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
307308 __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 theChapter 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