
Adult
Atrophy pathology
Child
Child, Preschool
Female
Humans
Magnetic Resonance Imaging
Male
Middle Aged
Muscle, Skeletal metabolism
Muscular Atrophy, Spinal metabolism
Prospective Studies
Spinal Muscular Atrophies of Childhood diagnostic imaging
Spinal Muscular Atrophies of Childhood metabolism
Spinal Muscular Atrophies of Childhood pathology
Young Adult
Muscle, Skeletal diagnostic imaging
Muscle, Skeletal pathology
Muscular Atrophy, Spinal diagnostic imaging
Muscular Atrophy, Spinal pathology
Subjects
Details
- Language :
- English
- ISSN :
- 1432-1459
- Volume :
- 267
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Journal of neurology
- Publication Type :
- Academic Journal
- Accession number :
- 31776722
- Full Text :
- https://doi.org/10.1007/s00415-019-09646-w