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Apraxia of Speech “This moming I was.appalled at my terrible reading aloud a new passage. I had difficulty enunciating most every word. Particularly troublesome were the words ‘manipulate’ and ‘manipulated,’ I couldn't seem to get Past ‘manifested.’ Later I tried again and was only barely pronouncing the words correctly Also, 1 was full of slurring the sounds of syllables.” (CHAPTER OUTLINE 1. Anatomy and basic functions of the motor speech programmer AA Functions of the motor speech programmer B. The motor speech programmer network IL Nonspeech, nonoromotor, and nonlinguistic char- act of patients with apraxia of speech IIL. Etiologies IV. Speech pathology A Terminology and theory B. Distribution of etiologies, lesions, and associated deficits in clinical practice CC. Patieat perceptions and complaints D. Clinical findings E. Acoustic and physiologic findings V. Cases, VE Summary We now turn our attention to a category of motor speech disorders (MSDs) that differs from the dysarthrias. Its designation, apraxia of speech (AOS), distinguishes it from the movement disorders represented by the dysarthrias, as well 2s from linguistically based speech errors associated with aphasia. The clinical manifestations of AOS are believed to reflect a disturbance in the planning or programming of movements for speech (From the writen diary of a 72-year-old woman with a progressive apraxia of speech and mild aphasia) Unlike the dysarthria, AOS can exist without clinically apparent impairments in the speech muscles for nonspeech tasks. Unlike aphasia, in which there are nearly always multimodality impair- ments of language, AOS can exist independent of problems with verbal comprehension, reading comprehension, and writing, as well as independent of verbal errors that are unrelated to articulation and prosody. Although AOS ofien coexists with dysarthria and aphasia, the distinctiveness of its clinical characteristics, its apparent nature as a motor planning or programming disturbance, and its ccea- sional emergence as the only disturbance of com- ‘munication justify its identification as a unique type of speech disorder. Its distinction from other MSDs is additionally warranted because of its localizing Valve; it is almost always the result of pathology in the left cerebral hemisphere. To repeat the simple definition provided in Chapter 1, AOS is a neuro- logic speech disorder that reflects an impaired capacity to plan_or program sensorimotor com- ‘mands necessary for directing movements that result in phonetically and prosedically normal speech. It can occur in the absence of physiologic disturbances associated with the dysarthrias and in the absence of disturbance in any component of language. “With the possible exception of speech induced movement disor ders, such ascertain dystonia-based hyperkinetic dysarthrias 307 308 __The Disorders and Their Diagnoses AOS is encountered as the primary speech disor- der in a large medical practice at a rate comparable to that of several of the major single dysarthria types. Based on data for primary communication disorder diagnoses in the Mayo Clinic Speech Pathology practice, it accounts for 7.6% of all MSDs (see Figure 1-3). It also occurs frequently as a secondary diagnosis in people with left (dominant) hemisphere lesions whose primary communication disorder is aphasia, and it can be a secondary diagnosis in people whose primary diagnosis is dysarthria or some other neurologic communication disorder. ‘Thus AOS is present in far more than 7.6% of people who have communication disorders associated with left hemisphere pathology ‘The clinical features of AOS convey the impres- sion that the appropriate message has been formu lated but that what should be automatic “decisions” about its physical expression have been inefficiently or poorly organized or controlled, although not because of problems with basic motor abilities. Care- ful study of AOS can illustrate some of the distine- tions between motor speech planning/programming and the neuromuscular execution of speech, and between motor speech planning/programming and the formulation and organization of the linguistic units that are spoken. Such study also highlights the difficulty often encountered in attempts to make such distinctions, both theoretically and clinically. The concept of AOS has had somewhat of a stomny history since Darley” introduced it in the 1960s and tied it to problems with the programming of movements for speech. There have been consid- erable and important debates about its very existence or its underlying nature.» A fundamental problem has been uncertainty about its defining clinical attributes, with subsequent uncertainty about whether clinicians and researchers who claim to have studied the problem have actually been dealing with the same entity. This has introduced considerable “noise” into efforts to better understand the disorder’s cognitive, motor, and anatomic bases. However, with refine- ments in models of language and speech motor control and efforts to fit careful clinical observations “This history is traced with varying degrees of detain a number of papers, chapters, and books." Comprehensive, ettcal reviews that capture curtent thinking about the nature, clinical characteristic, and management of AOS can be found in McNeil, Robin, and Schmidt," MENeil, Doyle, and Wambaugh,” a 2001 Forum in Aphasiology with a ead paper by Varley and White- side™ and commentaries from several investigators, and numer- (us papers in a recent issve of Seminars in Speech and Language edited by MeNeil.* to them, there has in recent years been some honing of the clinical boundaries of the disorder. Rather than ‘dwell too much on historic debate and controversy, an attempt is made here to focus on what at least some clinicians and researchers now propose may be the essential characteristics of AOS and how they fit with notions about speech motor planning/ programming. What is presented here seems to make sense at this time. Its staying power depends on future clinical and research efforts. In this chapter, the location and functions of the motor speech planning/programming network are summarized in bread, general terms. Some of the theoretical and clinical debate about the nature of AOS is reviewed but not dwelled upon. Emphasis is placed on the clinical milieu in which AOS is encountered, its auditory and visible perceptual attributes, relevant acoustic and physiologic data, and some clinical case studies. The distinctions between AOS and dysarthria and aphasia are addressed in some detail in Chapter 15, which focuses on differential diagnosis. [a ANATOMY AND BASIC FUNCTIONS OF THE MOTOR SPEECH PROGRAMMER Motor speech control involves the interactive, paral- Jel, and sequential participation of all components Of the motor speech system, as well as higher level activities related to conceptualization, language, and motor planning/programming. The motor planning/ programming component of these activities is referred to here as the motor speech programmer (MSP). The MSP is a network of interacting structures and pathway’ rather thana single anatomic structure. It is influenced by sensory feedback, the basal ganglia and cerebellar control circuits, the reticular formation and thalamus, and the limbic system and right hemisphere. From this perspective, motor speech programming involves widespread areas of the central nervous system (CNS). However, for the purpose of understanding the highest levels of speech programming—pathways and structures that specify the patterns and sequences of movements for speech—the left cerebral hemisphere, particularly parietal-frontal and related subcortical circuits, can be thought of as the headquarters of the MSP and the locus of lesions that lead to AOS. Functions of the Motor Speech Programmer ‘The MSP has a leading role in establishing the plans and programs for achieving the cognitive and linguistic goals of spoken messages. It organizes the motor commands that ultimately result in the Chapter 11 Apraxia of Speech _ 309 production of temporally ordered sounds, syllables, words, and phrases at particular rates and patterns of stress and rhythm. The left hemisphere functions of the MSP seem to be more strongly tied to the linguistic attributes of speech (phorologic, semantic, syntactic, morpho- logic, plus linguistic components of prosody) than to its emotional or affective attributes, the latter com- ponents perhaps being more strongly influenced by Contributions from the limbic system and right hemi- sphere. The linguistic input to the MSP comes largely from the left hemisphere’s perisylvian area, which includes the temporoparietal cortex, posterior portions of the frontal lobe, the insula, and, in less definitive ways, the basal ganglia and thalamus. The anatomic proximity or overlap of these language areas with those of the MSP makes it likely that damage to the perisylvian language zone often results in a cooccurrence of language-related deficits (aphasia) and AOS. In clinical reality, this indeed is often the case. When speech is the goal, it can be presumed that, ‘once the phonologic representation of a message has been established, the MSP must be activated to orga- nize and activate a plan for its motor execution. This seems to involve a transformation of the abstract phonemes to a neural code that is compatible with the operations of the motor system. This neuromotor code presumably specifies the parameters of move: ment for specific muscles or muscle groups, although McNeil, Robin, and Sehmidt® point out that “the exact parameters of movement that are programmed and that represent the control variables for the motor programmer are not agreed upon.” They suggest, however, that specifications for movement duration and displacement (amplitude), acceleration, deceler- ation, time to peak velocity, muscle stiffness, and rel- ative timing of speech events are examples of some Of the kinematic parameters of movement that might be programmed. Because much of normal, mature speech is pro- duced quickly and without conscious effort, it is rea- sonable 10 assume that the MSP commonly selects, sequences, activates, and controls preprogrammed ‘movement sequences* that, through learning and practice, can be activated automatically. tis thought that motor plans/programs are established before movement begins, but that they can be modified by peripheral feedback either before the program is readied for movement or during movement execu- “Other terms that might apply include generalized motor pro- grams; veibal motor memories; engrams; movement gestalt; well-established subroutines; or “macros,” to borow computer terminology. tion.*" All of this permits rapid speech rates and greater allocation of resources to the more conscious formulation and monitoring of the cognitive and linguistic goals of communication. This rapid, direct route for phonetic encoding may occur primarily for frequently used syllables, words, or phrases. For novel syllables or movement patterns (e.., infre- quently or never-before-used multisyllabie or non- sense words), speaking under adverse conditions, or attempting to be particularly precise, itis likely that phonetic encoding is less direct (ie., less automatic) because the motor patterns need to be freshly com- puted." It has been suggested that at least some of the speech characteristics of people with AOS could reflect problems with the access to or use of prepro- grammed subroutines and the subsequent need to construct programs anew for each syllable to be uttered (Ziegler, 2002). The Motor Speech Programmer Network The MSP seems to rely heavily on left hemisphere prefrontal, premotor areas, of which Broca's area and the supplementary motor area may be most important. Broca’s area is a candidate area for making important contributions to the specification of simultaneous and sequential speech movements based on input from sensory modalities and areas, involved in linguistic formulation. Recall also that premotor areas are linked to the basal ganglia and cerebellar control circuits that have reciprocal connections with the primary motor cortex that puts into effect the motor speech act. Broca’s area is often identified as a lesion site in people with AOS. ‘The supplementary motor area is also involved in the activities of the MSP, although it seems furl removed than Broca’s area from the actual speci cation of speech movements. It has connections with the primary motor cortex and Broca’s area, the basal ganglia, and the limbic system. It seems tied to cog- nitive and emotional processes that drive or motivate action and may play an important role in the initia tion of propositional speech, as well asin its control. In general, however, it is not a common site of lesions associated with AOS. The parietal lobe somatosensory cortex and the supramarginal gyrus are also implicated in the ac- tivities of the MSP, probably before initiation of ‘Cosem, comprehensive discussions of moter _ speech planning/programming and ts relationship to AOS can be found in several sources (McNeil, Robin, and Schick, 199749822 [plus following commentary by several authors); Ziegler, 2002), 310__ The Disorders and Their Diagnoses movement but, obviously, also during series of ‘movements. These areas may be particularly impor- tant in integrating sensory information necessary for skilled motor activity and for transforming sensory information and internal goals into plans and targets for action." The inswla* (Figure 2-16) also may have specialized role in motor planning/programming for speech, perhaps particularly during speech exe- ution." It recently has been identified as a shared site of damage in people with AOS” and sometimes the only site of damage,” although AOS can occur without lesions in the insula." Finally, the basal ganglia, consistent with their known role in motor control, seem active in the activities of the MSP. Lesions of the left basal ganglia have been associated with AOS.” although far from invariably. In general, conclusions about the presumed tomy and functions of the MSP are supported by clinical findings. That is, lesions that produce AOS, are usually located in the left posterior frontal lobe or parietal lobe, or in the insula or basal ganglia. The speech characteristics of people said to have AOS are distinguishable from those associated with the dysarthrias, and AOS can be evident in people whose speech muscles perform normally for nonspeech activities and who are able to express. language through nonspeech channels (e.g., writing). Careful observation and analysis of their speech suggests that something is awry with the planning/ programming of speech movements. This distur- bance has come to be called AOS by clinicians and investigators who recognize its distinctiveness, value in contributing to our understanding of the neurology of speech and the localization of disease, and the unique demands it places on patients and elinicians who try to minimize its effects on communication, [Ed NONSPEECH, NONOROMOTOR, AND NONLINGUISTIC CHARACTERISTICS OF PATIENTS WITH APRAXIA OF SPEECH Physical speech mechanism findings, oromotor behaviors, and disorders of language that testify to the presence of dominant hemisphere pathology frequently accompany AOS. These characteristics are discussed in the section on speech pathology later in this chapter. Several additional clinical findings commonly accompany AOS. They usually reflect damage to the left frontal or parietal lobe, OF 10 left subcortical pathways and structures associated with the direct and indirect activation pathways. Many patients have varying degrees of right- sided weakness and spasticity, and some have associated sensory deficits. A Babinski sign and hyperactive stretch reflexes on the right side are also common. A hyperactive gag reflex and pathologic oral reflexes (e.g., suck, snout, jaw jerk) are not com- monly present unless there are bilateral upper motor neuron (UMN) lesions, a condition not required for the presence of AOS. Patients with AOS sometimes, but by no means invariably, have limb apraxia (LA), a disorder also associated with left hemisphere pathology and char- acterized by deficits in the performance of purposive limb movements that cannot be explained by impair- ‘ments of strength, mobility, sensation, or coordina- tion, LA usually affects movements in both the right and left limbs, although it is often masked on the right side by hemiparesis or hemiplegia, LA has been more widely accepted in neurology as a distinct clinical entity than has AOS, in spite of approaches to its clinical diagnosis that have been highly vari- able and subjective. The psychologic, physiologic, and anatomic bases of LA have heen addressed extensively in the neurologic literature since before the early part of this century when Liepman* pre- sented his historically dominant and widely accepted conceptualization of apraxia. A comprehensive review of LA is beyond the scope of this chapter.* From the theoretical stand- point, it is noteworthy that there are important his- torical and conceptual similarities and differences between notions of apraxia as it affects the limbs versus speech. Anyone interested in in-depth study of AOS should be familiar with theoretical and clin- ical issues associated with LA. From the clinical standpoint, it is important to recognize that people with left hemisphere pathology may have difficulty organizing movements of both their right and left extremities, sometimes only on formal testing, but in some cases during activities of daily living. Of special relevance for issues related to commul tion, LA may interfere with writing as well as with propositional nonverbal communication (such as pantomime and sign language).” This is an important consideration for people with severe AOS who may be in need of an augmentative or altemative form of communication, ‘Overviews of theoretical and clinical assessment and diagnostic ‘issues in limb apraxia can be found in « number of sources, For example, brie basic summaries canbe found in Brookshire" and Mesulam. More detailed reviews and discussion can be found in DeRenzi,* Daffy and Duty, Ochipr and Gonzalez Rathi,” Roy and Square-Storer.* ard Square-Stcrer and Roy." *See Bennett and Netsel? for a comprehensive discussion ofthe possible roles ofthe insula in speech and language "Aphasia also is related to difficulty in expressing propositional cr symbolic meanings throvgh pantomime and sign guage." El ETIOLOGIES Any process that damages dominant hemisphere structures involved in motor speech planning/ programming can cause AOS. Because inflammatory and toxic-metabolic diseases usually produce diffuse effects, only rarely are they associated with an obvious AOS.* Demyelinating disorders, such as multiple sclerosis (MS), are not commonly associ- ated with AOS, although the association has been observed.’ Incontrast, tumors and trauma (especially surgical trauma) are more likely to cause focal uni- lateral signs. When they affect the left hemisphere, AOS may result. Stroke is the most common cause of AOS. There is nothing unique about the nature of the vascular disturbances (or any etiology for that matter) that cause AOS. except that they can be and ofien are localized to the dominant hemisphere’s network of structures and pathways that plan and program movements for speech. Degenerative neurologic diseases, in general, are not commonly associated with AOS. Even condi- tions in which dysarthria occurs frequently, such as progressive supranuclear palsy (PSP) and multiple system atrophy (MSA), are not usually associated with AOS or other forms of apraxia affecting speech muscles." However, it is increasingly recog- nized that AOS does occur fairly frequently in certain degenerative neurologic conditions and sometimes can be their first sign. For example, although the general clinical literature on corti cobasal degeneration (CBD) suggests that AOS ‘occurs in less than 5% of reported cases,” recent studies that have carefully examined speech and lan- uage suggest that it occurs in nearly 40% of cases and is sometimes the first or among the first signs of *An example of an uncommon, probable toxic-meuabolic cause is the occurrence of AOS a part of a prominen! speech disorder that, can emerge following orhotopc liver transpiantaton. It has been ‘estimated that this speeck disturbance occurs in approximately 1% ‘of adults undergoing the procedure. Temporary cessation of the drug cyclosporin has been associated with improvement of eee "The literature documents cases of MS with aphasia, and several case descriptions sugges that AOS was also present.” The author has seen a few cases of MS with AOS, all accompanied by aphasia. AOS has also been reported in a few patients receiving {mmunosuppressive agents following liver transplantation “Exceptions do occur, however. For example, AOS with aphasia hhas been reported as the first sign of disease in « paticat with autopsy-confirmed PSP ‘See Chapter 10 for more information about CBD, including the

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