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Neuromuscular Ultrasound Francis O. Walker, MD Professor Department of Neurology Wake Forest University School of Medicine Winston-Salem, North Carolina Michael S. Cartwright, MD Assistant Professor Department of Neurology Wake Forest University School of Medicine Winston-Salem, North Carolina 1600 John F. Kennedy Blvd. Ste 1800 Philadelphia, PA 19103-2899 NEUROMUSCULAR ULTRASOUND ISBN: 978-1-4377-1527-9 Copyright © 2011 by Saunders, an imprint of Elsevier Inc. No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopying, recording, or any information storage and retrieval system, without per- mission in writing from the publisher. Details on how to seek permission, further information about the Pub- lisher’s permissions policies and our arrangements with organizations such as the Copyright Clearance Center and the Copyright Licensing Agency, can be found at our website: www.elsevier.com/permissions. This book and the individual contributions contained in it are protected under copyright by the Publisher (other than as may be noted herein). Notices Knowledge and best practice in this field are constantly changing. As new research and experience broaden our understanding, changes in research methods, professional practices, or medical treatment may become necessary. Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using any information, methods, compounds, or experiments described herein. In using such information or methods they should be mindful of their own safety and the safety of others, including parties for whom they have a professional responsibility. With respect to any drug or pharmaceutical products identified, readers are advised to check the most current information provided (i) on procedures featured or (ii) by the manufacturer of each product to be administered, to verify the recommended dose or formula, the method and duration of administration, and contraindications. It is the responsibility of practitioners, relying on their own experience and knowledge of their patients, to make diagnoses, to determine dosages and the best treatment for each individual patient, and to take all appropriate safety precautions. To the fullest extent of the law, neither the Publisher nor the authors, contributors, or editors assume any liability for any injury and/or damage to persons or property as a matter of products liability, negligence or otherwise, or from any use or operation of any methods, products, instructions, or ideas contained in the material herein. Library of Congress Cataloging-in-Publication Data Neuromuscular ultrasound/[edited by] Francis O. Walker, Michael S. Cartwright. -- 1st ed. p. ; cm. Includes bibliographical references and index. ISBN 978-1-4377-1527-9 (hardcover : alk. paper) 1. Neuromuscular diseases--Ultrasonic imaging. I. Walker, Francis O. II. Cartwright, Michael S. [DNLM: 1. Neuromuscular Diseases--ultrasonography. WE 550] RC925.7.N48 2011 616.7’4407543--dc22 2011005935 Acquisitions Editor: Dan Pepper Developmental Editor: Jessica Pritchard Publishing Services Manager: Anne Altepeter Project Managers: Jessica Becher and Vijay Antony Raj Vincent Design Direction: Lou Forgione Marketing Manager: Cara Jespersen Printed in the United States of America Last digit is the print number: 9 8 7 6 5 4 3 2 1 Contributors Roy Beekman, MD, PhD Nens van Alfen, MD, PhD Department of Neurology Neurologist/Clinical Neurophysiologist Atrium Medical Center Department of Neurology & Clinical Neurophysiology Heerlen, The Netherlands Radboud University Nijmegen Medical Center Nijmegen, The Netherlands Andrea J. Boon, MBChB Assistant Professor Leo H. Visser, MD, PhD Department of Physical Medicine and Rehabilitation Department of Neurology Department of Neurology St. Elisabeth Hospital Mayo Clinic College of Medicine Tilburg, The Netherlands Rochester, Minnesota Francis O. Walker, MD Michael S. Cartwright, MD Professor Assistant Professor Department of Neurology Department of Neurology Wake Forest University School of Medicine Wake Forest University School of Medicine Winston-Salem, North Carolina Winston-Salem, North Carolina Robert S. Weller, MD C. Michael Harper, MD Professor Professor of Neurology Department of Anesthesiology Vice Chair Wake Forest University School of Medicine Department of Neurology Winston-Salem, North Carolina Mayo Clinic College of Medicine Rochester, Minnesota Craig Mitchell Zaidman, MD Assistant Professor Lisa D. Hobson-Webb, MD Department of Neurology Assistant Professor Division of Child Neurology Department of Medicine Washington University Division of Neurology St. Louis Children’s Hospital Duke University Medical Center Barnes Jewish Hospital Durham, North Carolina St. Louis, Missouri Christopher Harker Hunt, MD Machiel J. Zwarts, MD, PhD Assistant Professor of Radiology Professor Department of Radiology Clinical Neurophysiology Mayo Clinic College of Medicine Radboud University Nijmegen Medical Center Rochester, Minnesota Nijmegen, The Netherlands; Professor Sigrid Pillen, MD, PhD Epilepsy Centre Kempenhaeghe Department of Neurology and Clinical Neurophysiology Heeze, The Netherlands Department of Pediatrics Radboud University Nijmegen Medical Center Nijmegen, The Netherlands Steven J. Shook, MD Staff Neuromuscular Center, Neurological Institute Cleveland Clinic Cleveland, Ohio iii This page intentionally left blank Preface “All human science is but the increment of the power of things do in clinical medicine, with the help of an enthusias- the eye, and all human art is the increment of the power tic mentor, Dr. William McKinney, a former president of the of the hand. Vision and manipulation, in their countless American Institute of Ultrasound in Medicine and a pioneer in indirect and transfigured forms, are the two cooperating carotid ultrasonography. He encouraged F.W. 25 years ago to factors in all intellectual progress.” follow up on studies by Heckmatt and colleagues3 demonstrat- John Fiske, 18991 ing the utility of ultrasonography in the evaluation of muscular dystrophy. Casual use of the technique verified its sensitivity Medicine is both an art and a science, and nowhere is this as clear for detecting chronic muscle disease, but because this alone as in the practice of electrodiagnostic medicine and in neuro- did little to add to clinical care, use of the technique was lim- muscular ultrasound. Both techniques involve reasoning based ited primarily to research.4 However, over the next 15 years on perceptual information (science) and motor skills (arts). with significant enhancement in available resolution, the tech- Both lend themselves to discovering pathology in individual nique became useful for studying nerves and the routine evalu- patients and mechanisms of nerve and muscle disease that affect ation of entrapment neuropathies. About 10 years ago, M.C. larger populations. In an earlier publication Fiske explained became involved in neuromuscular discovery with ultraso- the synergistic effects of art and science in more detail:2 nography.5 Since then, we have been joined by a number of “Science is but an extension of our ordinary sense percep- others in the United States and abroad, some who have had tions by the aid of reasoning, while art is but an extension experience predating ours. The encouragement these pioneers of our muscular system, of expressing our psychical states by have provided us by their unexpected discoveries and elegant means of motion. Hence it is said that each great step towards research has led us to put together this introductory textbook a knowledge of laws has facilitated men’s operation on things; of neuromuscular ultrasonography. We have tried to credit and each more successful operation on things, by its results, as many of these remarkable individuals as possible either by facilitated the discovery of other laws.…There is scarcely an inviting them to contribute chapters or by generous citations observation made now in science but what involves the use of of their work. We look forward to finding more colleagues instruments supplied by the arts; while there is scarcely an art- and collaborators in the future who will further advance this process but what involves some of the previsions of science.” promising field. The description is apt because both electromyography (EMG) and ultrasound, discoveries of the twentieth century, Francis O. Walker, MD, and Michael S. Cartwright, MD are hands-on techniques (arts) that lead to a better under- standing of science. Of course, the better one understands the science of these techniques (arts), the more enterprising References an individual becomes with them. This description of how real advances occur in the world is somewhat at odds with 1. Fiske J: The destiny of man viewed in light of his origin, the notion of hypothesis planned research, which some see as Boston, 1899, Houghton Mifflin (the Riverside Press a critical antecedent of meaningful discovery. This idea fails Cambridge), p 60. to capture the implicit real-time reasoning and motor skill 2. Fiske J: Outlines of cosmic philosophy based on the doctrines involved in most innovations, particularly in the incremental of evolution with criticisms on the positive philosophy, vol discovery that characterizes much of the technologic advances 2, Boston, 1891, Houghton Mifflin (the Riverside Press routinely available today. In fact, it is the real-time aspects of Cambridge), p 310. physiologic investigations, such as ultrasound and EMG, that 3. Heckmatt JZ, Dubowitz V, Leeman S: Detection of patho- make them so engaging for the practitioner. These techniques logical change in dystrophic muscle with B-scan ultrasound encourage problem solving by integrating complex findings imaging, Lancet 1:1389-1390, 1980. on interview, physical examination, electrodiagnostic testing, 4. Walker FO, Donorio PD, Harpold GJ, Ferrell WG: Sono- and real-time ultrasonography. The process of integration, or graphic imaging of muscle contraction and fasciculations: a more commonly failed integration, stimulates curiosity and correlation with electromyography, Muscle Nerve 13:33-39, enhances discovery in this field. 1990. This book is written for those who want to further develop 5. Cartwright MS, Wiesler ER, Caress JB, et al: High- the art and science of neuromuscular medicine with the use resolution ultrasound in the evaluation of carpal tunnel of ultrasonography. Our interests in this field began, as many syndrome, Neurology 58(Suppl 3):A67, 2002. v Acknowledgments Of course, multiple individuals have helped us gather the Pete Santago PhD, Wells Reynolds MD, Ruth Fredericks MD, experience needed to write this book, either by performing Brian Sires MD, L. Andrew Koman MD, Joan Shik Yuen PhD, studies, referring patients, or giving us advice or guidance. The and Randall Braddom MD. Also, Jamie Lambert, Kevin Bone- list is not all-inclusive, but we do want to mention Kara Eick- break, Scott Kuhn, Nancy Wilkins-Smith, Susan Reid, Jamie man MD, James Caress MD, Peter Donofrio MD, Gary Harpold Hutchinson, Ashley Gwynn, Leah Griffin, Kathleen D. Walker, MD, William Ferrell MD, Joseph Chipman MD, Lee Kennedy April Edwards, Vickie Higgins, Victoria Hunt RN, Christine MD, James Johnson PhD, Fred Kremkau PhD, Chuck Tegeler O’Neill, David White, Samantha DeMar, and Lawrence Mey- MD, Deborah Kirby MD, Kashyap Patel MD, Cecile Becker MD, ers. We would also like to thank our children, Maya Walker and Kelly Ybema MD, Eric Pitts MD, Martin Brown MD, Haewon Adam, Alex, and Emma Cartwright, who have patiently put up Shin MD, Waqas Sohail MD, Preet Chahal MD, Mary Lussier with our time away at meetings, and particular thanks goes to MD, Ethan Wiesler MD, Zhongyu Li MD, George Chloros MD, our wives, Debra Liu MD and Sarah Cartwright MD, for their Thomas Sarlikiotis MD, Beth Smith PhD, Tom Smith PhD, willingness to support our careers in academic medicine. vi Contents 1 Basic Principles of Ultrasound 1 9 Ultrasound of Inflammatory Myopathies 125 Francis O. Walker, MD Steven J. Shook, MD 2 Ultrasound of Peripheral Nerves 24 10 Ultrasound of Muscular Dystrophies, M yopathies, Leo H. Visser, MD, PhD and Muscle Pathology 131 Roy Beekman, MD, PhD Craig Mitchell Zaidman, MD 3 Ultrasound of Muscle 37 11 Interventional Ultrasound 150 Sigrid Pillen, MD, PhD Francis O. Walker, MD Nens van Alfen, MD, PhD 12 Ultrasound as a Complement to E lectrodiagnostic Machiel J. Zwarts, MD, PhD Studies 166 4 Basic Approach to Ultrasound of Other Structures in Andrea J. Boon, MBChB the Extremities 57 C. Michael Harper, MD Christopher Harker Hunt, MD 13 Future Directions in Neuromuscular U ltrasound 177 5 Ultrasound of Focal Neuropathies 72 Francis O. Walker, MD Michael S. Cartwright, MD Appendix A 187 6 Ultrasound of the Brachial Plexus 91 Appendix B 190 Robert S. Weller, MD Index 193 7 Ultrasound of Polyneuropathies 106 Lisa D. Hobson-Webb, MD 8 Ultrasound of Motor Neuron Disease 118 Michael S. Cartwright, MD vii This page intentionally left blank 1 Chapter Basic Principles of Ultrasound Francis O. Walker, MD Overview KEY POINTS l In neuromuscular ultrasound, a linear array transducer is The ancient Greeks understood the fundamental relation- commonly used. This type of transducer typically contains ship of reflected light and sound as revealed in the story of a row of several hundred crystal or ceramic piezoelectric Echo and Narcissus in which man (Narcissus) is smitten by elements that convert electrical energy into sound waves, reflected light and immune to the nuance of reflected sound which then reflect off tissue, and the elements convert (Echo). It is unlikely that the creator of this story anticipated the returning sound back into an electrical impulse. The the development of underwater sonar or medical ultrasound, transducer is in receive mode, rather than transmit mode, but it seems plausible that she would have understood the greater than 99% of the time. value of echolocation as another way of perceiving the natural l Sound is a pressure wave that travels at variable speeds, world. depending on the tissue in which it is traveling. Ultrasound Nature has long recognized and appreciated the perceptual devices use the assumption that sound travels at an average gift of echolocation, which manifests in the biologic marvels speed of 1540 m/s to calculate the depth of returning of bats and whales, creatures that use echoes to map space echoes. in darkness and deep water, areas where sight is uninforma- tive. The ability possessed by these creatures to experience the l As sound travels through tissue it will reflect, or send world directly through echolocation will always remain for- an echo, off of dense structures, but it also attenuates eign to humans. We cannot replicate the vocal, auditory, and as it propagates through tissue, with higher frequencies particularly the cortical adaptations required for this type of attenuating more than lower frequencies. For this reason, experience, so we must settle for a surrogate, which is the use higher frequency transducers are capable of producing high of reflected sound energy to manufacture a visual representa- quality images of superficial structures but not of deeper tion of anatomic space. For an in-depth discussion of echo- structures. location and other unique sensory experiences in nonhuman l Most neuromuscular ultrasound is conducted using B-mode mammals, Howard Hughes’ award winning book Sensory (brightness mode) imaging, which is also known as real- Exotica is highly recommended.1 time imaging. Sound is continuously being transmitted and Almost everyone who is using this textbook will have received by the transducer, and the returning impulses are already had some experience with ultrasound, but even assessed by a computer, using assumptions and formulas to for the experienced ultrasonographer, it is helpful to begin produce a visual display that incorporates spatial and time with a review of instrumentation as a means of explaining resolution, as well as echo intensity. how an ultrasound image is created. The discussion begins with a description of how to get started generating images with an instrument, followed by discussions of the trans- Few neuromuscular clinicians are familiar with the fun- ducer and its elements, the behavior of sound and echoes damental physical principles of ultrasound. This chapter in human tissue, and how the ultrasound instrument regu- is designed to explain them qualitatively in the context of lates the transducer and translates echoes into an image. In operating the instrument. The intent is to make explicit parallel with these descriptions are some brief comparisons how engineering determines what appears on the screen, of ultrasound with electrodiagnostic technology. Neuro- and how understanding this process helps optimize the muscular pathology is largely corroborated, not by other image displayed. This introduction will be adequate for imaging modalities, but by electrodiagnostic findings, so the reader to use and understand the instrument and to an appreciation of this technology is of value. Further, guide further reading for those who see the potential for many readers have considerable experience with electrodi- further development of the technique. Current instru- agnosis, and this will help them appreciate the mechanics ments have been designed with organs and tissues in mind of ultrasound. There are also some parallel discussions on other than nerve and muscle, so enhancements in instru- the nature of human perception and how that also deter- mentation are possible, given sufficient feedback from mines display characteristics of ultrasound instrumenta- experienced users. tion (Fig. 1.1). 1

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