Muscle MRI of the Upper Extremity in the Myotonic Dystrophy Type 1

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Abstract

Background: The purpose of this study was to explore the relation between muscle MRI findings and weakness of the upper extremity muscles in patients with myotonic dystrophy type 1 (DM1). Methods: Nineteen DM1 patients from 15 families were enrolled in this study. Muscle weakness was evaluated using the modified Medical Research Council scale. Subjects also underwent a genetic study and muscle MRI of the upper extremities. Results: In patients with DM1, the flexor digitorum profundus (FDP), flexor pollicis longus, flexor digitorum superficialis (FDS), extensor pollicis, abductor pollicis longus (APL), lateral head of triceps brachii and infraspinatus (INF) muscles were frequently and severely affected. Muscle strength was significantly correlated with the severity of muscle MRI findings in the FDP, short head of biceps brachii (SBB), and medial head of triceps brachii muscles. Disease duration was correlated significantly with MRI findings in the FDP, FDS, long head of biceps brachii, INF, APL, and SBB muscles. Unexpectedly, the degree of trinucleotide expansion of myotonin protein kinase was not correlated with muscle MRI findings. Conclusion: Muscle MRI of the upper extremity is useful to detect affected muscles in DM1 patients.

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Author and article information

Journal

Journal ID (publisher-id): ENE

Journal ID (nlm-ta): Eur Neurol

Journal ID (doi): 10.1159/issn.0014-3022

Title: European Neurology

Publisher: S. Karger AG

ISSN (Print): 0014-3022

ISSN (Electronic): 1421-9913

Publication date Subscription-year: 2016

Publication date (Print): August 2016

Publication date (Electronic): 29 July 2016

Volume: 76

Issue: 1-2

Pages: 87-94

Affiliations

^aSecond Department of Internal Medicine, ^bDepartment of Radiology, Faculty of Medical Sciences, and ^cTranslational Research Center, University of Fukui, Fukui, ^dDepartment of Neurology, Fujita Health University, Aichi, ^eDepartment of Neurology, Kagoshima University School of Medicine, Kagoshima, and ^fBrain Attack Ota Memorial Hospital, Hiroshima, Japan

Author notes

*Tadanori Hamano, MD, PhD, Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, 23-3 Matsuokashimoaizuki, Eiheiji-cho, Yoshida-gun, Fukui 910-1193 (Japan), E-Mail hamano@u-fukui.ac.jp

Article

Publisher ID: 448328 Other ID: Eur Neurol 2016;76:87-94

DOI: 10.1159/000448328

PubMed ID: 27466802

SO-VID: 8ac93128-e163-4836-8cc7-9ebb484eef25

License:

Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

History

Date received : 09 February 2016

Date accepted : 08 July 2016

Page count

Figures: 4, Tables: 2, References: 23, Pages: 8

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