Nervenheilkunde 2019; 38(05): 304
DOI: 10.1055/s-0039-1685094
Poster
Muskeldystrophien und Myotone Dystrophien
Georg Thieme Verlag KG Stuttgart · New York

Longitudinal upper limb muscle MRI in dysferlinopathy: examining the relationship between semi quantitative MRI and physiotherapy outcome measures

K Storch
1   The John Walton Muscular Dystrophy Research Centre, Institute of Genetic Medicine, Newcastle, Vereinigtes Königreich
2   Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Abteilung für Neuropädiatrie, Dresden, Deutschland
,
R Fernández-Torrón
1   The John Walton Muscular Dystrophy Research Centre, Institute of Genetic Medicine, Newcastle, Vereinigtes Königreich
3   Donostia University Hospital, Neuromuscular Area. Biodonostia Health Research Institute. Neurology Service, Donostia-San Sebastian, Spanien
,
MK James
1   The John Walton Muscular Dystrophy Research Centre, Institute of Genetic Medicine, Newcastle, Vereinigtes Königreich
,
AG Mayhew
1   The John Walton Muscular Dystrophy Research Centre, Institute of Genetic Medicine, Newcastle, Vereinigtes Königreich
,
J Díaz-Manera
4   Hospital de la Santa Creu i Sant Pau, Neuromuscular Disorders Unit, Neurology Department, Barcelona, Spanien
,
AM Blamire
5   Newcastle University, Magnetic Resonance Centre, Institute for Cellular Medicine, Newcastle, Vereinigtes Königreich
,
PG Pierre
6   Pitié-Salpêtrière University Hospital, AIM & CEA NMR Laboratory, Institute of Myology, Paris, Frankreich
,
H Hilsden
1   The John Walton Muscular Dystrophy Research Centre, Institute of Genetic Medicine, Newcastle, Vereinigtes Königreich
,
T Stojkovic
7   Boulevard de l'Hôpital, Institut de Myologie, Paris, Frankreich
,
MC Walter
8   Ludwig-Maximilians-University of Munich, Friedrich-Baur-Institute, Dept. of Neurology, München, Deutschland
,
S Krause
8   Ludwig-Maximilians-University of Munich, Friedrich-Baur-Institute, Dept. of Neurology, München, Deutschland
,
EM Coppenrath
9   Ludwig-Maximilians-University Munich, Department of Clinical Radiology, München, Deutschland
,
A Peduto
10   University of Sydney, Department of Radiology, Westmead Hosptial, Faculty of Health Sciences, Sydney, Australien
,
KJ Jones
11   Children's Hospital at Westmead, Institute for Neuroscience and Muscle Research, Sydney, Australien
,
AM Sawyer
12   Stanford University School of Medicine, Lucas Centre for Imaging, Stanford, Deutschland
,
C Tesi Rocha
13   Stanford University School of Medicine, Department of Neurology and Neurological Sciences, Stanford, Deutschland
,
JW Day
14   Stanford University School of Medicine, Department of Neurology and Neurological Sciences, Stanford, Vereinigte Staaten
,
K Bushby
1   The John Walton Muscular Dystrophy Research Centre, Institute of Genetic Medicine, Newcastle, Vereinigtes Königreich
,
V Straub
1   The John Walton Muscular Dystrophy Research Centre, Institute of Genetic Medicine, Newcastle, Vereinigtes Königreich
› Author Affiliations
Further Information

Publication History

Publication Date:
06 May 2019 (online)

 
 

    Methods:

    The Clinical Outcome Study for Dysferlinopathy is a multi-centre natural history study in dysferlinopathy patients. 203 patients (11 to 86 years old) were enrolled. Patients underwent physiotherapy, medical and MRI assessments. 74 patients underwent upper limb muscle MRI at baseline and 61 patients had upper limb muscle MRI at year 3. Muscles were scored on axial T1-weighted sequences with the semi quantitative Mercuri visual scale modified by Fisher. Physiotherapy assessments included muscle strength and functional ability evaluations. Change between baseline and year 3 muscle MRI was assessed using Wilcoxon“s Signed Rank Tests and statistical significance was set at p < 0.05.

    Results:

    The subscapularis (80.8%), latissimus dorsi (82.6%), infraspinatus (73.8%) and supraspinatus (72.8%) were the most affected scapular muscles at baseline. The biceps brachii (57.1%) and the anterior muscles (53.8%) were the most affected muscles from the arm and forearm at baseline. Cranial muscles and cervical did not show longitudinal changes apart for tongue and cervical paraspinal muscles. Only the brachialis, triceps, and the posterior muscles from the forearm did not show longitudinal changes.

    Conclusion:

    The subscapularis muscle and the latissimus were the most affected muscles at baseline. Only the tongue and cervical muscles showed changes at year 3 but nearly all scapular and arm muscles showed longitudinal changes.


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