Whole‐Body MRI in Limb Girdle Muscular Dystrophy Type R1/2A: Correlation With Clinical Scores

Introduction/Aim The most common limb girdle muscular dystrophy (LGMD) worldwide is LGMD type R1 (LGMDR1). The aim of this study was to correlate the MRI findings with functional scores and to describe the whole‐body MRI (WBMRI) pattern in a LGMDR1 Brazilian cohort. Methods LGMDR1 patients under fol...

Full description

Saved in:
Bibliographic Details
Published in:Muscle & nerve 2022-10, Vol.66 (4), p.471-478
Main Authors: Aivazoglou, Laís U., Guimarães, Julio B., Costa, Maria Alice F., Aihara, André Yui, Cardoso, Fabiano N., Pinto, Wladimir B. V. De R., Souza, Paulo Victor S., Silva, André M. S., Zanoteli, Edmar, Oliveira, Acary S. B., Carvalho, Alzira A. S., Fernandes, Artur Da R. C.
Format: Article
Language:English
Subjects:
CAPN3
Clinical trials
Dystrophy
Edema
Evaluation
Functional magnetic resonance imaging
Infiltration
LGMD
limb‐girdle muscular dystrophy
Magnetic resonance imaging
MRI
muscle dystrophy
Muscles
Muscular dystrophy
Patients
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Introduction/Aim The most common limb girdle muscular dystrophy (LGMD) worldwide is LGMD type R1 (LGMDR1). The aim of this study was to correlate the MRI findings with functional scores and to describe the whole‐body MRI (WBMRI) pattern in a LGMDR1 Brazilian cohort. Methods LGMDR1 patients under follow‐up in three centers were referred for the study. Clinical data were collected and a functional evaluation was performed, consisting of Gardner‐Medwin and Walton (GMW) and Brooke scales. All patients underwent a WBMRI study (1.5T) with axial T1 and STIR images. Fifty‐one muscles were semiquantitatively assessed regarding fatty infiltration and muscle edema. Results The study group consisted of 18 patients. The highest fatty infiltration scores involved the serratus anterior, biceps femoris long head, adductor magnus, and lumbar erector spinae. There was a latero‐medial and caudo‐cranial descending gradient of involvement of the paravertebral muscles, with erector spinae being significantly more affected than the transversospinalis muscles (p 
ISSN:0148-639X
1097-4598
DOI:10.1002/mus.27686