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

K Storch; R Fernández-Torrón; MK James; AG Mayhew; J Díaz-Manera; AM Blamire; PG Pierre; H Hilsden; T Stojkovic; MC Walter; S Krause; EM Coppenrath; A Peduto; KJ Jones; AM Sawyer; C Tesi Rocha; JW Day; K Bushby; V Straub

doi:10.1055/s-0039-1685094

Nervenheilkunde, Table of Contents

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

Authors

K Storch

¹The John Walton Muscular Dystrophy Research Centre, Institute of Genetic Medicine, Newcastle, Vereinigtes Königreich

²Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Abteilung für Neuropädiatrie, Dresden, Deutschland
R Fernández-Torrón

¹The John Walton Muscular Dystrophy Research Centre, Institute of Genetic Medicine, Newcastle, Vereinigtes Königreich

³Donostia University Hospital, Neuromuscular Area. Biodonostia Health Research Institute. Neurology Service, Donostia-San Sebastian, Spanien
MK James

¹The John Walton Muscular Dystrophy Research Centre, Institute of Genetic Medicine, Newcastle, Vereinigtes Königreich
AG Mayhew

¹The John Walton Muscular Dystrophy Research Centre, Institute of Genetic Medicine, Newcastle, Vereinigtes Königreich
J Díaz-Manera

⁴Hospital 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

⁶Pitié-Salpêtrière University Hospital, AIM & CEA NMR Laboratory, Institute of Myology, Paris, Frankreich
H Hilsden

¹The John Walton Muscular Dystrophy Research Centre, Institute of Genetic Medicine, Newcastle, Vereinigtes Königreich
T Stojkovic

⁷Boulevard de l'Hôpital, Institut de Myologie, Paris, Frankreich
MC Walter

⁸Ludwig-Maximilians-University of Munich, Friedrich-Baur-Institute, Dept. of Neurology, München, Deutschland
S Krause

⁸Ludwig-Maximilians-University of Munich, Friedrich-Baur-Institute, Dept. of Neurology, München, Deutschland
EM Coppenrath

⁹Ludwig-Maximilians-University Munich, Department of Clinical Radiology, München, Deutschland
A Peduto

¹⁰University of Sydney, Department of Radiology, Westmead Hosptial, Faculty of Health Sciences, Sydney, Australien
KJ Jones

¹¹Children's Hospital at Westmead, Institute for Neuroscience and Muscle Research, Sydney, Australien
AM Sawyer

¹²Stanford University School of Medicine, Lucas Centre for Imaging, Stanford, Deutschland
C Tesi Rocha

¹³Stanford University School of Medicine, Department of Neurology and Neurological Sciences, Stanford, Deutschland
JW Day

¹⁴Stanford University School of Medicine, Department of Neurology and Neurological Sciences, Stanford, Vereinigte Staaten
K Bushby

¹The John Walton Muscular Dystrophy Research Centre, Institute of Genetic Medicine, Newcastle, Vereinigtes Königreich
V Straub

¹The John Walton Muscular Dystrophy Research Centre, Institute of Genetic Medicine, Newcastle, Vereinigtes Königreich

Abstract

Full Text

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.