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616.85 pssaM 2005 «7 SECOND EDITION _ MOTOR SPEECH DISORDERS Substrates, Differential Diagnosis, MOSBY and Management Joseph R. Duffy, PhD, BC-NCD Head Section of Speech Pathology Department of Neurology Maye Clinic Professor Speech Pathology Mayo Clinic College of Medicine Rochester, Minnesota with 95 illustrations ELSEVIER MOSBY 11830 Westine Industrial Drive St. Lous, Missouri 63146 MOTOR SPEECH DISORDERS: SUBSTRATES, DIFFERENTIAL DIAGNOSIS, AND MANAGEMENT, Second Elton Copyright © 2008, 1995 Mayo Foundation for Medical Education and Research Al rights reserved. 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Neither the publisher nor the author assumes any liability for any injury and/or damage to persons or property arising from this publication, ‘The Publisher ISBN-13: 978-0-323.02452-5 ISBN-10:0-323-02452-1 Acquisitions Editor: Kathy Falk Managing Editor: Christie Hart Publishing Services Manager: Pavcia Tannian Project Manager: Sharon Corel Designer: Paula Ruckenbrod Cover Design: Iyotika Shroff Printed in the United States of America Last digit isthe print number: 9-8 7 6 t to grow libraries in developing countries worwelseviercom | wwwcbookaid.org | wwwsabre.org To my parents, family, and colleagues for their support and inspiration Preface The first edition of this book was published at the midpoint of the “decade of the brain,” a time of unprecedented growth in our understanding of the neural bases of cognition and behavior and their dis- orders. Now, in the early years of the new millen- nium, the growth curve continues to accelerate, A crude index of the slope of the curve in the arena of motor speech disorders is reflected in a simple MEDLINE inquiry using “dysarthria” and “apraxia of speech” as subject headings or key words. Between 1995 (the year the first edition of this book was published) and September 2004 the search yielded 1075 citations, more than a 50% increase ‘over the number of citations in the preceding decade. Developments in this past decade reflect contri- butions from speech-language pathology, speech science, neurology, and a number of related disci- plines. They have had an impact on what we under- stand about the neurologic bases of motor speech disorders, their diagnoses, and their management. As is often the case in science and clinical practice, these advances are paradoxically humbling. There is still much to learn. Readers familiar with the first edition of this book will note that this second edition contains the same number of chapters, only 2 few changes in chapter titles, and the same basic organization of information within each chapter. The retention of this basic format reflects feedback from many instructors and. students who said that it facilitated learning and should not be altered. I have resisted the suggestion from some that the content be simplified because my hope is that the book will be useful to teachers and graduate students committed to a depth of under- standing, as well as to professionals in need of a source of information for clinical practice and research. The changes in this edition mostly re the integration into the original format of new infor- mation and refinements of previous knowledge, rather than any significant paradigm shifts. Some of the changes are based on my own clinical experi- ence, research, and reflection about the topic. This book addresses the neurologic underpin- nings of speech, the speech disorders that can develop when the nervous system goes awry, and the ways in which motor speech disorders can be assessed, diagnosed, and managed. Its contents reflect what we think we know about these things. Within and between the lines of each page, the lacunae will be apparent. The book is intended primarily for graduate stu- ing clinicians, and researchers in the discipline of speech-language pathology. It will also be of interest to people in related disciplines—such as neurology, neuropsychology, and rehabilitation medicine—who are interested in speech disorders as an index of neurologic disease and its localization, and the differential diagnostic value of speech dis- orders to medical diagnosis and care. The book is divided into three major parts that adress (1) the neurologic substrates of speech and its disorders, (2) the disorders and their diagnoses, and (3) management. The information included in these three parts is interrelated, Likewise, an understanding of the material presented in all three parts is necessary for one to excel in any of the areas described. Part One, Chapters 1 through 3, addresses sub- strates. Chapter 1 provides basic definitions of motor speech disorders (the dysarthrias and apraxia of speech) and discusses their distinction from other speech abnormalities. Data from the Mayo Clinic speech pathology practice—updated from the first edition—are reviewed to provide a sense of the prevalence and distribution of motor speech disor- ders in multidisciplinary medical practices. The chapter also provides an overview of perceptual, acoustic, and physiologic methods for studying motor speech disorders. Finally, it reviews approaches to characterizing the disorders and intro- duces the categorization scheme developed by Darley, Aronson, and Brown as the book's vehicle for discussing the dysarthrias. Chapter 2 reviews the neurologic bases of motor speech and its pathologies. It focuses on structures and functions that are important to speech, the pathologies that may produce motor speech disor- ders, and some of the physical and behavioral deficits that may accompany motor speech disorders. Its discussion of the relationship of motor speech to the nervous system’s final common pathway, direct and indirect activation pathways, and control circuits provides a foundation for understanding the distine- tions among the major categories of motor speech disorders that are addressed in subsequent chapters. vili___Preface Chapter 3 addresses the examination of motor speech disorders. It reviews the purposes and ‘methods of clinical examination, particularly as it relates to differential diagnosis, including history taking, evaluation of each component of the speech mechanism during nonspeech and speech the perceptual analysis of speech, and intel assessment, Part Two, Chapters 4 through 15, focuses on the disorders and their diagnoses. Chapters 4 through 11 address each major dysarthria type and apraxia of speech. In contrast to the first edition, flaccid dysarthrias (Chapter 4) and hyperkinetic dysarthrias (Chapter 8) are recognized as plural disorders, each with several subtypes that differ in lesion loc tion and/or underlying neuropathophysiology. Each chapter begins with a brief overview of relevant neu- rologic and neuropathologic underpinnings and reviews some of the conditions that are commonly or uniquely associated with the disorder under dis- cussion. This is followed by a review of the etiology, localization, associated cognitive problems, and intelligibility for a substantial number of quasi- randomly selected cases with each type of motor speech disorder. Finally, discussion of common patient perceptions and complaints, a review of con- firmatory oral mechanism and related findings, and a detailed description of salient perceptual speech char- acteristics and associated acoustic and physiologic findings are presented. Each chapter ends with four to nine case studies that illustrate some of the major points made in the text. The case studies provide a sense of the clinical reality of the disorders, the ways in which knowledge is applied in clinical practice, and the value and shortcomings of the enterprise. Chapier 12. addresses forms of neurogenic ‘mutism that reflect severe motor speech disorders, aphasia, or nonaphasic cognitive and affective deficits. Chapter 13 addresses several neurogenic speech disturbances (acquired neurogenic stuttering, palilalia, echolalia, cognitive and affective di ances, aphasia, pseudoforeign accent, and aproso- dia) that have close or distant relationships with motor speech disorders. Both chapters end with ilus- trative case studies One of the most challenging diagnostic problems in medical speech pathology practices involves the distinction between disorders that reflect neu- ropathology and those that reflect psychopathology or nonorganic influences. Chapter 14 addresses acquired psychogenic and related nonorganic speech disorders. It discusses their common etiologies and describes their most common speech characteristics. ‘The important aspects of history taking and the observations that contribute to diagnosis are reviewed. The variety of speech characteristics asso- ciated with psychogenic voice and fluency disorders, as well as less frequently occurring psychogenic culation, resonance, and prosodic abnormalities, are described. Case studies at the chapter's end show how people with these disorders sometimes present in clinical practice. Chapter 15 provides general guidelines for dif- ferential diagnosis. It synthesizes and summarizes the information in Chapters 4 through 14 that is most important to differential diagnosis. It emphasizes distinctions among the —dysarthrias, between dysarthrias and apraxia of speech, between motor speech disorders and aphasia, among different forms of mutism, between motor speech disor- ders and other neurogenic speech disorders, and between neurogenic and psychogenic speech disorders, Part 3, Chapters 16 through 20, addresses man- agement, Chapter 16 provides an overview of prin- ciples for managing motor speech disorders. It discusses broad management goals, factors that influence management decisions, and the medical, prosthetic, behavioral, augmentative and alternative communication, counseling, and support aspects of management. Itreviews in some detail principles and guidelines for behavioral treatment that can be applied to all motor speech disorders. Chapter 17 focuses on management of the dysarthrias. It discusses speaker-oriented approaches, that include medical, prosthetic, and behavioral interventions. It also examines management in rela- tion to specific types of dysarthria, highlighting the fact that differential diagnoses among the dysarthrias can influence management, and that some approaches are well suited to certain dysarthria types whereas other approaches are not. The chapter also addresses communication-orieated strategies that may be used by dysarthric speakers or their listeners, to facilitate communication, independent of dysarthria type and changes in speech production per se. Chapter 18 focuses specifically on the manage- ment of apraxia of speech. It makes clear that dysarthrias and apraxia of speech share a number of ‘management attributes but that, because their under- lying natures are fundamentally different, their man- agement differs in a number of important ways, Chapter 19 addresses the management of the other neurogenic speech disturbances discussed in Chapter 13. In keeping with the primary focus of the book, it emphasizes treatment of the speech charac~ teristics associated with them, rather than the affec- tive, cognitive, or linguistic disturbances that may underlie some of them. Chapter 20 addresses the management of acquired psychogenic speech disorders. This chapter is included because the successful management of psychogenic speech disorders can make a valuable contribution to diagnosis in cases where there is uncertainty about neurogenic versus psychogenic etiology. It is hoped that the chapter contributes to clinicians’ differential diagnostic skills as well as their treatment skills The,impetus for this book grew out of my desire to integrate what is known about the bases of motor speech disorders with the realities of my clinical practice in which differential diagnoses and man- agement are the order of the day. I have learned as much in writing this second edition as I did for the first and have become a wiser and better clinician because of it. [have also become convinced than our ignorance still surpasses our certainty, Some of what Preface ix I don’t know can be found in the minds and daily practices of other clinicians, scientists, and scholars, and some of it represents unanswered or unasked questions. I do hope that the facts and clinical obser- vations reflected in these pages provide a friendly leaming vehicle for clinicians and researchers. in training, a source of useful information for practic- ing clinicians and researchers, and some seeds of interest for increasing our understanding of these disorders and our ability to help people who have them, Joseph R. Duty Many people deserve recognition and my gratitude for their contributions to the birth of this second edition. They bear no responsibility for any of the ‘book's shortcomings. I thank the staff at Elsevier for their expert and collegial assistance; John Schrefer, Kellie White, and Jennifer White for helping to get the project off the ‘ground; and especially Kathy Falk and Sharon Corell for guiding it through to completion. The spirit and skill of my secretary, Carie Dittrich, made many things easier. Several people read drafts of portions of this book and provided valuable feedback. I thank Mick McNeil for his helpful comments on the apraxia of speech chapter, Ray Kent for his review of the uni- lateral upper motor neuron dysarthria chapter, and Geoff Fredericks for his feedback on many chapters. Tam particularly indebted to Jack Thomas for his ‘comments on every chapter of the book. ‘A special thanks to Ray Kent and his colleagues at the University of Wisconsin for the opportunity to collaborate on motor speech disorders. research, work that has influenced the substance of this book. ‘Ako, comments about faculty, students, and c! my judgments about what did and didn’t need fixing for this edition. ‘A number of people have served as my mentors over the years—the very special influences of Bob Dufly. Fred Darley, and Amie Aronson float smong these pages. The many thousands of patients who have taught me about motor speech disorders, my speech pathology and neurology colleagues in the Department of Neurology at the Mayo Clinic, and my colleagues and very good professional friends have all helped shape this book: Finally, a special thank you to my wife and col- league Penny Myers for her support, empathy, and patience. She provided the intangibles that helped ime finish the race. Joseph R. Duffy Part 1: Substrates 1. Defining, Understanding, and Categorizing Motor Speech Disorders 2. Neurologic Bases of Motor Speech and Its Pathologies 3. Examination of Motor Speech Disorders "art 2: The Disorders and Their Diagnoses 4, Flaccid Dysarthrias 5. Spastic Dysarthria 6. Ataxic Dysarthria 7. Hypokinetic Dysarthria 8. Hyperkinetic Dysarthrias 9. Unilateral Upper Motor Neuron Dysarthria 10, Mixed Dysarthrias 11. Apraxia of Speech 12. Neurogenic Mutism 13. Other Neurogenic Speech Disturbances 14, Acquired Psychogenic and Related Nonorgenic Speech Disorders 15, Differential Diagnosis, Part 3; Management 16. Managing Motor Speech Disorders: General Principles 17. Managing the Dysarthrias 18, Managing Apraxia of Speech 19. Managing Other Neurogenic Speech Disturbances 20. Managing Acquired Psychogenic and Related Nonorganic Speech Disorders 7 109 13 163 187 217 255 215 307 335 353 381 409 435 307 525 535 val ae fe apt 7 Ah c Defining, Understanding, and Categorizing Motor Speech Disorders CHAPTER OUTLINE I. The neurology of speech IL. The neurologic breakdown of speech IIL, Basie definitions A. Dysarthria B. Apraxia of speech C. Motor speech disorders IV, Speech disturbances that are distinguishable from motor speech disorders ‘A. Other neurologic disorders B. Nonneurologic disturbances C. Normal variations in speech production V. Prevalence and distribution of motor speech disorders VI. Methods for studying motor speech disorders A. Perceptual methods B. Instrumental methods C. The clinical salience of the perceptual analysis ‘of motor speech disorders VIL. Categorizing motor speech disorders A. Characterizing motor speech disorders B. The perceptual method of classification VIL Summary Speech is a unique, complex, dynamic motor activ- ity through which individuals express thoughts and emotions and respond to and control their environ- ‘ment. It is among the most powerful tools possessed by the human species, and it contributes enormously to the character and quality of life. Under most circumstances, speech is produced with an ease that belies the complexity of the oper- ations underlying it. The study of normal speech processes helps establish the enormity of the act. Unfortunately, neurologic disease can also unmask the complex underpinnings of speech by disturbing its expression in various predictable ways. These dis- turbances, the mechanisms that help explain them, the signs and symptoms that define them, and their ‘management are the subjects of this book. [Xl THE NEUROLOGY OF SPEECH Speech requires the integrity and integration of numerous neurocognitive, neuromuscular, and mus- culoskeletal activities. These activities can be sum- marized as follows: 1. When thoughts, feelings, and emotions gen- erate an intent to communicate, they must be organized and converted to verbal symbols in manner that abides by the rules of language. These activities are referred to as cognitive- linguistic processes 2. The intended verbal message must be orga- nized for neuromuscular execution. This activity includes the selection and sequene- ing of sensorimotor “programs” that activate the speech muscles at appropriate coartc lated times, durations, and intensities. These activities are referred to as motor speech planning and programming 3. Central and peripheral nervous system activ- ity must combine to regulate and execute speech motor programs by innervating the respiratory, phonatory, resonatory, and artic- ulatory muscles in a manner that generates an acoustic signal that faithfully reflects the goals of the programs. The neuromuscular transmission and subsequent muscle contrac- tions and movements of speech structures are referred to as neuromuscular execution. The combined processes of speech motor plan- ning, programming, and neuromuscular execution are referred to as motor speech processes. [Ea THE NEUROLOGIC BREAKDOWN OF SPEECH When the nervous system becomes disordered, so may the production of speech. In fact, changes in speech may announce the presence of neurologic disease. The effects of neurologic disease on speech are often lawful, predictable, and clinically unique and recognizable. Recognizing and understanding 3 Substrates predictable patterns of speech disturbance and their underlying neurophysiologic bases are valuable for at least four reasons: 1 Understanding nervous system organization {for speech motor conirol. The predictable ‘association of patterns of speech deficit with localizable pathology can contribute to our understanding of the nervous system’s anatomic and physiologic organization for speech motor control. Just as. the study of aphasia teaches us something about the organization and localization of cognitive- linguistic processes associated with language behavior, the study of motor speech disorders informs us about the physiology and local- ization of speech production. Differential diagnosis and localization of neurologic disease. In 1987 Aronson’ called the contribution of speech diagnosis to medical diagnosis one ofthe best-kept secrets of our time by both speech pathology and medicine. Although this is somewhat less true today, the secret is that speech changes can be the first or only manifestation of both organic and psychiatric disease, and their recognition and diagnosis can contribute to disease localization, diagnosis, and care. This necessitates modification of beliefs that speech diagnosis always follows medical or neurologic diagnosis, and that speech diag~ nosis and management are separate from medical diagnosis and management. This value becomes evident in many succeeding chapters, especially within the context of the case histories on major motor speech disor- ders at the end of each chapter. Prevalence. Neurologic disorders are com- mon, Few are truly curable, and they are a ‘major cause of disability in the population as a whole.” Neurologic speech disorders rep- resent a significant proportion of acquired communication disorders (Figure 1-1). An Voice Idiopathic (nonneurelogic a ‘nonpsychagenic) 8% Other cognitive. language disorders 11% Other neurologic Aohasia speech disorders 19% % FIGURE 1-1 Distribution of acquired communication disorders, Speech Pathology, Department of Neurology, Mayo Clinic, 1987-1990 plus 1993-2001. Based on 14,269 evaluations of people with « primary speech psthology diagnosis of an acquired communication disorder. Referrals came primarily from neurology, ctorhinolaryngology, neurosurgery, physical medicine, intr- nal medicine, and patierts themselves, Numbers reflect diagnostic consultations snd not the number of patients receiving test- ‘ment, ‘Motor speech disorders include the dysarthrias and apraxia of speech. Aphasia includes all types of acquired aphasia. Other neurologic speech disorders include stuttering-like behavior, aprosodia, spasmodic dysphonia sssociated with dystonia or tremor, nonspecific central nervous system mutism or isolated aphonia and reduced loudness, and speech deficits associated with sensory disturbances. Other cognitive-language disorders include dementia, confusion, nonaphasic cognitive communication deficits associated with traumatic brain injury, akinetc mutism, alexia with or without agraphia, specific memory loss, ictal speech arrest, neurologic language disorder of undetermined type, and unresponsiveness associated with neurologic disease. Anatomic def- ciency includes laryngestomy and glssectomy. Voice (nonneurologic or nonpsychogenic) disorders include etiologies of vocal abuse, papilloma, intubation and tracheostomy, vocal fold bowing, hormonal imbalance, neoplasm, acomegaly, and surgery. P3)- ‘chogenic includes nonorganic speech disorders characterized by mutism, aphonia or dysphonia, spasmodic dysphoria, dys- prosody, stuttering-like behavior, infantile speech, articulation disturbance, foreign accent, high pitch, and abnormal loudness. Idiopathic disorders (ie., of unknown etiology) include dysphonias, stridor, palilalia, monopitch, pseudoforeign accent, ard stuttering-like behavior, Chapter 1 increase in their prevalence can be antici pated because of increased survival rates for a number of neurologic conditions and because increasing longevity in the general Population gives neurologic disease “more ‘opportunity to introduce itself to u 4. Management. The identification of deviant speech characteristics, their localization to various levels of the speech system, and an understanding of their likely neuropatho- physiology can provide important clues for ‘management. For example, knowing that an individual's articulatory distortions are pri- marily related to incoordination and not to weakness might lead to efforts to assist coordination (¢.g., by modifying rate and prosody) rather than atiempis to increase strength through exercise. El BASIC DEFINITIONS Itis necessary to define some terms used to refer to certain neurologic speech disturbances. For those learning about these disorders for the first time, the definitions provide a framework for beginning to think about them. For those more familiar with the topic, the definitions establish boundaries of ‘meaning that are sometimes blurred in the medical and speech pathology literature, Dysarthria ‘The work of Darley, Aronson, and Brown!" pro- vided the modem definition of dysarthria. Because their pivotal 1969 and 1975 contributions are refer- enced frequently throughout this book, the abbrevi- ation DAB is used to refer to them. DAB defined dysarthria as the following: a collective name for a group of speech disor-

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