APHASIA, APRAXIA, AND AGNOSIA

APHASIA
Left-hemisphere dominance for speech and language applies to more than 95%
of all populations studied. Right-hemisphere dominance in a right-handed person is
rare enough to prompt case reports in the literature. Most left-handed persons show
some disturbance in speech and language from either left- or right-hemisphere
lesions, making predictions for hemisphere dominance for left-handed persons
difficult to predict on an individual basis. The most predictable site for disturbances
in speech and language are the regions in and bordering on the sylvian fissure of the
hemisphere controlling the hand preferred for skilled movements. The farther from
this zone that the lesion occurs, the less the lesion disturbs speech and language. The
disturbances in speech and language resulting from a lesion form a group of disorders
known as the aphasias.
The popular classifications of aphasia are based on classic views that the front
half of the brain performs motor or executive functions and the back half sensory or
receptive functions, with the two regions connected by pathways in the white matter.
Classically, frontal lesions have been inferred to cause motor aphasia, those affecting
the posterior regions cause sensory aphasia, and those interrupting the pathways
between the frontal and posterior regions cause conduction aphasia. This formulation
posits an anatomic functional loop with an afferent portion from the eyes and ears
connecting to the visual and auditory system, an intrahemispheral portion through the
white matter connecting the temporal with the frontal lobes (the arcuate fasciculus),
and an efferent portion from the frontal lobes to the mouth and hand permitting, in its
simplest function, words heard to be repeated aloud and words seen to be copied
manually. Apart from the crude replication of sounds heard and shapes seen, of which
any person even ignorant of the language conveyed by the sounds or forms is
capable, meaning is thought to be conveyed to these shapes and sounds by access of
the perisylvian region to the rest of the brain through intrahemispheral and
transcallosal pathways. Interruption of these linkage pathways is postulated to
produce transcortical sensory aphasia, in which words heard are repeated aloud or
copied without comprehension, or transcortical motor aphasia, in which words can be
repeated and copied but no spontaneous communication by conversation or writing
occurs. Other “disconnections” have also been proposed for pathways to or from the
periphery, which presumably would be in the subcortical white matter.
Disconnections of incoming pathways bearing visual lexical information yield pure
alexia; those of pathways conveying auditory material cause pure word deafness. The
combination of these two disconnections causes subcortical sensory aphasia.
Disconnections of efferent pathways from the motor speech zones produce pure word
mutism or subcortical motor aphasia.
Although these generalizations are widely held to account for the major
principles of cerebral organization, uncritical acceptance of the expected effects of
certain lesion locations or prediction of lesion locations by the clinical features, as
based on the classical formulas, often proves misleading for clinicians seeking the
site and cause of a clinical disorder of speech and language. To avoid this problem,

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the material that follows emphasizes the clinical features that aid in local lesion
diagnosis, with less emphasis on the classical concepts.
Motor Aphasias
An acute focal lesion (the most frequent and best known being an infarct)
involving any portion of the insula or the individual gyrus forming the upper banks of
the opercular cortex (from the anteroinferior frontal region to the anterior parietal)
acutely disrupts the acquired skills involving the oropharyngeal, laryngeal, and
respiratory systems that mediate speech, causing mutism. Writing may be preserved,
although it is usually confined to a few simple words. Comprehension of words heard
or seen is generally intact because these functions are largely subserved by posterior
regions. The speech that emerges within minutes or days of the onset of motor
aphasia consists mostly of crude vowels (dysphonia) and poorly articulated
consonants (dysarthria). Disturbed coordination (dyspraxia) of speaking and
breathing alters the rhythm of speech (dysprosody). This faulty intonation, stress, and
phrasing of words and sentences is known collectively as speech dyspraxia. The
language conveyed through this speech is usually only slightly disturbed, but the
grammatic forms used in speaking or writing are sometimes simplified.
The more anterior that the lesion is along the operculum, the more speech
dyspraxia predominates, especially with involvement of the inferior frontal region
(Broca area) located adjacent to the sensorimotor cortex. When the sensorimotor
cortex itself is affected, dysarthria and dysphonia are more prominent than
dysprosody and dyspraxia. The errors in pronunciation may make it impossible to
understand the language conveyed by the patient's speech, but they are not, strictly
speaking, a language disorder.
When an acute lesion occurs more posteriorly along the sylvian operculum, the
precise sensorimotor control over the positioning of the oropharynx may be impaired,
causing unusual mispronunciations as well as mild dysphasia. The disturbed
pronunciation is not simple dysarthria. Instead, the faulty oropharyngeal positionings
yield sounds that differ from those intended (e.g., “dip” is said instead of “top”). The
errors, analogous to the typing errors of a novice unfamiliar with the typewriter
keyboard, are called literal paraphasias. The listener may mistake the utterances as
language errors (paraphasias) or may be impressed with some of the genuine
paraphasias and give the condition the name conduction aphasia (see the following).
The patient's comprehension is intact despite the disordered pronunciation.
Stroke is the most common cause of acute lesions. The arrangement of the
individual branches of the upper division of the sylvian artery favors the wide variety
of focal embolic obstructions that produce this remarkable array of syndromes. The
more specific that the speech abnormality is, the more limited is the focal infarction.
Because the sensorimotor cortex is part of the same arterial supply of the upper
division of the middle cerebral artery, the larger infarcts and other disorders such as
basal ganglia hemorrhages, abscesses, large tumors, and acute encephalitis usually
cause accompanying contralateral hemiparesis and hemisensory syndromes, making
the diagnosis of perisylvian disease fairly easy. One disorder, known as primary
progressive aphasia, appears to be an unusual form of atrophy, causing mainly a
relentless decline in speech and language function without the accompanying motor,
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sensory, visual, or other clinical evidence of a large lesion affecting the main
pathways serving these functions.
For speech and language, the smaller and more superficial that the injury is, the
briefer and less severe is the disruption. Rapid improvement occurs even when the
lesion involves sites classically considered to cause permanent speech and language
disturbances, such as the foot of the third frontal gyrus (Broca area). The larger the
acute lesion, the more evident is dysphasia and the longer is the delay before speech
improves. In larger sylvian lesions, dysphasia is evident in disordered grammar,
especially when tests involve single letters, spelling, and subtleties of syntax.
Problems with syntax occur not only in speaking and writing but also in attempts to
comprehend the meaning of words heard or seen. For example, the word “ear” is
responded to more reliably than is “are,” “cat” more than “act,” and “eye” more than
“I.” The language content of spontaneously uttered sentences is condensed, missing
many of the filler words, causing telegraphic speech, or agrammatism. Agrammatism
is an important sign of a major lesion of the operculum and insula. When the
causative lesion involves many gyri, as with large infarcts, hemorrhages, and
neoplasms or abscesses large enough to produce unilateral weakness, the reduction of
both speech and comprehension is profound and is called total aphasia. Within weeks
or months in cases of infarction and hemorrhage, comprehension improves,
especially for nongrammatic forms, and speaking and writing seem to be affected
more than listening and reading. This last syndrome, in which dysphasia is most
evident in speaking and writing, is known as motor aphasia; the eponym Broca
aphasia is often used. This syndrome emerges from an initial total aphasia as a late
residual. It is not the usual acute syndrome of a circumscribed infarction, even when
the lesion is confined to the pars opercularis of the inferior frontal gyrus (Broca area).
Sensory Aphasias
A different set of acute symptoms follows acute focal lesions of the posterior
half of the temporal lobe and the posterior parietal and lateral occipital regions.
Infarction is also the usual cause of the discrete syndromes, while hemorrhage,
epilepsy, and acute encephalitis may account for sudden major syndromes. Even
large lesions in these areas are usually far enough removed from the sensorimotor
cortex so that hemiparesis and speech disturbances (e.g., dysprosody, dysarthria, or
mutism) are only occasionally part of the clinical picture.
In patients with large posterior lesions, the effects are almost the reverse of the
insular-opercular syndromes: Syntax is better preserved than semantics; speech is
filled with small grammatic words, but the predicative words (i.e., words that contain
the essence of the message) are omitted or distorted. Patients vocalize easily, engage
in simple conversational exchanges, and even appear to be making an effort to
communicate; however, little meaning is conveyed in the partial phrases, disjointed
clauses, and incomplete sentences. In the most severe form, speech is
incomprehensible gibberish. Errors take the form of words that fail to occur
(omissions), are mispronounced as similar-sounding words (literal paraphasias), or
are replaced by others that have a similar meaning (verbal paraphasias). A similar
disturbance affects understanding words heard or seen. These language disturbances
may require prolonged conversation to be revealed in mild cases. Because this
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disturbance in language contrasts with motor aphasia, it is often labeled as sensory
aphasia, or Wernicke aphasia, but neither syndrome is purely motor or sensory.
The posterior portions of the brain are more compact than the anterior portions.
As a result, large infarctions or mass lesions from hemorrhage, abscess, encephalitis,
or brain tumors in the posterior brain tend to cause similar clinical disorders with few
variations in syndrome type. Contralateral hemianopia usually implies a deep lesion.
When hemianopia persists for longer than about 1 week, the aphasia is likely to
persist.
Highly focal lesions are uncommon and, when present, usually mean focal
infarction. Those limited to the posterior temporal lobe usually produce only a part of
the larger syndrome of sensory aphasia. Speech and language are only slightly
disturbed, reading for comprehension may pass for normal, but auditory
comprehension of language is grossly defective. This syndrome was classically
known as pure word deafness. Patients with this disorder also usually reveal verbal
paraphasias in spontaneous speech and disturbed silent reading comprehension. This
syndrome might be better named the auditory form of sensory aphasia. It has a good
prognosis, and useful clinical improvement occurs within weeks; some patients are
almost normal.
A similarly restricted dysphasia may affect reading and writing, more so than
auditory comprehension, because of a more posteriorly placed focal lesion that
damages the posterior parietal and lateral occipital regions. Reading comprehension
and writing morphology are strikingly abnormal. This syndrome has traditionally
been known as alexia with agraphia, but spoken language and auditory
comprehension are also disturbed (although less than reading and writing). A better
label might be the visual form of sensory aphasia. It also has a good prognosis.
The more limited auditory and visual forms of Wernicke aphasia are rarely
produced by mass lesions from any cause and tend to blend in larger lesions. Whether
the major syndrome of sensory aphasia is a unified disturbance or a synergistic result
of several separate disorders has not been determined.
Amnestic Aphasia
Anomia or its more limited form dysnomia is the term applied to errors in tests
of naming. Analysis requires special consideration because the mere occurrence of
naming errors is of less diagnostic importance than is the type of error made. In all
major aphasic syndromes, errors in language production cause defective naming
(dysnomia), taking the form of paraphasias of the literal (e.g., “flikt” for “flight”) or
verbal (e.g., “jump” for “flight”) type. For this reason, it is not usually of diagnostic
value to focus a clinical examination on dysnomias alone, as they have little value as
signs of focal brain disease.
A pattern known as amnestic dysnomia has a greater localizing value. Patients
act as though the name has been forgotten and may give functional descriptions
instead. Invoking lame excuses, testimonials of prowess, claims of irrelevance, or
impatience, patients seem unaware that the amnestic dysnomia is a sign of disease.
The disturbance is common enough in normal individuals, but in those with disease it
is prominent enough to interfere with conversation. Amnestic aphasia, when fully
developed, is usually the result of disease of the deep temporal lobe gray and white
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matter. A frequent cause is Alzheimer disease, in which atrophy of the deep temporal
lobe occurs early, and forgetfulness for names may be erroneously attributed to old
age by the family. Identical symptoms may occur in the early stages of evolution of
mass lesions from neoplasms or abscess but are rarely a sign of infarction in the deep
temporal lobe. Other disturbances in language, such as those involving grammar,
reading aloud, spelling, or writing, are usually absent, unless the responsible lesion
encroaches on the adjacent temporal parietal or sylvian regions. When due to a mass
lesion, the disturbance often evolves into the full syndrome of Wernicke aphasia.
Thalamic Lesions and Aphasia
An acute deep lesion on the side of the dominant hemisphere may cause
dysphasia if it involves the posterior thalamic nuclei that have reciprocal connections
with the language zones. Large mass lesions or slowly evolving thalamic tumors
distort the whole hemisphere, making it difficult to recognize the components of the
clinical picture. Small lesions are most often hematomas and are the usual cause of
the sudden syndrome. As in delirium, consciousness fluctuates widely in this
syndrome. As it fluctuates, language behavior varies from normal to spectacular
usage. The syndrome may be mistaken for delirium due to metabolic causes (e.g.,
alcohol withdrawal). It is also important in the theory of language because the
paraphasic errors are not due to a lesion that affects the cerebral surface, as was
claimed traditionally. Prompt computed tomography usually demonstrates the
thalamic lesion.
APRAXIA
The term apraxia (properly known as dyspraxia because the disorder is rarely
complete) refers to disturbances in the execution of learned movements other than
those disturbances caused by any coexisting weakness. These disorders are broadly
considered to be the body-movement equivalents of the dysphasias and, like them,
have classically been categorized into motor, sensory, and conduction forms.
Limb-kinetic or Innervatory Dyspraxia
This motor form of dyspraxia occurs as part of the syndrome of paresis caused
by a cerebral lesion. Attempts to use the involved limbs reveal a disturbance in
movement beyond that accounted for simply by weakness. Because attempted
movements are disorganized, patients appear clumsy or unfamiliar with the
movements called for in tasks such as writing or using utensils. Although difficult to
demonstrate and easily overlooked in the presence of the more obvious weakness,
innervatory dyspraxia is a useful sign to elicit because it indicates that the lesion
causing the hemiparesis involves the cerebrum, presumably including the premotor
region and other association systems. Dyspraxias of this type are thought to be caused
by a lesion involving the cerebral surface or the immediately adjacent white matter;
apraxia is not seen in lesions that involve the internal capsule or lower parts of the
neuraxis.
Ideational Dyspraxia
Ideational dyspraxia is a different type of disorder altogether. Movements of
affected body parts appear to suffer from the absence of a basic plan, although many
spontaneous actions are easily carried out. This disorder is believed to be analogous
to sensory aphasia (which features a breakdown of language organization despite
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continued utterance of individual words). The term is apparently derived from the
simplistic notion that the lesion disrupts the brain region containing the motor plans
for the chain of individual movements involved in complex behaviors such as
feeding, dressing, or bathing. To the observer, patients appear uncertain about what to
do next and may be misdiagnosed as confused. The lesion causing ideational
dyspraxia is usually in the posterior half of the dominant hemisphere. The coexisting
sensory aphasia often directs diagnostic attention away from the dyspraxia, which,
like innervatory dyspraxia, is only rarely prominent enough to result in separate
clinical recognition.
Ideomotor Dyspraxia
This form of dyspraxia is frequently encountered. The term derives from the
notion that a lesion disrupts the connections between the region of the brain
containing ideas and the region involved in the execution of movements. The
disturbance is analogous to conduction aphasia: Motor behavior is intact when
executed spontaneously, but faulty when attempted in response to verbal command.
For movements to be executed by the nondominant hemisphere in response to
dictated commands processed by the dominant hemisphere, the lesion could involve
the presumed white-matter pathways through the dominant hemisphere to its motor
cortex, the motor cortex itself, or the white matter connecting to the motor cortex of
the nondominant hemisphere through the corpus callosum. Because so many
presumed pathways are involved, ideomotor dyspraxia is common. The syndrome is
most frequently encountered in the limbs served by the nondominant hemisphere
when the lesion involves the convexity of the dominant hemisphere. Concomitant
right hemiparesis and dysphasias, usually of the motor type, often occupy the
physician's attention so that the ideomotor dyspraxia of the nondominant limbs passes
without notice. Dysphasia may make it impossible to determine whether ideomotor
dyspraxia is present, but, when mild, dyspraxia can be demonstrated by showing that
patients cannot make movements on command, although they can mimic the behavior
demonstrated by the examiner and execute it spontaneously at other times. The
disturbances are most apparent for movements that involve the appendages (e.g.,
fingers, hands) or oropharynx. Axial and trunk movements are often spared.
AGNOSIA
When patients with a brain lesion respond to common environmental stimuli as
if they had never encountered them previously, even though the primary neural
pathways of sensation function normally, this disorder is called an agnosia. Because
the disturbance seen in response to a few stimuli is assumed to apply to others with
similar properties, agnosias embrace specific classes of stimuli (e.g., agnosia for
colors) or more global disturbances for a form of sensation (e.g., visual or auditory
agnosia).
Such sweeping generalizations are usually unjustified in practice because
careful examination often shows that the abnormality can be explained in some other
way, including genuine unfamiliarity with the stimuli, faulty discrimination due to
poor lighting, poor instructions from the examiner, or an overlooked end-organ
failure (e.g., peripheral neuropathy, otosclerosis, cataracts). Faulty performance may
also result from a dysphasia or dyspraxia. Errors arising from a dysphasia are easily
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understood; a dyspraxia may be more difficult to recognize. Sometimes, it is not clear
whether dyspraxia produces agnosia, or vice versa. Posterior parietal lesions arising
from cardiac arrest, neoplasm, or infections may impair cerebral control of the
precise eye movements involved in the practiced exploration of a picture or other
complex visual stimuli; the resulting chaotic but conjugate eye movements prevent
the victim from naming or interacting properly with the stimuli. This abnormality
seems to be a form of cerebral blindness (which patients may deny) and is an
essential element of Balint syndrome (biparietal lesions causing disordered ocular
tracking, bilateral hemineglect, and difficulties deciphering complex thematic
pictures). Similar disturbances in skilled manual manipulation of objects may be
documented in anterior parietal lesions that interfere with the ability to name or use
an object properly.
When all these variables have been taken into account, a small group of
patients may remain for whom the term agnosia may apply. Some neurologists deny
that such a state exists, the errors presumably resulting from a combination of
dementia and impaired primary sensory processing; others postulate anatomic
disconnections due to lesions that lie between intact language areas and intact
cerebral regions responsible for processing sensory input.
Two claimed clinical subtypes of visual agnosia embrace these differing
theories of agnosia: Apperceptive agnosia refers to abnormality in the discrimination
process, and associative agnosia implies an inability to link the fully discriminated
stimulus to prior experience in naming or matching the stimulus to others. Clinically,
patients with apperceptive visual agnosia are said to fail tests of copying a stimulus or
cross-matching a stimulus with others having the same properties (i.e., different
views of a car), whereas patients with the associative form can copy and cross-match;
neither type can name the stimulus as such. Disturbances of the ability to respond to
stimuli have been described for colors (color agnosia) and for faces (prosopagnosia).
Although the definition of agnosia requires that a patient treat the stimuli as
unfamiliar, the errors often pass almost unnoticed (i.e., dark colors are misnamed for
other dark colors; names of famous people are mismatched with their pictures). In the
auditory system, a similar disturbance may occur with a normal audiogram in
discrimination of sounds (cortical deafness or auditory agnosia), including words
(pure word deafness or auditory agnosia for speech). A patient's inability to recognize
familiar objects by touch while still being able to recognize them by sight is referred
to as tactile agnosia.
In practical clinical terms, the clinical diagnosis of agnosia warrants
consideration when patients respond to familiar stimuli in an unusually unskillful
manner, treat them as unfamiliar, or misname them for other stimuli having similar
hue, shape, or weight, but do not show other signs of dysphasia or dyspraxia in other
tests. The special testing is time-consuming but may yield a diagnosis of a disorder
arising from lesions of the corpus callosum, the deep white matter, or the cerebrum
adjacent to the main sensory areas. The usual cause is atrophy or metastatic or
primary tumor. When the disorder develops further, the more obvious defects occur
in formal confrontation visual field testing, and the “agnosia” is even more difficult
to demonstrate.
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