Aphasia

Aphasia (pronounced /əˈfeɪʒə/ or pronounced /əˈfeɪziə/), from the Greek root word "aphatos", meaning
speechless, is an acquired language disorder in which there is an impairment of any language modality. This
may include difficulty in producing or comprehending spoken or written language.

Traditionally, aphasia suggests the total impairment of language ability, and dysphasia a degree of
impairment less than total. However, the term dysphasia is commonly confused with dysphagia, a
swallowing disorder, and thus aphasia has come to mean both partial and total language impairment in
common use.

Depending on the area and extent of brain damage, someone suffering from aphasia may be able to speak
but not write, or vice versa, or display any of a wide variety of other deficiencies in language comprehension
and production, such as being able to sing but not speak. Aphasia may co-occur with speech disorders such
as dysarthria or apraxia of speech, which also result from brain damage.

Aphasia can be assessed in a variety of ways, from quick clinical screening at the bedside to several-hour-
long batteries of tasks that examine the key components of language and communication. The prognosis of
those with aphasia varies widely, and is dependent upon age of the patient, site and size of lesion, and type
of aphasia.

Contents
[hide]

 1 Classification
o 1.1 Localizationist model
o 1.2 Other ways to Classify Aphasia
o 1.3 Fluent, non-fluent and "pure" aphasias
o 1.4 Primary and secondary aphasia
o 1.5 Cognitive neuropsychological model
o 1.6 Acquired childhood aphasia
 2 Signs and symptoms
 3 Causes
 4 Treatment
 5 History
 6 Notable cases
 7 See also
 8 Notes
 9 References
o 9.1 Handbooks
o 9.2 Bibliographic Databases
o 9.3 Specialized Bibliographies
o 9.4 Academic references
o 9.5 Personal experiences of aphasia

[edit] Classification
Classifying the different subtypes of aphasia is difficult and has led to disagreements among experts. The
localizationist model is the original model, but modern anatomical techniques and analyses have shown that
precise connections between brain regions and symptom classification don't exist. The neural organization of
language is complicated; language is a comprehensive and complex behavior and it makes sense that it isn't
the product of some small, circumscribed region of the brain.
No classification of patients in subtypes and groups of subtypes is adequate. Only about 60% of patients will
fit in a classification scheme such as fluent/nonfluent/pure aphasias. There is a huge variation among
patients with the same diagnosis, and aphasias can be highly selective. For instance, patients with naming
deficits (anomic aphasia) might show an inability only for naming buildings, or people, or colors.[1]

[edit] Localizationist model

Cortex

The localizationist model attempts to classify the aphasia by major characteristics and then link these to
areas of the brain in which the damage has been caused. The initial two categories here were devised by
early neurologists working in the field, namely Paul Broca and Carl Wernicke. Other researchers have added
to the model, resulting in it often being referred to as the "Boston-Neoclassical Model". The most prominent
writers on this topic have been Harold Goodglass and Edith Kaplan.

 Individuals with Broca's aphasia (also termed expressive aphasia) were once thought to have ventral
temporal damage, though more recent work by Dr. Nina Dronkers using imaging and 'lesion analysis'
has revealed that patients with Broca's aphasia have lesions to the medial insular cortex. Broca
missed these lesions because his studies did not dissect the brains of diseased patients, so only the
more temporal damage was visible. Dronkers and Dr. Odile Plaisant scanned Broca's original
patients' brains using a non-invasive MRI scanner to take a closer look. Individuals with Broca's
aphasia often have right-sided weakness or paralysis of the arm and leg, because the frontal lobe is
also important for body movement.

 In contrast to Broca's aphasia, damage to the temporal lobe may result in a fluent aphasia that is
called Wernicke's aphasia (also termed sensory aphasia). These individuals usually have no body
weakness, because their brain injury is not near the parts of the brain that control movement.

 Working from Wernicke's model of aphasia, Ludwig Lichtheim proposed five other types of aphasia,
but these were not tested against real patients until modern imaging made more indepth studies
available. The other five types of aphasia in the localizationist model are:

1. Pure word deafness

2. Conduction aphasia
3. Apraxia of speech, which is now considered a separate disorder in itself.
4. Transcortical motor aphasia
5. Transcortical sensory aphasia

 Anomia is another type of aphasia proposed under what is commonly known as the Boston-
Neoclassical model, which is essentially a difficulty with naming. A final type of aphasia, global
aphasia, results from damage to extensive portions of the perisylvian region of the brain.
[edit] Other ways to Classify Aphasia

[edit] Fluent, non-fluent and "pure" aphasias

The different types of aphasia can be divided into three categories: fluent, non-fluent and "pure" aphasias.[2]

 Fluent aphasias, also called receptive aphasias, are impairments related mostly to the input or
reception of language, with difficulties either in auditory verbal comprehension or in the repetition of
words, phrases, or sentences spoken by others. Speech is easy and fluent, but there are difficulties
related to the output of language as well, such as paraphasia. Examples of fluent aphasias are:
Wernicke's aphasia, Transcortical sensory aphasia, Conduction aphasia, Anomic aphasia[2]

 Nonfluent aphasias, also called expressive aphasias are difficulties in articulating, but in most
cases there is relatively good auditory verbal comprehension. Examples of nonfluent aphasias are:
Broca's aphasia, Transcortical motor aphasia, Global aphasia[2]

 "Pure" aphasias are selective impairments in reading, writing, or the recognition of words. These
disorders may be quite selective. For example, a person is able to read but not write, or is able to
write but not read. Examples of pure aphasias are: Pure alexia, Agraphia, Pure word deafness[2]

[edit] Primary and secondary aphasia

Aphasia can be divided into primary and secondary aphasia.

 Primary aphasia is due to problems with language-processing mechanisms.

 Secondary aphasia is the result of other problems, like memory impairments, attention disorders, or
perceptual problems.

[edit] Cognitive neuropsychological model

The cognitive neuropsychological model builds on cognitive neuropsychology. It assumes that language
processing can be broken down into a number of modules, each of which has a specific function. Hence
there is a module which recognises phonemes as they are spoken and a module which stores formulated
phonemes before they are spoken. Use of this model clinically involves conducting a battery of assessments
(usually from the PALPA), each of which tests one or a number of these modules. Once a diagnosis is
reached as to where the impairment lies, therapy can proceed to treat the individual module.

[edit] Acquired childhood aphasia

Acquired childhood aphasia (ACA) is a language impairment resulting from some kind of brain damage.
This brain damage can have different causes, such as head trauma, tumors, cerebrovascular accidents, or
seizure disorders. Most, but not all authors state that ACA is preceded by a period of normal language
development.[3] Age of onset is usually defined as from infancy until but not including adolescence.

ACA should be distinguished from developmental aphasia or developmental dysphasia, which is a primary
delay or failure in language acquisition.[4] An important difference between ACA and developmental
childhood aphasia is that in the latter there is no apparent neurological basis for the language deficit.[5]

ACA is one of the more rare language problems in children and is notable because of its contribution to
theories on language and the brain.[4] Because there are so few children with ACA, not much is known about
what types of linguistic problems these children have. However, many authors report a marked decrease in
the use of all expressive language. Children can just stop talking for a period of weeks or even years, and
when they start to talk again, they need a lot of encouragement. Problems with language comprehension are
less common in ACA, and don't last as long.[6]
[edit] Signs and symptoms
People with aphasia may experience any of the following behaviors due to an acquired brain injury,
although some of these symptoms may be due to related or concomitant problems such as dysarthria or
apraxia and not primarily due to aphasia.

 inability to comprehend language

 inability to pronounce, not due to muscle paralysis or weakness
 inability to speak spontaneously
 inability to form words
 inability to name objects
 poor enunciation
 excessive creation and use of personal neologisms
 inability to repeat a phrase
 persistent repetition of phrases
 paraphasia (substituting letters, syllables or words)
 agrammatism (inability to speak in a grammatically correct fashion)
 dysprosody (alterations in inflexion, stress, and rhythm)
 incompleted sentences
 inability to read
 inability to write
 limited verbal output
 difficulty in naming

The following table summarizes some major characteristics of different types of aphasia:

Auditory
Type of Repetition Naming
comprehension Fluency Presentation
aphasia
Individuals with Wernicke's aphasia may
speak in long sentences that have no
meaning, add unnecessary words, and
even create new "words" (neologisms).
For example, someone with Wernicke's
aphasia may say, "You know that
smoodle pinkered and that I want to get
him round and take care of him like you
Wernicke's mild– fluent want before", meaning "The dog needs to
mild–mod defective
aphasia severe paraphasic go out so I will take him for a walk".
They have poor auditory and reading
comprehension, and fluent, but
nonsensical, oral and written expression.
Individuals with Wernicke's aphasia
usually have great difficulty
understanding the speech of both
themselves and others and are therefore
often unaware of their mistakes.
Transcortical
mod– Similar deficits as in Wernicke's aphasia,
sensory good poor fluent
severe but repetition ability remains intact.
aphasia
Conduction poor poor relatively good fluent Conduction aphasia is caused by deficits
aphasia in the connections between the speech-
comprehension and speech-production
areas. This might be caused by damage to
 the arcuate fasciculus, the structure that
transmits information between
Wernicke's area and Broca's area. Similar
symptoms, however, can be present after
damage to the insula or to the auditory
cortex. Auditory comprehension is near
normal, and oral expression is fluent with
occasional paraphasic errors. Repetition
ability is poor.
Anomic aphasia is essentially a difficulty
with naming. The patient may have
difficulties naming certain words, linked
by their grammatical type (e.g. difficulty
Nominal or naming verbs and not nouns) or by their
mod–
Anomic mild mild fluent semantic category (e.g. difficulty naming
severe
aphasia words relating to photography but
nothing else) or a more general naming
difficulty. Patients tend to produce
grammatic, yet empty, speech. Auditory
comprehension tends to be preserved.
Individuals with Broca's aphasia
frequently speak short, meaningful
phrases that are produced with great
effort. Broca's aphasia is thus
characterized as a nonfluent aphasia.
Affected people often omit small words
such as "is", "and", and "the". For
example, a person with Broca's aphasia
may say, "Walk dog" which could mean
non-fluent, "I will take the dog for a walk", "You
Broca's mod– mod–
mild difficulty effortful, take the dog for a walk" or even "The
aphasia severe severe
slow dog walked out of the yard". Individuals
with Broca's aphasia are able to
understand the speech of others to
varying degrees. Because of this, they are
often aware of their difficulties and can
become easily frustrated by their
speaking problems. It is associated with
right hemiparesis, meaning that there can
be paralysis of the patient's right face and
arm.
Similar deficits as Broca's aphasia,
except repetition ability remains intact.
Auditory comprehension is generally fine
Transcortical mild– for simple conversations, but declines
good mild non-fluent
motor aphasia severe rapidly for more complex conversations.
It is associated with right hemiparesis,
meaning that there can be paralysis of the
patient's right face and arm.
Global aphasia poor poor poor non-fluent Individuals with global aphasia have
severe communication difficulties and
will be extremely limited in their ability
to speak or comprehend language. They
may be totally nonverbal, and/or only use
facial expressions and gestures to
 communicate. It is associated with right
hemiparesis, meaning that there can be
paralysis of the patient's right face and
arm.
Mixed
Similar deficits as in global aphasia, but
transcortical moderate poor poor non-fluent
repetition ability remains intact.
aphasia
Characteristics and symptoms depend
upon the site and size of subcortical
Subcortical
lesion. Possible sites of lesions include
aphasias
the thalamus, internal capsule, and basal
ganglia.

Jargon aphasia is a fluent or receptive aphasia in which the patient's speech is incomprehensible, but appears
to make sense to them. Speech is fluent and effortless with intact syntax and grammar, but the patient has
problems with the selection of nouns. They will either replace the desired word with another that sounds or
looks like the original one, or has some other connection, or they will replace it with sounds. Accordingly,
patients with jargon aphasia often use neologisms, and may perseverate if they try to replace the words they
can't find with sounds.

Commonly, substitutions involve picking another (actual) word starting with the same sound (e.g.
clocktower - colander), picking another semantically related to the first (e.g. letter - scroll), or picking one
phonetically similar to the intended one (e.g. lane - late).

 Physical findings of aphasias

 Broca aphasia

 This aphasia syndrome contains a number of distinct components that occur in various combinations.
In the complete syndrome, patients present with a nonfluent aphasia. They speak haltingly, without
intonation, and have difficulty producing spontaneous speech, naming, and repeating. They may
initially be mute, and their articulation may be impaired. Patients are often hypophonic.
Comprehension is relatively spared, though it is not normal. Phrases are short and may be telegraphic
or agrammatic, including major nouns and verbs but no functor words (articles, adjectives, adverbs,
or conjunctions). Patients have telegraphic speech, also called agrammatism. Naming of actions is
typically worse than naming of objects.
 A writing deficit usually parallels the phonologic deficit.
 Repetition is abnormal and often consists of omission of functor words. Patients almost always have
syntactic and comprehension deficits. Comprehension of passive constructions and of complex
syntactic constructions, such as dependent clauses, may be abnormal. Neighborhood signs include
buccofacial or limb apraxia and right hemiparesis, often involving the face and arm more than the
leg.
 Buccofacial apraxia can be tested by asking the patient to pantomime blowing a kiss or blowing out a
match. Speech therapists may observe oral apraxia and difficulty swallowing. Limb apraxia may also
accompany Broca aphasia, but it is most commonly caused by a large lesion including additional
areas in the parietal or frontal lobes. Depression is extremely frequent because patients are typically
aware of their deficits; in extreme form, this is associated with a complete withdrawal, termed by
Kurt Goldstein the catastrophic reaction.
 Reading is often more affected than auditory comprehension. Patients may make semantic errors (eg,
reading "symphony" when the word is "concert"), one of the components of deep dyslexia. Patients
may lose the ability to sound out words (they can no longer map graphemes to phonemes) but may
be able to read frequent, previously learned, highly imageable words by recognition (they could read
"tree" but not "proscription").
 Typically, the lesions in Broca aphasia are localized to the dorsolateral frontal cortex (the posterior
two thirds of the inferior frontal gyrus operculum), though some cases have associated lesions in the
anterior parietal cortex and lateral striate and periventricular white matter. Frontal subcortical
 connections, such as the subcallosal fasciculus, are important for speech initiation and may disrupt
thalamofrontocortical connections. Alexander et al argued that the full syndrome would not occur
without involvement of the underlying white matter tracts.1
 Kreisler et al have attempted to relate specific components of the common aphasia syndromes with
neurologic localization. The authors investigated 107 patients with a standard aphasia battery and
looked at 69 predetermined areas of interest. They found an analysis to identify 67-94% of patients.
They found the following:2
o Nonfluent aphasias depended on frontal or putamenal lesions (mutism, low fluency).
o Repetition depended on insula-external capsule lesions and posterior internal capsule rather
than the classically described arcuate fasciculus.
o Comprehension depended on posterior lesions of the temporal gyri or inferior frontal gyrus.
o Phonemic paraphasias depend on external capsule lesions extending to the posterior temporal
lobe or internal capsule.
o Verbal paraphasias depended upon temporal or caudate lesions.
o Perseveration depended upon caudate lesions.
 Note that these localizations largely confirm teachings about aphasia that have been in circulation
since the writings of Broca and Wernicke, and others, in the 19th century.
 Studies involving functional imaging, including positron emission tomography (PET) and functional
magnetic resonance imaging (fMRI) suggest that separate modules within the left inferior frontal
gyrus subserve different aspects of speech, including semantic, syntactic, and phonologic functions.
Complete Broca aphasia syndrome occurs with a large lesion destructive of the whole area, whereas
partial syndromes occur with smaller lesions. In fact, patients with large left perisylvian lesions often
have global aphasia in the early days and weeks after their strokes, and they slowly evolve into Broca
aphasia. Patients with isolated Broca area lesions may have Broca aphasia on the first day of their
stroke. On the receptive side, comprehension of complex sentences with embedded clauses requires
activation of the left frontal cortex of the Broca area, and this task is usually deficient in patients with
Broca aphasia.
 Recovery from Broca aphasia may occur over months and sometimes years. Patients may progress in
the nosology of Broca aphasia and may develop anomic aphasia or become normal over time.
 Broca area aphasia, also called a baby-Broca lesion, occurs with a lesion limited to area 44 (the
frontal operculum). This aphasia includes what has been called aphemia, cortical dumbness,
anarthria, and subcortical motor aphasia. The condition is also closely tied to what speech/language
pathologists call apraxia of speech. This condition affects production of phonemes, especially
multiconsonant words, and may not represent a true language disorder or aphasia. Aphemia often
improves rapidly. A similar syndrome can occur with a lesion limited to the lower prerolandic
fissure. Patients may be mute, or they may express themselves in slow, effortful productions, with
normal or nearly normal language and syntax. Foreign-accent syndrome is a variant of aphemia,
involving damage to the motor speech outflow mechanism. Foreign accent syndrome is more of a
cortical dysarthria, akin to acquired stuttering, than a true aphasia.

 Wernicke aphasia

 Patients with Wernicke aphasia have fluent language expression, but their speech sounds empty and
does not convey meaning. There may be fluent phrases without nouns and verbs, containing
nonexistent word forms (neologisms). The patient's speech and writing may include paraphasic
errors with sound substitutions (phonemic paraphasias), word substitutions (semantic paraphasias),
hesitations, pauses, and circumlocutions. Grammar is better preserved than it is in Broca aphasia.
This abnormal speech output is called paragrammatic, as compared to the agrammatic output of
Broca aphasia.
 Naming and repetition are typically impaired, but the most significant problem is the abnormal
language comprehension. Although reading impairment often parallels the auditory comprehension
deficit, patients occasionally have preserved oral reading or even reading comprehension. This is
important in establishing communication with the patient. Written expression is abnormal; unlike
patients with Broca aphasia, these patients can write fluently, but their word choice and spelling are
usually very abnormal. In mild Wernicke aphasia, abnormal spelling in written productions may be a
 clue to the deficit. In acute stroke with Wernicke aphasia, patients may seem confused in addition to
their language deficits, and they may even appear psychotic.
 Patients with Wernicke aphasia are not always aware of their deficits, and over time they may
become frustrated that others do not understand them. Some patients become overtly paranoid about
their failure to communicate. Patients with Wernicke aphasia may recognize their errors if the
mistakes are presented to them offline (eg, on an audio tape).
 The lesion is variable but usually involves the posterior one-third of the superior temporal gyrus.
Involvement of deep temporal white matter, the middle or inferior temporal gyri, or the inferior
parietal lobule may predict a lesser degree of recovery. Wernicke aphasia is most typically associated
with embolic strokes affecting the inferior division of the middle cerebral artery, supplying the
temporal cortex and sparing the frontal, motor cortex.

In studies by Naeser and colleagues3 , destruction of Wernicke area predicted lasting loss of
comprehension, and recent, acute studies by Hillis and colleagues4 have found single word
comprehension deficits to correlate with hypoperfusion of Wernicke area. Recovery also depends on
the size of the lesion, the amount of the traditional Wernicke area that is destroyed, the age of the
patient, and the status of the contralateral hemisphere. Recovery can be complete or the aphasia can
progress to conduction or anomic aphasia.
 Similar or identical lesions can produce different syndromes of aphasia at different points in the
disease process. Neighborhood signs should be sought to help in localization. In Wernicke aphasia,
neighborhood signs include a superior quadrantanopsia due to involvement of optic radiations, limb
apraxia due to involvement of the inferior parietal lobule or its connections to the premotor cortices,
finger agnosia, acalculia, or agraphia (components of the Gerstmann syndrome) due to involvement
of the angular gyrus. The key neighborhood sign is a negative one; patients with Wernicke aphasia
usually have no hemiparesis.
 Research has debated the category specificity of semantic, naming, and language deficits.5 For
example, lesions of the fusiform or occipital gyrus may be more likely to cause an inability to name
living things or highly imageable words, perhaps due to the proximity to the visual areas. Lesions of
the temporal lobes are more likely to affect the naming of tools or inanimate objects, whereas frontal
lesions may specifically impair verb naming.

 Conduction aphasia

 This classical aphasia type is less common than Broca, Wernicke, or global aphasia. Language
output is fluent, though some patients make phonemic errors in speech and pause to correct them,
giving the speech a somewhat halting quality. This attempt to correct errors is called conduit
d'approche. Naming may or may not be impaired. Repetition impairment is the hallmark of
conduction aphasia. Careful studies have shown the ability of patients with aphasia to correct their
tape-recorded speech, suggesting an offline ability to monitor output in some cases. Auditory
comprehension is typically normal in conduction aphasia. Oral reading and writing abilities are
variable. Patients with conduction aphasia may have normal comprehension of written language;
cases of patients with conduction aphasia who are able to read novels have been reported.
 The classic disconnection hypothesis, originally formulated by Wernicke and more recently adopted
by Geschwind,6 highlights the importance of the arcuate fasciculus connecting the temporal and
frontal language cortices, thereby connecting comprehension with speech-output centers. By this
theory, a disconnection between these centers results in the inability to repeat, in the setting of intact
comprehension and verbal fluency. Other theories of conduction aphasia include a deficit of
auditory-verbal short-term (immediate) memory or “inner speech.”
 The supramarginal gyrus is often affected in conduction aphasia, though disruption of the subcortical
connections in the arcuate fasciculus may also be important. Research has implicated the
supramarginal gyrus in the decoding of phonemes in receptive language and presumably their
translation into oral expression. Recovery is usually good, but residual semantic and phonologic
difficulties may remain. Kreisler et al, in the work cited, could not confirm the roles of the arcuate
fasciculus or supramarginal gyrus as classically described.2
  Neighborhood signs in conduction aphasia include superior quadrantanopsia, if the lesion undercuts
the parietal lobe, and limb apraxia, which is typically more disabling and less often diagnosed than
the aphasia itself. Temporal lobe lesions that do not totally damage the Wernicke area may result in
conduction aphasia, and such cases do not have associated apraxia, whereas patients with left parietal
lesions often have associated limb apraxia.

 Global aphasia

 In this type of aphasia, the patient has deficits in all aspects of language: spontaneous speech,
naming, repetition, auditory comprehension, reading, and writing. The deficits need not be total.
Global aphasia may result from strokes, tumors, dementia, or other causes.
 Global aphasia is commonly seen in patients with large infarctions of the left cerebral hemisphere,
typically involving the occlusion of the internal carotid or middle cerebral artery and resulting in a
large, wedge-shaped infarction of the frontal, temporal, parietal, and deep portions of the middle
cerebral artery territory. Right hemiplegia (face and arm worse than the leg) is the rule, as is right
homonymous hemianopsia. Limb apraxia is common. Some patients have a catastrophic reaction,
described by Kurt Goldstein as an emotional meltdown when the patient is asked to perform
language tasks; this phenomenon is likely related to depression.
 Global aphasia rarely occurs with right hemispheric lesions (also called crossed aphasia). About one
fifth of left-handed people and 1% of right-handed people have global aphasia after mirror-image
lesions of the homologous cortex of the right hemisphere; in this case, left homonymous
hemianopsia and left hemiplegia are expected.
 Global aphasia rarely occurs without hemiparesis. In such cases, dual lesions in the left cerebral
hemisphere are expected; these spare the motor areas but affect both anterior and posterior
perisylvian language areas. Although multiple strokes could produce such a clinical picture, in
practice, the possibility of tumors should be considered with such multiple lesions. In cases of
aphasia without hemiparesis, a thalamic lesion should also be considered in the differential
diagnosis.
 Although global aphasia is often considered a devastating injury, gradations of global aphasia exist.
Many patients with poststroke global aphasia evolve toward Broca aphasia, or mixed nonfluent
aphasia, with improvement in language comprehension over time. Many patients with global aphasia
are proficient at making their needs understood without spoken or written speech. Prosody,
inflection, pointing, and expressions of approval or disapproval are some of the ways in which
patients with global aphasia may communicate successfully.
 Patients with large, left hemisphere lesions and global aphasia are vastly different from patients with
large, right hemispheric lesions whose language may appear normal but the nonlinguistic aspect of
language expression is lost, including the prosody or emotional aspect of language expression and
the ability to understand humor or sarcasm in the speech of others. Patients with such right-
hemisphere syndromes are less aware of their deficits than patients with aphasia and may be less
responsive to rehabilitation.
 Factors affecting the prognosis may include the nature of the underlying injury (eg, dementia, tumor,
stroke), the age of the patient, area of infarction (if present), the health of the remaining brain, and
the availability of rehabilitation services.
 Recovery in the first 6 months generally outpaces later recovery; however, some patients can recover
function years after the initial injury. In one study of patients with global aphasia, more improvement
occurred during the second 6 months after the injury than during the first 6 months.

 Pure word deafness

 Patients with pure word deafness cannot comprehend spoken language, but they are not deaf. Their
verbal output and reading comprehension are said to be intact, but most published cases have shown
some degree of fluent, paraphasic speech.
 The condition can occur because of damage to the superior temporal (Heschl) gyrus bilaterally, but
cases have been described with unilateral, left temporal lesions. Disconnection theory proposes that
 inputs from both Heschl gyri are cut off from input into the left hemisphere Wernicke area where
sounds are decoded into language.
 Pure word deafness should be differentiated from cortical deafness, in which both language and
nonlinguistic sounds are affected, and also from auditory nonverbal agnosia. Patients with cortical
deafness may appear deaf, but they often have some sparing of pure-tone hearing, especially as
recovery occurs. Auditory nonverbal agnosia involves failure of recognition of familiar sounds, such
as the moo of a cow or the ringing of a bell. A related disorder is phonagnosia, in which familiar
voices are not recognized. All of these cortical auditory deficits (pure word deafness, cortical
deafness, auditory nonverbal agnosia, and phonagnosia) usually reflect bilateral temporal lobe
lesions.

 Transcortical aphasias

 The term transcortical aphasia was originally chosen by Lichtheim to indicate aphasias related to
primary lesions not involving the language cortex but rather connected areas of the association
cortex, which he called the "area of concepts." By definition, patients with transcortical aphasia can
repeat, but they have difficulty naming or producing spontaneous speech or understanding spoken
speech. Patients with transcortical motor aphasia can comprehend speech but have diminished
speech output and an inability to name items. Sometimes they speak only in single words, after a
delay, or in a soft voice.

Transcortical motor aphasia involves a deficit in the initiation of speech, reduced phrase length, and
abnormal grammar. Mutism may be present initially. Repetition may be relatively unimpaired,
distinguishing these patients from those with Broca aphasia who cannot repeat fluently. In some
patients, a stroke in the anterior cerebral artery territory is the cause; leg greater than arm weakness,
shoulder greater than hand weakness, and often an involuntary grasp response are associated
findings.
 In transcortical sensory aphasia, patients can produce fluent speech, but it is often empty and
paraphasic. Patients also have a severe deficit in comprehension of speech. Their naming is often
abnormal, and they lose semantic associations of speech. In general, they act much like patients with
Wernicke aphasia, except they can repeat. This type of aphasia is typically seen in advancing
Alzheimer disease and other progressive dementias, but it is also seen occasionally in patients with
stroke, typically those with bilateral lesions in the parieto-occipital cortex or a lesion in the left
temporo-occipital cortex.
 Mixed transcortical aphasia, also called the syndrome of isolation of the speech area, involves ability
to repeat but not to produce spontaneous speech or comprehend language. Patients may repeat in an
echolalic fashion, and they may complete common phrases begun by the examiner. This syndrome
resembles global aphasia, except for the preserved repetition.

 Anomic aphasia

 Patients with anomic aphasia present with fluent speech, intact or mostly intact repetition, intact
auditory comprehension, reading, and writing, but an inability to name objects and body parts.
Anomic aphasia may follow recovery from another type of aphasia. Anomic aphasia can be an initial
presentation of an aphasia syndrome, and it warrants its own aphasia syndrome.
 Anomic aphasia is less specific in lesion localization than the other syndromes mentioned previously.
Anomia may occur with lesions in the dorsolateral frontal cortex, temporal or temporo-occipital
cortex, or thalamus. Tumors of the left temporal lobe may present with anomic aphasia. This aphasia
type is also the typical language deficit in patients with early Alzheimer disease.

 Subcortical aphasias

 Broca reported lesions of the deep basal ganglia with cortical lesions in his original autopsy report of
his famous patient, Tan-tan. More controversial than that association is whether a basal ganglia
lesion by itself can cause aphasia.
  A series of reports in the early 1980s, which used computed tomography (CT) as the primary
neuroimaging modality, associated lesions of the head of the caudate nucleus, anterior putamen, and
anterior limb of the internal capsule with a nonfluent aphasia syndrome, often with dysarthria and
with better repetition and comprehension than typically seen with Broca aphasia.7,8,9,10 This syndrome
has been called the anterior subcortical aphasia syndrome. When the lesion extends into the
temporal isthmus area, subcortical versions of Wernicke and even global aphasia can occur. The
diagnosis of subcortical aphasia is based more on the imaging of a subcortical lesion than on the
specific language characteristics of the aphasia syndrome.
 In some cases, MRIs have revealed cortical lesions in patients with aphasia whose CT scans
demonstrated only subcortical lesions. Blood-flow imaging has shown flow abnormalities in the
cortex of patients whose MRIs depicted only lesions in the basal ganglia. Such diminished flow may
partly reflect cortical ischemia and partly reflect a reduced perfusion of functionally connected areas
called diaschisis.
 Weiller et al examined patients with striatocapsular lesions, some with aphasia or neglect, and some
without. On MRI, lesions in both groups were similar. However, patients with aphasia and neglect
had low blood flow in the cortex, suggesting that cortical ischemia may also be important in some
subcortical aphasias. Weiller also found that among patients with identical vascular syndromes, those
who had strokes due to atrial fibrillation typically had aphasia and neglect, whereas those who had
strokes due to large vessel stenosis did not. They attributed the finding to the ability of patients with
chronic low flow due to large vessel stenosis to develop collaterals, whereas those with a sudden
occlusion due to an embolus could not do so.11

 Thalamic aphasias

 Thalamic aphasia, like the subcortical aphasia syndromes, is defined by the anatomic documentation
of a lesion in the thalamus rather than by the specific language characteristics of the aphasia
syndrome. Patients with thalamic aphasia usually present with fluent language disorders, often
without hemiparesis. Associated findings include anomia, jargon speech, semantic paraphasic errors,
intact repetition, and relatively preserved comprehension. Such patients may also manifest an acute
affective syndrome with abulia or severe depression.
 Thalamic aphasia was initially described in patients with left thalamic hemorrhage. The first author
reported a left-handed patient with a right thalamic hemorrhage, indicating that language dominance
extends down to thalamic level. In hemorrhage, of course, the language disorder possibly results
from mass effect or pressure on adjacent structures rather than on the specific focus of the
hemorrhage. More recent cases of thalamic aphasia secondary to ischemic stroke have solidified the
evidence that the thalamus is important to language function.

The vascular lesion that affects the anterior thalamus is a small-vessel disease affecting the polar or
tuberothalamic artery of the thalamus. The lesion is easily seen on CT scans or MRIs. Lesions in the
anterior thalamus also affect memory. Crosson et al argued persuasively for the importance of
pulvinar and other posterior structures in language, but their data were based on stimulation rather
than lesion ablation.12

Pulvinar strokes causing aphasia are exceedingly rare because of the vascular anatomy of the
thalamus. Lesions of the paramedian thalamus (thalamoperforating artery), especially if bilateral
(some patients have a single artery, sometimes called the artery of Percheron, supplying both sides),
cause deficits in memory and language. In his book , Crosson discusses the possible role of the
ventral lateral nucleus in atypical aphasias, but usually this vascular territory (inferolateral arteries)
involves a pure sensory stroke, while the posterior choroidal artery territory mainly involves the
lateral geniculate body, causing an isolated hemianopia.
 Lesions of the white matter between the thalamus and the temporal lobe, the temporal isthmus, or
temporal stalk may produce aphasia due to deafferentation of the overlying temporal lobe. These
aphasias closely resemble Wernicke aphasia. As mentioned above, however, the cases reported have
not entirely excluded cortical involvement or hypometabolism as a cause of the syndrome.
 Pure alexia without agraphia

 Pure alexia is known by a variety of names, including alexia without agraphia, posterior alexia, and
literal or letter-by-letter alexia. Patients with pure alexia have normal expressive speech, normal
naming (except in some cases for color anomia or inability to name colors), normal repetition,
normal auditory comprehension, and even normal ability to write. Their alexia is a relatively pure
deficit. Patients may be able to write a sentence, then be unable to read it. They have no difficulty
spelling aloud and no difficulty in recognizing words spelled to them aloud or spelled in tactile
fashion on the palm of the hand. Patients may be able to read individual letters, then laboriously
piece them together and say the words (letter-by-letter alexia).
 Neighborhood signs useful in the diagnosis of pure alexia include a contralateral (right) superior
quadrantanopsia or hemianopia and color anomia. The syndrome is almost always associated with a
stroke in the territory of the left posterior cerebral artery. The lesion may also involve the splenium
of the corpus callosum and the medial temporal lobe.
 Dejerine first described this syndrome in 1892, postulating a disconnection between the right
occipital cortex (and intact left visual field) and the left hemisphere language area, such that visual
information cannot be decoded into language in the left hemisphere.13 Later contributors recognized
that the posterior left hemisphere has a word-form recognition area that, if damaged, prevents
patients from reading words at a glance, as normal readers do. Nearly a century later, Geschwind6
and then Damasio7 refined the disconnection hypothesis of pure alexia. Cognitive neuropsychologists
and behavioral neurologists have recognized the concept of damage to the orthographic recognition
areas in the left occipital lobe.

 Alexia with agraphia

 Alexia with agraphia is also known as the angular gyrus syndrome and central alexia. It is, in effect,
an acquired illiteracy; patients lose their previously acquired reading and writing skills. Most lose
spelling and the ability to understand words spelled to them. Many patients have fluent, paraphasic
speech, unlike the preserved speech of pure alexia without agraphia, but auditory comprehension is
much superior to reading comprehension. The lesion usually involves the angular gyrus area in the
left inferior parietal lobule. This syndrome was also described by Dejerine.
 Closely related to the pure alexia with agraphia syndrome is the Gerstmann syndrome. Gerstmann
brought together the 4 deficits of agraphia, acalculia, right-left confusion, and finger agnosia and
associated them with lesions of the dominant parietal lobe. Alexia, though not originally a cardinal
feature of the Gerstmann syndrome, is often associated.
 Modern authors such as Benton have questioned the validity of the Gerstmann syndrome.14,15 Some
patients may have one or more of the deficits without the others. Stimulation studies in epileptic
patients, however, have reproduced combinations of these deficits with stimulation in the angular
gyrus area, confirming the association of the key elements of the Gerstmann syndrome.

 Right hemisphere language disorders

 Right hemisphere contributions to language are numerous, and recent research has led to a better
understanding of right hemisphere functions related to communication. The right hemisphere can
maintain an extensive vocabulary and read at the word and phrase level. Higher functions of right
hemisphere speech, subserved in part by right frontal and temporal lobes, include the comprehension
of metaphor, sarcasm, and humor, as well as the emotional prosody of speech, ie, the extralinguistic
aspects of human communication.
 Patients with right hemisphere lesions may understand words but fail to understand the emotional
context of a conversation or the facial expressions and tones of voice that convey meaning in normal
communication. In addition, they may fail to observe normal turn-taking and other pragmatic aspects
of a conversation. In patients with normal speech and language comprehension, these deficits can be
disabling in a social context. Patients with right hemisphere lesions may have a problem with
discourse and have difficulty stringing together several sentences into a spoken paragraph with a
beginning, middle, and end, as a storyteller or lecturer would do.
[edit] Causes
Aphasia usually results from lesions to the language-relevant areas of the frontal, temporal and parietal lobes
of the brain, such as Broca's area, Wernicke's area, and the neural pathways between them. These areas are
almost always located in the left hemisphere, and in most people this is where the ability to produce and
comprehend language is found. However, in a very small number of people, language ability is found in the
right hemisphere. In either case, damage to these language areas can be caused by a stroke, traumatic brain
injury, or other brain injury. Aphasia may also develop slowly, as in the case of a brain tumor or progressive
neurological disease, e.g., Alzheimer's or Parkinson's disease. It may also be caused by a sudden
hemorrhagic event within the brain. Certain chronic neurological disorders, such as epilepsy or migraine,
can also include transient aphasia as a prodromal or episodic symptom.[citation needed] Aphasia is also listed as a
rare side effect of the fentanyl patch, an opioid used to control chronic pain.[7]

[edit] Treatment
There is no one treatment proven to be effective for all types of aphasias. Melodic intonation therapy is often
used to treat non-fluent aphasia and has proved to be very effective in some cases.

[edit] History
The first recorded case of aphasia is from an Egyptian papyrus, the Edwin Smith Papyrus, which details
speech problems in a person with a traumatic brain injury to the temporal lobe.[8]

[edit] Notable cases

 Ralph Waldo Emerson[9]

[edit] See also

 Aphasiology
 Speech disorder
 Dysnomia disorder
 Aprosodia
 Dysprosody
 Glossolalia
 Paragrammatism

[edit] Notes
1. ^ Kolb, Bryan; Whishaw, Ian Q. (2003). Fundamentals of human neuropsychology. [New York]:
Worth. pp. 502, 505, 511. ISBN 0-7167-5300-6. OCLC 464808209.
2. ^ a b c d Kolb, Bryan; Whishaw, Ian Q. (2003). Fundamentals of human neuropsychology. [New
York]: Worth. pp. 502–504. ISBN 0-7167-5300-6. OCLC 464808209.
3. ^ Murdoch, B. E. (1990). Acquired neurological speech/language disorders in childhood.
Washington, DC: Taylor & Francis. ISBN 0-85066-490-X. OCLC 21976166.
4. ^ a b Woods, Bryan T. (1995). "Acquired childhood aphasia". In Kirshner, Howard S.. Handbook of
neurological speech and language disorders. New York: M. Dekker. ISBN 0-8247-9282-3.
OCLC 31075598.
5. ^ Paquier, P.F.; van Dongen (1998). "Is Acquired Childhood Aphasia Atypical?". In Basso, Anna;
Coppens, Patrick; Lebrun, Yvan. Aphasia in atypical populations. Hillsdale, N.J: Lawrence Erlbaum
Associates. ISBN 0-8058-1738-7. OCLC 37712996.
6. ^ Baker, Lorian; Cantwell, Dennis P. (1987). Developmental speech and language disorders. New
York: Guilford Press. ISBN 0-89862-400-2. OCLC 14520470.
7. ^ "Fentanyl Transdermal Official FDA information, side effects and uses.". Drug Information
Online. http://www.drugs.com/pro/fentanyl-transdermal.html#A02A9CB6-35CF-4F01-A980-
C3733E0F861A.
8. ^ McCrory PR, Berkovic SF (December 2001). "Concussion: the history of clinical and
pathophysiological concepts and misconceptions". Neurology 57 (12): 2283–9. PMID 11756611.
http://www.neurology.org/cgi/content/abstract/57/12/2283.
9. ^ Richardson, Robert G. (1995). Emerson: the mind on fire: a biography. Berkeley: University of
California Press. ISBN 0-520-08808-5. OCLC 31206668.