Chapter 10 Intentional Motor Disorders and the Apraxias

Kenneth M. Heilman, Edward Valenstein, Leslie J. Gonzalez Rothi, and Robert T. Watson

Intentional (When) Disorders Akinesia Hypokinesia Hypometria Motor Extinction Motor Impersistence
Defective Response Inhibition Motor Perseveration Pathophysiology of Intentional or When Disorders

117 Clinical Pathology 117 Praxic (How) Disorders 119 Limb-Kinetic Apraxia 119 Ideomotor Apraxia 119
Conduction Apraxia 119 Dissociation Apraxia [20 Ideational Apraxia 121 Conceptual Apraxia Clinical
Pathology 121 Conclusion

123 123 124 124 127 128 128 129 129 I 1(1

In humans the corticospinal motor system together with the motor units can mediate an almost infinite
number of movements. Because the purpose of motor systems is to allow people to interact with their
environment, the pyramidal motor neurons need to be guided by instructions or programs. For the
motor system to deal effectively with the environment, it needs at least two major types of programs:
praxic and intentional. The praxic programs provide the corticospinal system with the knowledge of how
to make learned skilled movements. The intentional programs provide the corticospinal system with
information about when to move. In this chapter, we first discuss disorders of the intentional, or
"when," system and then we discuss praxic, or "how," system disorders.

INTENTIONAL (WHEN) DISORDERS

Unlike the praxic systems that program the temporospatial aspects of a movement, the intentional
systems provide instructions about goals. There are four types of intentional instructions: (1) when to
start a movement; (2) when not to start a movement; (3) when to continue or sustain a movement or
posture; and (4) when to stop or complete a movement. The inability to initiate a movement in the
absence of a corticospinal or motor unit lesion is termed akinesia. Hypokinesia is a delay in initiating a
response. The inability to withhold a response to a sensory stimulus is called defective response
inhibition. The inability to sustain a movement or posture is called motor impersistence, and the
inability to stop a movement or an action program is termed motor perseveration.

In the next section, we describe each of these intentional disorders, including subtypes of each category,
and we discuss bow to examine patients for these disorders. We also discuss the pathophysiological and
neuropsychological mechanisms that may be associated with these disorders.

Akinesia

An organism might fail to initiate a movement for many reasons, but comprehension, attentional,
perceptual, and sensory disorders that lead to a failure of movement initiation should not be termed
akinesia. Dysfunction of the motor system, including the motor unit (lower motor neuron, myoneural
junction, and muscle) and the upper motor neuron (pyramidal or corticospinal system) may also be
associated with a failure to initiate a movement. Disorders of these systems, however, cause weakness
rather than akinesia, and akinesia is defined by an initiation failure that cannot be attributed to
dysfunction in the corticospinal system or the motor units. Akinesia is caused by a failure of the systems
that activate these corticospinal motor neurons.
There are three major methods by which a clinician can distinguish an akinesia from extreme weakness
caused by dysfunction of the motor systems. One is behaviotal, the second depends on the pathological
locus of a lesion, and the third uses stimulation of the corticospinal system and recording of motor
evoked potentials. Regarding the behavioral method, certain types of akinesia are present under certain
sets of circumstances and absent in others. If one can demonstrate that a patient makes movements in
one set of circumstances and not in the other, then one

117

118 APPROACH TO COMMON NEUROLOGICAL PROBLEMS

cannot attribute this failure to move purely to dysfunction in the motor system. However, if the akinesia
is not limited to a set of circumstances but is global, then one may have to depend on brain imaging
(e.g., computed tomography or magnetic resonance imaging), pathology, or physiological techniques
such as magnetic stimulation to demonstrate that the brain lesion did not involve the motor system.

Types of Akinesia

There arc several forms of akinesia. For ease of discussion, we have divided them into three major
categories: (1) body part, (2) action space, and (3) stimulus-response conditions. These three categories
may be interactive.

1. Body part: Akinesia may involve the eyes, the head, a limb, or the total body. 2. Action space: Akinesia
of the limbs, eyes, or head may depend on where in space the body part is moved or in what direction it
is moved. There may also be a hemispatial or directional component to the akinesia. In directional
akinesia, there is a reluctance to move in a specific spatial direction usually contralateral to a
hemispheric lesion. For example, horizontal gaze palsy is a form of directional akinesia. Similarly, there
may be directional akinesias of both head and arms that results in a reluctance to move these in a
contralesional direction. Spatial akinesia has been described for the arm where independent of
direction, an arm that fails to move or has decreased movements in contralesional hemispacc, as
defined by the midsagittal plane of the body, can move in ipsilesional hemispace. Whether hemispatial
akinesia that is totally independent of direction has been described for the eyes or head is not known.

3. Stimulus-response conditions: Movements can be produced in response to an external stimulus or

they can occur in the absence of an external stimulus. We term movements that arc in response to a
stimulus exogenously evoked motor activation (exo-evoked), and those that appear to be spontaneous
endogenously evoked activation (endo-evoked). A patient may have both exo-cvoked and endo-evoked
akinesia, which we term mixed akinesia.

Testing for Akinesia

When testing for akinesia, one may want to assess the various body parts discussed earlier. To
determine whether a patient has endo-evoked akinesia, one has to observe spontaneous behavior or
the lack of it. Patients with endoevoked akinesia often have symptoms of abulia, an uncommon lack of
spontaneous, goal-directed behavior that is seen predominantly with lesions of the basal ganglia and the
frontal lobes. Patients with endo-evoked akinesia, despite having reduced spontaneous activity, may
respond
normally to external stimuli. For example, endo-evoked akinesia is usually associated with Parkinson's
disease. The patient with severe Parkinson's disease will often fail to move spontaneously; however,
when stimulated, such a patient may show almost normal movements (paradoxical kinesia). When a
patient has good strength and spontaneously moves but fails to move to a specific stimulus, the failure
to move in response to a stimulus is often attributed either to an elemental sensory defect or to sensory
inattention and neglect. Although sensory defects and sensory neglect may be responsible for a failure
to respond, exo-cvoked akinesia is often confused with sensory defects and sensory neglect. The basic
testing method used for dissociating sensory defects and sensory neglect from exo-evoked akinesia
(motor neglect) is the crossed response task. This was first tested in monkeys by training a monkey to
respond with its right arm to a left-sided stimulus and to respond with its left arm to a right-sided
stimulus. If the animal failed to respond to a contralesional stimulus using the ipsilesional arm, it was
considered to have sensory neglect. However, if the animal demonstrated no weakness of the
contralesional extremity bur failed to move the contralesional extremity in response to ipsilesional
stimuli, it was considered to have exo-evoked akinesia, or motor neglect. The crossed response task can
be used not only to test the limbs but also to test the eyes, the head, or the whole body.

When assessing for akinesia, as a function of action space, one should test both directional and
hemispatial movements of the eyes, the head, and the limbs. One may want to determine whether the
directional or hemispatial movements are endogenously or exogenously evoked. When attempting to
determine whether there is an endoevoked directional akinesia of the eyes, one can observe the
spontaneous eye movements and see whether there is ipsilesional deviation of the eyes or a failure of
the patient to spontaneously look into contralesional space. To determine whether a patient has an exo-
evoked directional akinesia of the eyes, one can use a modification of the crossed response paradigm in
which the patient must look cither toward (ipsilesional direction) or away (contralesional direction) from
ipsilesional and contralesional stimuli. For example, the examiner stands directly in front of a patient
and asks the patient to fixate on the examiner's nose. The examiner raises both hands to eye level and
keeps one hand in the patient's right visual field and the other in the left visual field. The patient is
instructed to look away from the moving hand if the hand moves downward and toward the hand if it
moves upward. When the contralesional hand is moved upward, a failure to look at the contralesional
hand (moving in a contralesional direction) may be related to either a hemianopia, sensory neglect, or a
directional akinesia. However, when the ipsilesional hand moves downward, a failure to look toward the
contralesional hand suggests an exo-evoked directional akinesia of the eyes (Butter et al. 1988).

INTENTIONAL MOTOR DISORDERS AND THE APRAXIAS 119

To test for directional and hemispatial akinesia of the head or an arm, one can use similar tests. To test
fot a directional bias of an arm (similar to eye deviation), one can ask the patient to close his or her eyes
and point to his or her sternum. The subject is then asked to point with his or her index finger to a point
in space perpendicular to the sternum (e.g., the midsagittal plane). Patients with a motor (intentional)
bias will point toward their lesioned hemisphere. A task was developed that can be used to test for
endoevoked directional limb akinesia. In our modification, a patient is blindfolded and small objects
such as pennies are randomly placed in wells that are scattered on a board in both body hemispatial
fields within arm's reach. The patient is asked to retrieve as many pennies as possible. The task is
considered endogenously evoked because the patient cannot see the pennies and must initiate
exploratory behavior in the absence of an external stimulus. Patients with an endo-evoked directional
akinesia of the arm may fail to move their arm fully into contralateral hemispace and explore for
pennies.

To test for directional and hemispatial akinesia, one can also use one of the several video camera
paradigms that allows one to dissociate deficits of perception and attention from those of intention and
action (Coslett et al. 1990). In these paradigms, patients can see only their hand bisecting a line through
a TV monitor. In this technique, the line (where the action takes place) can be positioned in
contralesional or ipsilesional hemispace and the TV monitor can be independently placed in either
hemispace or the image can be reversed. If, as demonstrated by Coslett et al., abnormal performance on
the line bisection is not affected by the spatial position of the monitor or the reversal of the image on
the monitor, it suggests that the patient has a hemispatial or directional akinesia of the arm.

Hypokinesia

Many patients with milder defects in their intentional ("when") systems may not demonstrate a total
inability to initiate a response (e.g., akinesia); rather their intentional disorder may be primarily a delay
in initiating a response. We have termed this delay hypokinesia. This hypokinesia may be defined in a
manner similar to akinesia. Because a teaction time paradigm is required to detect hypokinesia, it
cannot be divided into exo-evoked and endo-evoked subtypes. Hypokinesia can be seen both in the
limbs and in the eyes and may be either independent of direction or directionally specific such that
when making directional movements, there is a greater delay initiating movements in a contralesional
direction than there is initiating movements in an ipsilesional direction. Hypokinesia can also be
hemispatial such that movements with the same limb may be slower in one hemispace than they are in
the other hemispace.

Testing

The same patadigms that are used to test for akinesia of the eyes and limbs can be used to test for
hypokinesia. Although some patients with hypokinesia have such markedly slowed initiation times that
hypokinesia can easily be detected, others have more subtle defects and reaction time paradigms may
be needed to observe their defects. Reaction times can be slowed for various reasons including impaired
attention, bradyphrcnia, or hypokinesia. To detect hypokinesia, one should use simple reaction times
that do not require cognition and therefore cannot be impaited by bradyphrcnia. Similarly, to test for
hypokinesia, one has to use stimulus parameters that ensure that inattention cannot masquerade as
hypokinesia.

Hypometria

Movements of a decreased amplitude are called hypometria. Hypometria may be directional or

hemispatial or it may involve specific body parts such as a limb or the eyes. For example, as mentioned
earlier, a patient with a right hemisphere lesion may at first be unable to saccade to the left. However,
as the patient recovers, he or she may be delayed at initiating a leftward saccade or may make multiple
small (hypometric) saccades. Hypometria may be related to akinesia or impersistence.

Motor Extinction

Patients with sensory extinction may be able to detect a single stimulus on the contralesional side of
thcit body, but when presented with a simultaneous distracting stimulus, they may then be unaware of
the contralesional stimulus. To test for milder forms of intentional deficits including akinesia and
hypokinesia, one can use a similar extinction principle. For example, one patient made unilateral
movements well and did not have sensory extinction. However, when he had to make bilateral
simultaneous movements, he either did not move his contralesional arm or moved it after a prolonged
delay.

Motor Impersistence

Motor impersistence is the inability to sustain a motor act. Persistence is the intentional equivalent of
attentional vigilance, and impersistence may be analogous to increased attentional distractibility. Like
akinesia, impersistence can be associated with various body parts including the limbs and eyes.
However, impersistence may also include other body parts such as the eyelids, jaw, and tongue. Like
akinesia, it may also be directional or hemispatial.

120 APPROACH TO COMMON NEUROLOGICAL PROBLEMS

Testing

When testing for impersistence of midline structures, one can ask patients to keep their eyes closed for
20 seconds or to keep their mouth open or protrude their tongue for 20 seconds. Patients who can
successfully persist at these acts may be further taxed by asking them to persist at two movements
simultaneously. For example, they may be asked to both keep theit eyes closed and keep theit mouth
open for 20 seconds. Limb impersistence can be tested by asking a patient to maintain a posture such as
keeping an atm extended for 20 seconds. Because limb impersistence can be hemispatial, one may want
to test each limb in its own and in its opposite hemispace. i leinispatial impersisrence, in the absence of
directional impersistence, has not been tepotted tor the bead or eyes, perhaps because it has never
been tested. One could ask a patient to sustain upgaze (or downgaze) for 20 seconds while the eyes are
directed either toward the right or toward the left. If the patient can maintain gaze in one hemispace
(e.g., the right), but cannot do so in the other (e.g., left), it would suggest that the patient has
hemispatial impersistence of the eyes. If a patient has a directional impersistence of the eyes, he or she
may be unable to maintain his ot het eyes directed to either the right or the left hemispace, and
therefote one may not be able to test for hemispatial impersistence. To test for directional
impersistence of the eyes, one tequests the patient to look either to the left or to the right for 20
seconds. A similar procedure can also be used to test the head for directional impersistence.

Defective Response Inhibition

Defective response inhibition is defined as responding when no response of that body part is required.
Defective response inhibition can be seen in the eyes, head, ot limbs. Directional defective tesponse
inhibition has been reported for the eyes, but not for the limbs or head. However, it may never have
been tested. Similarly, hemispatial defective response inhibition has not been reported; however, attain
ll may never have been lusted.

Testing

There are several forms of defective response inhibition. In one form, when using the crossed response
task, the ipsilesional limb moves when the correct response was a movement of the contralesional limb,
or the eyes or head move in an ipsilesional direction when the correct response was a movement in the
contralesional direction. This type of defective response inhibition may be termed motor {limb or
directional) allochiria or ipsilesional response disinhibition. However, before one terms such a condition
motor allochiria or ipsilateral disinhibition,

one must be certain a perceptual problem has not induced the abnormal behavior, in which case it is not
defective tesponse inhibition but true allochiria or allesthesia. In the second form of defective response
inhibition, when performing the crossed response task, the patient's contralesional limb moves when it
should not move or movement is in a contralesional direction when there should be either no
movement or movements should have been in an ipsilesional direction. We call this contralesional
response disinhibition. Contralesional response disinhibition can also be exogenously ot endogenously
evoked. If a patient is able to understand complex instructions, the best way to test for both ipsilesional
and contralesional exo-evoked response disinhibition of the limbs is to insttuct the patient to move or
lift the opposite hand (off a table) when a hand is sttoked downward and to move the same hand as
touched when it is sttoked upward. Before testing for the different forms of defective response
inhibition, it is important to establish that the patient does not have a perceptual disorder and can
correctly detect stimuli and recall instrucrions. If, when the contralesional hand is brushed up, the
patient moves the ipsilesional atm rather than the contralesional, the patient has ipsilesional
disinhibition ot motor allochiria. If, when the contralesional arm is brushed downward (which is a signal
to move the ipsilesional arm), the contralesional arm is moved instead of the ipsilesional arm, the
patient has a contralesional exo-evoked limb disinhibition. If this happens with both arms, the patient
has bilateral exo-evoked disinhibition.

Patients with exo-evoked limb disinhibition may also fail on the types of go-no-go tasks described by
Lufia. Fot example, the patient may be instructed to put up two fingers when the examiner puts up one
finger and to put up no fingers if the examiner puts up two fingers. If the patient mimics the examiner
such that when the examiner puts up one fingct, the patient puts up one finger and when the examiner
puts up two fingers, the patient puts up two fingers, the patient has echopraxia (a third form of
defective response inhibition). A paradigm similar to that used for directional akinesia of the eyes can be
used to determine wherher there is an ipsilesional, or a contralesional disinhibition of the eyes or head.
The patient is told to fix the eyes on the examinet's nose. If either hand moves down, the patient is to
direct the eyes to the opposite hand, and if the hand moves up, the patient is to direct the eyes to the
hand that moved. If, when the hand in the patient's contralesional visual field moves up and instead of
looking at that hand, the patient looks at the opposite hand, an ipsilatetal directional disinhibition is
present. If, when the contralesional hand moves down, the patient looks at this hand ratber than
looking in the opposite direction, a contralesional directional disinhibition (ot visual grasp) is present.
This directional disinhibition or visual grasp may also be bilateral.

INTENTIONAL MOTOR DISORDERS AND THE APRAXIAS 121

Motor Perseveration

Perseveration is when a patient incorrectly repeats a prior response. Although there are many types of
perseveration and several classification systems, there seems to be a spectrum between cognitive and
motor perseveration. Cognitive perseveration is when one uses a previously used cognitive strategy
inappropriately for a new or different task. Sandson and Albert (1987) call this "stuck in set"
perseveration. Luria discussed two types of motor perseveration, In one type of motor perseveration,
the patient is unable to switch to a different motor program and repeats the prior program even though
the task requirements have changed. Luria (1965) calls this inertia of program action and Sandson and
Albert (1987) call this recurrent perseveration. In rhe second type, the patient continues to perform a
movement even though the task is completed, but when instructed, the patient can switch to other
movements. Luria (1965) called this efferent perseveration, This is similar to Sandson and Albert's (1987)
continuous perseveration.

Both continuous and recurrent perseveration are forms of motor perseveration and may represent
defects in the when or intentional system. This is a defect of when to stop a motor program. Patients
may show motor (efferent) perseveration on drawing and copying tasks. For example, a patient can be
asked to draw or copy a cube. Patients with motor perseveration will repeatedly draw over lines. When
performing a cancellation task, patients with motor perseveration will perform multiple cancellations of
the same target. When asked to draw or copy a double loop, patients with motor (efferent)
perseverarion will draw more than two loops.

Pathophysiology of Intentional or When Disorders

Right-Left Asymmetries

In our introductory discussion, we advanced the hypothesis that there are generally two major types of
programs that control the motor system, the how or praxis system and the when or intentional system.
As we discuss in the next section, in right handers disorders of the praxis production system, as
evidenced hy ideomotor apraxia (IMA), are almost always associated with left hemisphere dysfunction
(Heilman and Rothi 1985). Although intentional disorders are often associated with bilateral
hemispheric lesions, when the lesions that induce intentional disorders are unilateral, they arc more
commonly associated with right hemisphere lesions. For example, limb akinesia is more often associated
with right hemisphere lesions than left hemisphere lesions. The intentional defects associated with right
hemisphere dysfunction, however, are often nor limited to the left limb. Using a reaction time paradigm,
right hemisphere infarctions are associated with a greater slowing of reacrion times

than left hemisphere infarctions even when rhe ipsilcsional arm is used and lesions arc matched for size.
These patients may have hypokinesia. Rehabilitation specialists have noted that it is more difficult to
rehabilitate patients with left hemiplegia than those with right hemiplegia. In addition, patients with left
hemiplegia are more likely to develop decubiti and pulmonary emboli. Both of these conditions may be
related to a global akinesia associated with right hemisphere dysfunction. Directional kinesia of the
limbs as determined by tasks such as those used by Heilman and Rothi (1985) were also more often
reported with right hemisphere lesions. The case of hemispatial limb akinesia also had a right
hemisphere lesion. Alrhough impersistence is often associated with bilateral hemispheric dysfunction,
when it is associated with unilateral hemispheric disease, it most commonly occurs with right
hemisphere lesions. Defective response inhibition of the eyes or arms may be seen with bilateral
hemispheric dysfunction, but when it is seen with unilateral hemispheric disease, it has been associated
with right hemispheric dysfunction. Lasdy, motor (or continuous) perseveration has also been reported
to be associated with right hemisphere dysfunction.

The term dominance implies that one hemisphere contains specialised processing systems or
representations (programs) cither that the other hemisphere does not contain or that are less
developed. The nondominant hemisphere therefore by itself is not fully competent to mediate a specific
activity. Although our discussion has provided evidence that the right hemisphere may be dominant for
inrentional control of the motor systems, this evidence is indirect. However, several studies in normal
subjects provide further evidence for right hemisphere intentional dominance. Although the anatomic
and physiologic basis for the right hemisphere's special role in intentional activity is unknown, the limbic
system, which plays a critical role in motivation, has two major outputs to the cortex: one from the
hippocampus and the other via the cingulate gyrus. Although bilateral medial temporal lobe lesions are
associated with profound amnesia, severe motivational or intentional disorders have not been
associated with these lesions. However, bilateral medial hemispheric lesions that involve the cingulate
gyrus are associated with a profound intentional disorder termed akinetic mutism, suggesting that the
cingulate gyrus provides motivational information to ncocortical areas. The right cingulate gyrus has
more input into the neocortex than the left.

Intrabemispheric Networks

The intrahemisphcric networks that are important in mediating intention have also not been fully
elucidated. However, studies of patients with focal lesions and studies of monkeys suggest that the
frontal lobes may play a critical role in intentional activity. For example, motor neglect of

122 APPROACH TO COMMON NEUROLOGICAL PROBLEMS

the limbs has been reported in monkeys from dorsolateral frontal lesions and although the ipsilesional
limbs are not as akinetic as the contralcsional limbs, there is a hypokinesia of the ipsilesional limbs as
measured by reaction times. Medial frontal lesions are also associated with limb akinesia. An ocular
directional akinesia can also be seen with dorsolateral frontal lesions, and a directional limb hypokinesia
may be seen with frontoparietal lesions (Heilman and Rothi 1985). In addition, motor impersistence is
most frequently seen with dorsolateral frontal lesions defective response inhibition of the eyes is seen
with dorsolateral frontal lesions, and defective response inhibition of the limbs is associated with medial
frontal lesions. Lastly, motor perseveration may also be associated with frontal lesions.

The frontal cortex has strong projections to the striatum. The dorsolateral frontal lobe projects to the
caudate, the supplementary motor area (SMA) projects to the putamen, and the cingulate gyrus projects
to the ventral striatum. The striatum projects to the internal portion of the globus pallidus and the pars
reticularis of the substantia nigra, which in turn projects to thalamic nuclei (e.g., VA, VL, and Ml)). These
thalamic nuclei project back the same area of the frontal cortex where this frontal, basal ganglia, and
thalamic loop was initiated including the dorsolateral frontal lobe, SMA, and cingulate gyrus. Based on
the previous discussion, it should not be surprising that intentional disorders may be associated with
diseases that affect both the basal ganglia and the thalamus. The most common disorder that induces
akinesia is Parkinson's disease. The akinesia associated with Parkinson's appears to be induced by a loss
of the dopaminergic neurons that project to the striatum. Dopamine antagonists may also produce
akinesia and dopamine agonists may reverse this akinesia. Thalamic lesions of VA/VL or the medial
nuclei such as centromedian parafascicularis can also induce akinesia. Not only do diseases that affect
frontal lobe cortex, basal ganglia, and thalamus induce intentional disorders, but these disorders,
especially akinesia, are also associated with diseases that affect the white matter that connects the
frontal lobes with these subcortical structures. Therefore akinesia is often associated with white matter
diseases such as arteriosclerotic encephalopathy (Binswanger's disease or multiple lacunar infarcts),
advanced multiple sclerosis, and hydrocephalus. Lastly, akinesia has also been reported with
temporoparietal lesions, in both monkeys and humans. Although the akinesia associated with basal
ganglia diseases such as Parkinson's disease appears to be endogenously evoked, the akinesias
associated with frontal and parietal cortical dysfunction appear to be exogenously evoked. Based on the
pathological evidence cited, one can postulate that the frontal lobes play a central role in human's
intentional network. The dorsolateral frontal lobes receive projections from both the parietal lobe,

which is a polymodal association cortex, and the multimodal primary association cortices. The frontal
lobes also have strong reciprocal connections to the cingulate gyrus, the medial thalamic nuclei, and
nonreciprocal connections with the striatum, which project to the globus pallidus and substantia nigra
and from there to the thalamus and hack to the cortex, as previously described. The frontal lobe's
connections with the inferior parietal lobe may provide the frontal lobe with stored knowledge (e.g.,
semantic and spatial information) and the limbic connections may provide the frontal lobe with
motivational information. Unimodal sensory and polymodal sensory association areas may provide the
frontal lobe with information about external stimuli that may call the organism to action. Afferents from
the mesencephalic and thalamic reticular system may be important for modulating arousal and
activation. Physiological studies have provided support for the role of the frontal lobes in intention-
motor activation. Neurons in the dorsolateral frontal lobe of monkeys who were trained to make a rapid
movement to a stimulus were recorded. When the animal was prepared to make a movement, the cells
were active. When the animal was not prepared to initiate a response, as determined by a delay in
response time, these cells were less active. Stimulus parameters did not affect these cells' activity,
suggesting that these cells were intentional neurons. The dorsolateral frontal lobes contained neurons
that discharge before purposeful saccades. Lesions of the frontal lobe destroy these intentional cells,
and in their absence, there is defective activation of the motor neurons.

The manner in which these frontal lobe intentional neurons influence the motor neurons has not been
definitely established. However, as discussed, the dorsolateral frontal lobe and the thalamic areas such
as the ventrolateral, dorsomedial, and intralaminar nuclei share anatomic connections with each other
and form a network with the basal ganglia, premotor, and motor cortices. Because lesions in these
structures (dorsolateral frontal lobe, basal ganglia, ventrolateral thalamus, medial thalamus) and
premotor areas (i.e., SMAs) induce akinesia, Watson, Valenstein, and Heilman (1981) posited that this
network mediates intentional activity.

We do not know whether the different forms of intentional activity we have discussed arc mediated by
the same network or whether different systems or subsystems mediate different forms of intentional
activity. Some of the intentional disorders we discussed may be related to the "release" of
phylogenctically more primitive systems. For example, neurons in the dorsolateral frontal lobes have a
role in the preparation of saccadic eye movements. One would predict that unilateral frontal lobe lesion
would induce a directional akinesia of the eyes. However, patients with frontal lobe lesions have been
reported unable to saccade away from a stimulus before they made a saccade toward the stimulus
(defective response inhibition or

INTENTIONAL MOTOR DISORDERS AND THE APRAX1AS 123

visual grasp). Directional akinesia and defective response inhibition would appear to be mutually
incompatible behaviors, but a patient with a unilateral frontal lobe lesion was assessed using a crossed
response task. Initially the patient showed both contra lesional sensory neglect and a directional
contralcsional akinesia. Subsequently, the patient was able to detect contralcsional stimuli and move his
eyes in a contralesional direction. However, in the crossed response task, when presented a
contralesional stimulus that was a signal to move the eyes in an ip si lesional direction, the patient often
incorrectly responded by first making a contralesional saccade before making an ipsilesional saccade.
Although it has been demonstrated that neurons in the dorsolateral frontal lobe have a role in preparing
for a saccade, collicular neurons can perform a similar function. Perhaps after frontal lobe damage,
which initially was associated with a directional akinesia, recovery was mediated by the colliculus.
Activity of the colliculus, however, unlike the frontal lobes, cannot be altered by task instructions.
Perhaps normally the dorsolateral frontal lobes exert an inhibitory influence on the colliculus, which is
absent aftct ftontal lesions. This inhibitory effect cannot be direct because no frontal lobe eye field cells
have been reported that arc tonically active except during saccades. However, the pars reticulata of the
substantia nigta projects to the colliculus and has tonic activity. The frontal lobe may influence the
substantia nigra through its connections to the caudate. A similar release of inhibition may also be
responsible for other nonocular defects whereby the brain-damaged patients cannot either withhold or
terminate responses.

Clinical Pathology

Intentional disotders can be caused by any neurological disease that impairs the systems we have
discussed. Neoplasms of the frontal lobes, the cingulatc corpus callosum region and colloid cysts of the
third ventricle may all be associated with intentional disorders. However, vascular diseases including
multiple lacunae and Binswanger's disease may be the most common cause of intentional disotders.
Infarctions of the thalamus and ventral tegmental areas may also cause intentional disorders.
Hydrocephalus and drugs that block dopamine receptors such as neuroleptics may also cause intentional
disorders. Head trauma and central nervous system infections are other common causes of intentional
disorders. Some infections and inflammatory diseases that must be considered include acquired
immunodeficiency syndrome, syphilis, Lyme, prion diseases such as Creut/ieldt-Jakob disease, chronic
meningitis (e.g., fungal, sarcoid), Whipple's, and progressive multifocal leukoencephalopathy. There are
many degenerative diseases that also may present with intentional disorders. These include Pick's
disease, and

Pick's disease without Pick's bodies (frontotcmporal dementia), olivopontocerebellar atrophy,

progressive supranuclear palsy, corticobasal degeneration, striatonigral degeneration, Shy-Drager
syndrome, Parkinson's disease, Huntington's disease, Behcet's disease, and ftontal lobe dementia
associated with motor neuron disease. Demyelinating diseases such as multiple sclerosis and the
leukodystrophies may also have intentional components. Lastly, there are several toxic/metabolic
diseases that may he associated with intentional disorders. These include chronic alcoholism with
alcohol dementia and MarchiafavaBignami disease, vitamin Bj2 deficiency, hypothyroidism, anoxia,
hypoparathyroidism with basal ganglia calcification, Wilson's disease, and status dysmyelinatus (iton
deposition in the basal ganglia with rigidity, athetosis, and mental degeneration).

PRAXIC (HOW) DISORDERS

The praxic programs provide several types of instructions:

1. How to position one's limb when petfotming skilled movements, including working with tools and
objects. 2. How to move the limb in space or the spatial trajectory of the skilled movement. This
program may contain both alloccmric (in relation to the object upon which the organism is acting) and
egocentric (in relation to the organism's own body) information. 3. How to orient the limb toward the
target of the limb's action, 4. How tapidly to move in space, or the timing of a skilled movement. 5. How
to imitate a movement. 6. How to solve mechanical ptoblems. 7. How to order components of an act to
achieve a goal.

Disorders of this how ot praxic system are called apraxias (Table 10.1). A loss of the ability to make
precise and independent movement (i.e., a loss of deftness) is called limb-kinetic apraxia. Failure to
correctly position a limb, move the limb correctly in space, and properly orient the limb is called IMA.
Patients with IMA also make temporal errors. There are patients, however, who perform imitation
worse than they gesture to command. These patients have what is called conduction apraxia. Patients
with disassociation apraxia may be impaired when attempting to perform skilled movements in
response to stimuli in one modality (e.g., verbal command) but be able to cotrectly perform movements
in response to stimuli of a different modality (e.g., seeing a tool). The inability to solve mechanical
problems is termed conceptual apraxia. Lastly, the inability to cotrectly order a series of movements is
termed ideational apraxia. In the following sections, we discuss each of these disorders.

Although limb apraxia is defined as an inability to correctly perform skilled movements with a limb,
when

124 APPROACH TO COMMON NEUROLOGICAL PROBLEMS

Table 10.1: Error types associated with each of the apraxii: syndromes

Apraxia Postural l'.<i»t»iaitd Movement Discrimination Imitation Series Mechanical type orientation
comprehension knowledge

Ideomotor Anterior -H-+ +++ +++ — ++ — Posterior +++ +++ +++ +++ ++ Conduction + + -I- — +++ — —
Dissociation +++- +++ +++ — — — — Ideation — — — — — Mr — Conceptual — — — — — —

Note: This tahle lists the error types that define each apraxia syndrome. However, often patients may
have more than one apraxic disorder. +++ = severe; ++ = moderate; + = less severe.

this inability to perform skilled movements is not caused by sensory loss or by more elemental motor
disorders such as weakness, tremors, dystonia, chorea, ballismus, athetosis, myoclonus, ataxia, and
seizures. Patients with severe cognitive, memory, motivational, and attentional disorders may also have
difficulty performing skilled acts. Whereas the presence of these disorders does not preclude that the
patient also has apraxia, before diagnosing apraxia, the clinician should be certain that these behavioral
disorders do not fully account for the patient's inability to perform skilled acts. Apraxia often goes
unrecognized and there are several possible reasons for poor recognition. The apraxia associated with
hemispheric injury such as strokes and trauma is often associated with an injury to the dominant
hemisphere. Thus these patients often have a hemiparesis of theit preferred arm and hand. When these
patients attempt to perform skilled acts with their nonpreferred arm and find that they are impaired,
they may attribute their difficulty to premorbid clumsiness of the nonpreferred arm. However, even
when these patients are able to use their preferred arm, apraxic patients may be anoSOgnosic for their
deficits. Unfortunately, many health professionals also do not test for limb apraxia, and they are not
fully aware of the nature of errors associated with apraxia.

Limb apraxia has been noted to be a heterogeneous group of disorders with both different clinical
pictures and anatomic substrates. In the following sections, we discuss several forms of limb apraxia.
These types are defined by both the nature of errors made by the patient and the means by which these
errors are elicited. Liepmann {1920) was the first to systematically study limb apraxia. He discussed
three types of limb apraxia: melokinetic (or limb kinetic), ideomotor, and ideational. In addition to
describing these forms of apraxia, we also discuss three other forms of apraxia which we have called
disassociation apraxia, conduction apraxia, and conceptual apraxia. Although dressing and
constructional apraxia are disorders of learned skills that do involve limb use, these disorders are often
associated with neglect and visual perceptual disorders, so we do not discuss these here.

Limb-Kinetic Apraxia

Patients with limb-kinetic apraxia demonstrate a loss of deftness or the ability to make finely graded,
precise, independent finger movements.

Testing

There are several means by which deftness can be tested. In most patients, both hands should be
tested, A small flat object such as a dime may be placed on a table and the patient is asked to pick up
the dime. Patients with limb-kinetic apraxia will not be able to use a pincher grasp, which requires
independent movements of the thumb and index finger, to pick up the dime. Another aspect of deftness
can be tested by measuring rapid finger tapping and by tests that use a pegboard (e.g., Purdue). We
have found that one of the most sensitive bedside tests is asking patients to rotate a coin between their
thumb, index finger, and middle finger as rapidly as they can. Patients with limbkinetic apraxia have
trouble rotating the coin.

Pathophysiology

Limb-kinetic apraxia most often occurs in the limb contralateral to a hemispheric lesion. Monkeys with
lesions confined to the corticospinal system do not show severe weakness but have difficulty making
independent finger movements including the pincher grasp. In the clinic, however, patients with limb-
kinetic apraxia often have injured their premotor cortex. Recent studies have revealed that when limb-
kinetic apraxia is induced by injury to the hemisphere opposite the preferred hand, limb-kinetic apraxia
may also be present in the nonpreferred hand, suggesting that the dominant hemisphere may in part
have ipsilateral projections.

Ideomotor Apraxia (IMA)

IMA is probably the most common type of apraxia. As discussed in a later section, when patients with

INTENTIONAL MOTOR DISORDERS AND THE APRAXIAS 125

IMA perform learned skilled movements including the performance of pantomimes, imitations, and
using actual objects, they make spatial and temporal errors.

Testing

When possible, both the right and the left arm and hand should be tested. When one arm is weak or has
another motor disorder that would preclude testing, the nonparctic limb should be tested. Testing of
praxis involves selectively varying input as well as varying task demands, When possible, the same items
should be used for all subtests. First, patients should be requested to pantomime to verbal command
(e.g., "Show me how you would use a bread knife to cut a slice of bread"). Both transitive (i.e., using
tools and instruments) and intransitive gestures (i.e., communicative gestures such as waving good-bye)
should be tested. Independent of the results of the gesture to command tests, patients should be asked
to imitate the examiner performing both meaningful and meaningless gestures. The patient should also
be allowed to hold actual tools or objects and to demonstrate how to use the tool or object. In addition
to having a patient pantomime to a verbal command, the examiner may want to show the patient
pictures of tools or objects and have the patient pantomime in response to these stimuli. The examiner
may also want to show the patient real tools or the objects that tools work on (e.g., nail) and without
having the patient hold the tool or object request that the patient pantomime the action associated
with the tool or object. It may be valuable to see whether the patient can name or recognize transitive
and intransitive pantomimes made by the examiner and discriminate between well and poorly
performed pantomimes.

When performing skilled acts, patients with IMA make ptimarily spatial and temporal production errors.
Spatial errors can be divided into several subtypes including postural (or internal configuration), spatial
movement, and spatial orientation. Regarding postural errors, Goodglass and Kaplan (1963) noted that
when apraxic patients are asked to pantomime, they often used a body part as the tool. For example,
when patients with IMA are asked to pantomime using a pair of scissors, they may use their fingers as if
they were the blades. Many normal subjects make similar errors and it is important that the patient be
instructed not to use a body part as a tool. Unlike normal subjects who imptove with these instructions,
patients with IMA may continue using their body patts as tools (Raymer et al. 1997). When not using
their body parts as tools, patients with IMA will often fail to cortectly position their hands as if they were
holding the tool or object.

When norma] subjects are asked to use a tool, they will orient that tool to an imaginary target of that
tool's action. Patients with IMA often fail to correctly orient their foreiimbs to an imaginary target. For
example, when asked to pantomime cutting a piece of paper in half

with scissors, rather than keeping the scissors oriented in the sagittal plane, cither the scissors may be
oriented laterally (Rothi et al. 1988) or the scissors may not maintain any consistent plane of movement.
When patients with IMA attempt to make a learned skilled movement, they will often make the correct
core movement {e.g., twisting, pounding, cutting), but the trajectory of their limb through space is often
incorrect (Rothi et al. 1988; Poizner et al. 1990). These spatial trajectory errors are caused by incorrect
joint movements. Apraxic patients will often stabilize a joint that they should be moving and move joints
they should not be moving, For example, when pantomiming the use of a screwdriver, patients with IMA
may rotate their arm at the shoulder and fix their elbow. Shoulder rotation moves the hand in arcs when
the hand should be rotating on a fixed axis. When multiple joint movements must be coordinated,
patients with apraxia may be unable to coordinate multiple joint movements to get the desired spatial
trajectory. For example, when asked to pantomime slicing bread with a knife, the shouldet and elbow
joints must be alternately flexed and extended. When the joint movements are not well coordinared,
these patients may make primarily chopping or stabbing movements.

Poizner et al. (1990) have noted that patients with IMA may also make timing errors including a long
delay before initiating a movement and brief multiple stops (stutteting movements). When normal
subjects make a curved movement, they reduce the speed of their movement, and when they move in a
straight line, they increase the speed of their movement. Patients with IMA, howevet, do not
demonstrate a smooth sinusoidal hand speed when performing cyclic movements such as cutting with a
knife.
Path op bysiology

In right-handed individuals IMA is almost always associated with left hemisphere lesions, but in left-
handed people, IMA is usually associated with right hemisphere lesions. IMA is associated with lesions in
a variety of structures including the corpus callosum, the inferior parietal lobe, and the premotor areas.
IMA has also been reported with subcortical lesions that involve the basal ganglia and white matter. We
discuss each of these anatomic areas and also attempt to develop a model of how the brain mediates
learned purposive movements.

Lesions of the Corpus Callosum. A male patient was described by I.iepmann and Mass (1907) with a right
hemiparesis from a lesion of the pons. The patient also had a lesion of his corpus callosum. This patient
was unable to correctly pantomime to command with his left atm. Because this patient had a right
hemiparesis, his right hand could not be tested. Since the work of Broca, it has been known that the left
hemisphere of right-handed people is dominant for language. The patient's inability to

126 APPROACH TO COMMON NEUROLOGICAL PROBLEMS

p.um>mime could lie associated with a disconnection between language and motor areas such that the
left hemisphere that mediates comprehension of the verbal command could not influence the right
hemisphere's motor areas that are responsible for controlling the left hand. This patient, however, could
also nor imitate gestures or correctly use actual tools or objects. Therefore a lan^iLi^-inmiii'
disconnection could 1101 account tor these findings. Thus Liepmann and Mass posited that the left
hemisphere of right handers contains movement formulas and that the callosal lesion in this patient
disconnected these movement formulas from the right hemisphere's motor areas.

Gazzaniga et al. (1967) also found that their patients with callosal disconnection could not correctly
pantomime to command with their left hand, but unlike the patient described by Liepmann and Mass,
their patients could imitate and correctly use actual tools and objects with their left hand. The preserved
ability to imitate and use actual tools and objects suggests that the inability to gesture to command in
these patients with callosal lesions was induced by a language-motor disconnection, rather than a
movement formula-motor disconnection. In addition, a disconnection between movement formula and
motor areas should produce spatial and temporal errors, but many of the errors made by the patient
discussed by Liepmann and Mass appeared to be content errors. Watson and Heilman (1983), however,
described a patient with an infarction limited to the body of the corpus callosum, Watson and Heilman's
patient had no weakness in her right hand and performed all tasks flawlessly with her right hand. In
contrast, with her left hand, she could not correctly pantomime to command, imitate, or use actual
tools. Immediately after her cerebral infarction, she made some content errors, but subsequently she
made primarily spatial and temporal errors. Her performance indicated that not only language but also
movement representations were stored in her left hemisphere and her callosal lesion disconnected
these movement formulas from the right hemisphere's motor areas.

Lesions of the Inferior Parietal Lobe. It has been proposed by Heilman et al. (1982) that the movement
re presentations or movement formulas are stored in the left parietal lobe of right handers and that
destruction of the left parietal lobe should induce not only a production deficit (apraxia) but also a
gesture comprehension-discrimination disorder. Apraxia induced by (1) premotor lesions, (2) lesions of
the pathways that connect premotor areas to motor areas, and (3) lesions of the pathways that lead to
the premotor areas from the parietal lobe may also cause production deficits. In contrast to parietal
lesions, however, these premotor lesions should not induce gesture comprehension and discrimination
disorders. Patients with anterior and posterior lesions have been tested (Heilman et al. 1982); although
both groups of patients were apraxic, the patients

with a damaged parietal lobe had comprehension and discrimination disturbances, but those without
parietal lesions did not. Liepmann and Mass (1907) proposed that handedness was related to the
hemispheric laterality of the movement representations. However, it is not unusual to see righthanded
patients with left hemisphere lesions who are not apraxic. Although it is possible that such patients'
lesions did not destroy one of the left hemispheric areas important for praxis, many of these patients'
lesions arc large and do involve the parietal lobe and other critical left hemisphere areas. As we
discussed, not all callosal lesions cause an IMA of the left hand. These results suggest that not all right
handers have movement formulas entirely represented in their left hemisphere. Some people may have
cither bilateral movement representations or even right hemisphere representations. Apraxia from a
right hemisphere lesion in a right hander is rare but has been reported, suggesting that hand preference
is not entirely determined by the laterality of the movement representations and may he multifactorial.
Whereas the laterality of the movement formula may be the most important factor, there are other
factors including deftness (i.e., speed, precision), strength, and even environmental factors.

Supplementary Motor Area Lesions. Muscles move joints, and motor nerves from the spinal cord
activate these muscles. The spinal motor nerves are acrivated by corticospinal neurons and the
corticospinal neurons are activated by the premotor areas. The premotor areas must instruct the motor
area on which neurons to fire and in what order these should he tired. For each specific skilled
movement there is a set of spatial loci that must be traversed in a specific temporal pattern. We
proposed that movement formulas represented in the inferior parietal lobe are stored in a three-
dimensional supramodal code (Heilman and Rothi 2003). For the corticospinal neurons to properly
activate the motor nerves, the stored spatial temporal knowledge has to be transformed into a motor
program. The medial premotor cortex or SMA appears to play an important role in mediating skilled
movements. Whereas electrical stimulation of the primary motor cortex induces simple movements,
SMA stimulation induces complex movements that may include the entire forelimb. The SMA receives
projections from parietal neurons and projects to motor neurons. SMA neurons discharge before
neurons in the primary motor cortex. Studies of cctebtal blood flow, an indicator of cerebral metabolism
and synaptic activity, have revealed that single repetitive movement increases activation of the
contralateral primary motor cortex, but complex movements increase flow in contralateral motor cortex
and bilaterally in the SMA. When subjects remain still and think about making complex movements,
blood flow is increased to the SMA but not rhe primary motor cortex. Watson et al. (1986)

INTENTIONAL MOTOR DISORDERS AND '1 UK APRAX1AS 127

reported several patients with left-sided medial frontal lesions that included the SMA who
demonstrated an IMA when tested with cither arm. Unlike patients with parietal lesions, these patients
could both comprehend questions or pantomimes and discriminate between correctly and incorrectly
performed pantomimes. Apraxia has also been reported to be associated with lesions of the convexity
premotor cortex. The convexity premotor cortex may be important in coordinating the synchronous
actions of multiple joints. Figure 10.1 provides a model for the action processing system we have
described thus far. In this figure the label "praxicon" is the theoretical store of the temporospatial
representations of learned skilled movements. When performing a skilled act, these representations are
transformed into innervatory patterns by SMA and convexity premotor cortex. By the term innervatory
patterns we mean the program that activates the motor neurons such that the extremity moves in the
correct spatial trajectory with the correct timing of each movement.

Conduction Apraxia

A patient was reported who, unlike patients with IMA was more impaired when imitating than when
pantomiming to command (Ochipa et al. 1994). Because this patient

was similar to the conduction aphasic who repeats poorly, the researchers termed this disorder
conduction apraxia.

Testing

Testing for conduction apraxia is the same as testing for IMA. Whereas most patients with IMA will
improve with imitation, patients with conduction apraxia appear to perform worse with imitation than
they do to command.

Patbnphysio logy

The patient with conduction apraxia in the study by Ochipa et al. (1994) could comprehend the
examiner's pantomimes and gestures. Wc therefore believe that the patient's visual system could access
the movement representations or what we have termed praxicons, and these movement
representations could activate semantics. It is possible that decoding a seen gesture requires accessing
different movement representations than programming an action. Therefore there may be two different
stores of movement representations, an input praxicon and an output praxicon. In the verbal domain a
disconnection of the hypothetical input and output lexicons induces conduction aphasia, and in the
praxis domain a disconnection between the input and output praxicons could induce conduct ion
apraxia.

FIGURE 10.1 Diagrammatical model of the praxis system. Degradation of action semantics (A) produces
conceptual apraxia. Lesions that prevent afferent stimuli from activating movement representations (B,
C, D) induce dissociation apraxia. Degradation of the movement representations (F.) causes ideomotor
apraxia with impaired gesture discrimination and comprehension. Dysfunction of the premotor cortex
(F) where the movement representations are converted to motor programs or innervatory patterns
induces an ideomotor apraxia with preserved discrimination and comprehension of transitive gestures.
Injury to the corpus eallosum (G) produces an ideomotor apraxia of the nonpreferred limb, and injury to
the motor cortex (H) induces a loss of independent finger movement and precision called limb-kinetic
apraxia. SMA = supplementary motor area.

128 APPROACH TO COMMON NEUROLOGICAL PROBLEMS

Wheteas the lesions that induce conduction aphasia are usually in the supramarginal gyrus, the arcuate
fasciculus, ot Wernicke's atea, the lesions that induce conduction apraxia arc unknown.

Dissociation Apraxia

Heilman (1973) described three patients who, when asked to pantomime to command, looked at their
hand but would not perform any recognizable actions. Unlike patients with ideomotot or conduction
apraxias described earlier, these patients' imitation and use of objects were flawless. Other researchers
not only reported patients similar to those reported by Heilman (1973) but also other patients who had
a similat defect in other modalities. For example, when asked to pantomime in response to visual or
tactile stimuli, they may have been unable to do so but could correctly pantomime to vetbal command.

Testing

The testing that is performed to assess for this disorder is the same as that used for IMA.

Pathopb ysiology

Callosai lesions may not only be associated with an IMA, but callosai disconnection may also cause
dissociation apraxia. The subjects of Gazzaniga, Bogcn, and Sperry (1967) and others had dissociation
apraxia of theit left hand. With their left hand they could not gesture normally to command but
perfotmed well with imitation and actual tools. Whereas language in these patients was mediated by
the left hemisphere, movement representations may have been bilaterally represented. Thetefore their
callosai lesion induced a dissociation apraxia only of the left hand because the verbal command could
not get access to the movement representations stored in the right hemisphere. Whereas the patient
with callosai dissociation apraxia will not be able to correctly perform skilled purposive movements of
the left arm to command, these patients can imitate normally and use actual tools with their left hand.
They perform normally because these tasks do not need vetbal mediation and the movement
representations stored in their right hemisphere can be activated by tactile or visual input.

Right-handed patients who have both language and movement formulas represented in their left
hemisphere may show a combination of dissociation and IMA with callosai lesions (Watson and Heilman
1983). When asked to pantomime with their left hand, they perform no recognizable movement
(disassociation apraxia), but when imitating or using actual rools, they may demonstrate the spatial and
temporal errors seen with IMA. Left banders may demonstrate an IMA without aphasia from a right
hemisphere lesion. These left handers are

apraxic because their movement tepresentations are stored in their right hemisphere and their lesions
destroyed these representations (Heilman 1973; Valenstein and Heilman 1979). These left handers were
not aphasic because language was mediated by their left hemispheres (as is the case in most left
handers). If these left banders had a callosai lesion, they may have demonstrated a dissociation apraxia
of theit left arm and an IMA of their right arm. These patients reported by Heilman (1973) and those of
othct studies probably have an intrahemispheric language-movement formula, visual-movement
formula, or somesrhetic-movement formula dissociation. The locations of the lesions that cause these
intrahemispheric dissociation apraxias are not known.

Ideational Apraxia

Unfortunately, use of the term ideational apraxia has been confusing, with the term erroneously used to
label a variety of disorders. For example, Heilman (197,3) used this term when he first desctibed
dissociation apraxia. Patients with IMA usually improved when using actual tools and objects, but other
researches have reported patients who made errors with the use of actual tools and objects, Heilman
(1973) also termed this disturbance ideational apraxia. Although the inability to use actual tools and
objects may be associated with a conceptual disorder, a severe production disotder may also impair
object use. Lastly, the term has also been used to desctibe patients who make conceptual errors. These
patients are discussed in the next section. The inability to catry out a series of acts or sequence of
actions, an ideational plan that leads to a goal, has also been called ideational apraxia. In this chapter,
we also define ideational apraxia as an inability to correctly sequence a scries of acts that lead to a goal.

Testing

Unfortunately there are no standatdized tests that assess fot ideational apraxia. To test for ideational
apraxia, the patients should be tested for their ability to perform multistep sequential tasks, bor
example, the examiner may place two slices of bread, mustard, a knife (not too sharp), several slices of
ham, and a sandwich bag in front of the patient and ask the patient to prepare a sandwich for work. If
the subject fails to perform each step in the correct order that subject may have ideational apraxia.

Path ophysioiogy

Ideational apraxia is most often associated with degenerative dementia but may also be associated with
focal lesions of the left hemisphere.

INTENTIONAL MOTOR DISORDERS AND THE Al'RAXIAS 129

Conceptual Apraxia

To perform a skilled act, we require two types of knowledge: conceptual knowledge and production
knowledge. Whereas dysfunction of the praxis production system induces IMA, defects in the
knowledge needed to successfully select and use the tools and objects we term conceptual apraxia.
Therefore patients with IMA make production errors (e.g., spatial and temporal errors), and patients
with conceptual apraxia make content and tool-selection errors. The patients with conceptual apraxia
may not recall the type of actions associated with specific tools, utensils, or objects (tool-object action
knowledge) and therefore make content errors (DcRenzi and Luccelli 1988; Ochipa, Rothi, and Heilman
1989). For example, when asked to demonstrate the use of a screwdriver either by pantomiming or
using the tool, the patient with the loss of tool-object action knowledge may pantomime a hammering
movement or use the screwdriver as if it were a hammer.

Content errors (i.e., using a tool as if it were another tool) can also be induced by an object agnosia.
However, Ochipa et al. (1989) reported a patient who could name tools (and therefore was not agnosic)
that he used inappropriately. Patients with conceptual apraxia may be unable to recall which specific
tool is associated with a specific object (tool-object association knowledge). For example, when shown a
partially driven nail, they may select a screwdriver rather than a hammer from an array of tools. This
conceptual defect may also be in the verbal domain such that when an actual tool is shown to a patient,
the patient may be able to name it (e.g., hammer), but when this patient with conceptual apraxia is
asked to name or point to a tool when its function is described, he or she cannot. The patient may also
be unable to describe the functions of tools.

Patients with conceptual apraxia may also have impaired mechanical knowledge. For example, if they
are attempting to drive a nail into a piece of wood and there is no hammer available, they may select a
screwdriver rather than a wrench or pliers (which are hard, heavy, and good for pounding) (Ochipa et al.
1992). Mechanical knowledge is also important for tool development and patients with conceptual
apraxia may also be unable to correctly develop tools (Ochipa et al. 1992).

Testing
In one test of associative knowledge (tool or object-action), patients may be shown tools such as a
screwdriver or objects that tools work on, such as nails, and asked to demonstrate the action that is
associated with this tool or object. Patients with conceptual apraxia may make content errors such that
they demonstrate the actions of tools other than the one they wetc asked to pantomime. In

another test of associative knowledge (tool-object), the patients may be shown an object such as a
screw and then are asked to select the proper tool from a group of five tools. To test mechanical
knowledge in patients with normal associative knowledge, one can show them an object such as a
partially driven in nail and have them select from five tools the one that would work best for completing
the task. The tools from which they may select could include a wrench, a knife, a hand saw, a
screwdriver, and scissors. Unfortunately, tests of tool fabrication require special equipment.

Pathophysiology

Licpmann (1920) thought that conceptual knowledge was located in the caudal parietal lobe and
DeRenzi and Luccelli (1988) placed it in the temporoparietal junction. The patient reported by Ochipa et
al. (1989) was left handed and rendered conceptually apraxic by a lesion in the right hemisphere,
suggesting that both production and conceptual knowledge have lateralized representations and that
such representations are contralateral to the preferred hand. Further evidence that these conceptual
representations are lateralized contralateral to the preferred hand comes from the observation of a
patient who had a callosal disconnection and demonstrated conceptual apraxia of the nonpreferred
(left) hand (Watson and Heilman 1983). More recently, it has been demonstrated that conceptual
apraxia in people who prefer their right hand is most often associated with left hemisphere lesions
(Heilman et al. 1997). Conceptual apraxia is perhaps most commonly seen m degenerative dementia of
the Alzheimer type (Ochipa et al. 1992). Although both IMA and conceptual apraxia co-occur, Ochipa et
al. also noted that the severity nl conceptual and IMA did not always correlate. The observation that
patients with IMA may not demonstrate conceptual apraxia and patients with conceptual apraxia may
not demonstrate IMA provides support for the postulate that the praxis production and praxis
conceptual systems are independent. Although Heilman et al. (1997) demonstrated that lesions located
in the hemisphere opposite the preferred hand induce conceptual apraxia, these investigators did not
find a specific anatomic area that appeared critical, suggesting that conceptual representations might be
widely distributed.

Clinical Pathology

The different forms of limb apraxia discussed in this chapter are most commonly associated with strokes
and degenerative dementia of the Alzheimer's and Pick's types. Aptaxia may be seen with many other
diseases of the central nervous system including tumors and trauma. Certain forms of apraxia (i.e., limb
kinetic and ideomotor)

130 APPROACH TO COMMON NEUROLOGICAL PROBLEMS

may be the presenting symptom of basal ganglia disorders such as corticobasal degeneration.

CONCLUSION

Although cognitive-motor disorders are a common, disabling, and enduring sequelae of brain damage,
these may be the least recognized neuropsychological disorders associated with cerebral disease. The
proper evaluation and diagnosis of these cognitive-motor disorders may not only aid in the diagnosis of
the underlying neurological disease but knowledge of these disabilities may help the physician provide
the patient and caregiver with information that may help optimize residual resources and thereby
improve the patient's quality of life. When possible, the underlying disease should he treated. Although
there are no proven specific pharmacologic treatments, cogni