Review Article

Language Dysfunction
Address correspondence to
Dr David J. Gill, Unity
Rehabilitation and Neurology,
2655 Ridgeway Avenue, Suite
David J. Gill, MD; Krista M. Damann, PhD, ABPP-CN 420, Rochester, NY 14626,
[email protected].
Relationship Disclosure:
Drs Gill and Damann report
ABSTRACT no disclosures.
Purpose of Review: Language is a complex brain function requiring a number of Unlabeled Use of
Products/Investigational
cognitive processes and is commonly affected by both focal brain lesions and neuro- Use Disclosure:
degenerative disorders. This article reviews the neuroanatomic basis of language, as- Drs Gill and Damann discuss
sessment techniques of language function, and disorders affecting language. the unlabeled/investigational
use of donepezil for the
Recent Findings: Recent functional imaging studies of language suggest that the classic treatment of poststroke aphasia.
connectionist models of language function may be incomplete. These studies and those * 2015, American Academy
analyzing how the primary progressive aphasias (PPAs) affect language function suggest of Neurology.
that language processing is completed through large-scale distributed networks. The use
of structured, standardized techniques allows for the diagnosis of focal brain lesions
affecting language function as well as neurodegenerative and psychogenic causes of
language dysfunction.
Summary: By employing an accurate, neuroanatomically grounded language assess-
ment technique, the neurologist can reach the correct diagnosis and implement the
optimal management plan for patients with language disorders. Neurologists should
also be aware of new information regarding the neural basis of language function as our
understanding of the complex cognitive process of language continues to evolve.

Continuum (Minneap Minn) 2015;21(3):627–645.

INTRODUCTION cepts used in linguistics, which is the

Language is communication through scientific study of language.
use of symbols and is unique to humans. The smallest unit of language that con-
Language can be aural or visual (eg, writ- veys unique information is the phoneme
ing or sign language). The development (termed grapheme when referring to
of language is universal to all healthy writing). Phonemes are units of sound
humans who are exposed to language that, when put together, form a mor-
during childhood and is integral to all pheme. A phoneme roughly corresponds
human interaction. This article focuses to a letter of the alphabet, and differ-
on language and not on speech, the neu- ent languages have different numbers
romuscular system required for verbal of phonemes. (English has approximately
output, and covers the neuroanatomical 30 phonemes, whereas some languages
basis of language, assessment techniques, such as Mandarin have more than 50).
and common disorders of language. A morpheme is the smallest piece into
Similar to the experience of memory, which words can be separated. Some
healthy individuals perceive communica- morphemes are words (eg, town), whereas
tion by language as seamless despite the other morphemes require a root to which
fact that the complex process of linguis- they are attached (eg, ing) and are re-
tic communication involves a number of ferred to as bound morphemes. The
interconnected, yet functionally and an- purpose of language is to convey mean-
atomically separable cognitive processes. ing, and semantics refers to the mean-
To understand the complex neuroana- ing of language. This term overlaps with
tomic basis of language, neurologists semantic memory. For more information
must have an understanding of the con- on semantic memory, refer to the article

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 Language Dysfunction

KEY POINTS
h Language is unique and ‘‘Memory Dysfunction’’ by Brandy R. the rhythm and intonation of speech and
universal to humans. Matthews, MD, FAAN, in this issue is necessary for emotional communica-
of . tion such as sarcasm and humor. It also
h Linguistics has shown
The meaning of language can be con- allows for clarification of whether a sen-
that language contains
separable units at the
veyed at both the word level and the tence is a question or a statement. Prag-
sound, word, sentence, sentence level. At the word level, the matics (or discourse) is how language
and discussion levels meaning of a word includes two parts: is utilized and includes how sentences
of language. (1) knowledge shared by a community are made to fit into a conversation.
about the word (eg, cows live on a farm;
cows are used for meat) and (2) the fea- NEUROANATOMY OF LANGUAGE
tures that all things called by that word Normal language function requires proper
share (eg, cows produce milk; cows eat neural function over a wide geography of
grass). Syntax (or grammar) is the set of brain regions. A person with dysfunction
rules of a particular language that deter- in this neural network has an aphasia.
mine the ways words are combined to (This article will use the more com-
make sentences and what they mean monly used term aphasia despite that,
when they are combined. Prosody is in most individuals, the term dysphasia,

TABLE 3-1 Classic Aphasia Syndromes

Aphasia Disorder of Language Classical Localizationa Spoken Fluency

Broca Disruption of speech Left posterior inferior Impaired: Speech is sparse and
planning and production frontal lobe involving effortful; function words and bound
Broca area morphemes are often missing

Transcortical Disruption of speech Left frontal cortex and white Impaired: Speech is sparse and
motor planning and production matter sparing Broca area effortful; function words and bound
morphemes are often missing

Wernicke Disruption of representations Posterior half of left Normal, but speech has abnormal
of word sounds superior temporal gyrus word sound and structure
involving Wernicke area (paraphasic errors)
Transcortical Disruption of representations Left posterior temporal/ Normal, but speech has abnormal
sensory of word sounds parietal cortex and word sound and structure
white matter sparing (paraphasic errors)
Wernicke area
Global Disruption of all Left hemisphere involving Impaired
language processing the majority of the
perisylvian area
Conduction Disconnection of Lesion of arcuate fasciculus Mildly impaired with
representation of words and frequent paraphasic errors
the motoric process of speech
Anomic Disruption of the network Does not localize well; Intact with word finding pauses
allowing proper sound can involve the inferior
structure of words parietal lobe
a
Presumes left hemispheric dominance.

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implying dysfunction rather than absence (Brodmann areas 41 and 42), whereas
of function, is probably more accurate.) visual forms of communication (eg, read-
The classic aphasias are summarized in ing and sign language) are processed by
Table 3-1. the primary and secondary visual cortices
The presumed deficit and common that then project to Wernicke area through
lesion locations of the aphasias has led the ventral visual stream. The arcuate
to the connectionist models developed fasciculus then projects from Wernicke
by Broca, Wernicke, Lichtheim, and area to Broca area (Brodmann areas 44
Heilman1 shown in Figure 3-1.2 and 45) and the surrounding area to per-
Figure 3-23 shows the areas involved mit repetition. Broca area is the center
in language function in the classic con- for expressive language planning.
nectionist model. The conceptual frame- The majority of individuals are left
work behind this model is that Wernicke dominant for language, meaning that lan-
area (Brodmann area 22) and the sur- guage function is localized to the left
rounding area mediate comprehension. hemisphere. Lateralization of language is
Auditory stimuli are projected to Wernicke associated with handedness, with approx-
area from the nearby Heschl gyrus imately 90% of right-handed individuals

Auditory Comprehension Writing Reading Repetition Naming

Mostly normal Impaired: Writing is Mostly normal Impaired Expressive
effortful; function naming affected
words and bound
morphemes are
often missing
Mostly normal Impaired: Writing is Mostly normal Normal Expressive
effortful; function naming affected
words and bound
morphemes are
often missing
Impaired Mostly normal, but Impaired Impaired Both expressive and
contains paraphasic receptive naming
errors affected
Impaired Mostly normal, but Impaired Normal Both expressive
contains paraphasic and receptive
errors naming affected

Impaired Impaired Impaired Impaired Impaired

Intact Intact Mildly impaired Impaired Mostly normal

with paraphasic
errors
Intact Intact Intact Intact Impaired

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 Language Dysfunction

FIGURE 3-1 Classic connectionist model of language function. In this model, the frontal lobe
may play a role in activating the semantic-conceptual areas, which then activate the
phonological lexicon, allowing the person to produce spontaneous speech.
When the phonological lexicon activates the semantic-conceptual field, the person is able to
comprehend speech. The phonological lexicon is thought to contain memories of word sounds.
Therefore, to understand speech, speech information enters the system through the auditory
cortex, which is then sent to Wernicke area and then to semantic-conceptual areas (more widely
distributed) to allow comprehension of the speech in the semantic-conceptual areas. To produce
spontaneous speech, the frontal lobe (intentional/motivational systems) would activate the
semantic-conceptual areas in order to activate the corresponding areas in Wernicke area, which
would then project to Broca area and then to the motor cortex to activate the appropriate motor
programs to produce the desired speech.
2
Data from Heilman KM, Oxford University Press.

and 70% of left-handed individuals being population is right handed, this article
left hemisphere dominant for language, will use the left hemisphere dominance
although some debate exists about exact for lateralization.
percentages. In left-handed individuals, The right hemisphere is thought to play
about a third are either right hemisphere a role in the prosody of language. Prosody
dominant or have language represented is often described as the lyrical aspect
bilaterally. As over 90% of the world’s of language that conveys information

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 KEY POINTS
h The existing classic
models of language
function are being
critically examined and
updated based on
use of functional
imaging studies.
h Many patients with
aphasia do not fit well
into one of the classic
aphasia syndromes.

FIGURE 3-2 Neuroanatomy of language. Numbers refer to Brodmann areas.

3
Reprinted with permission from Kirshner HS, Saunders. B 2004 Elsevier.

beyond syntax and includes pitch, tions,7 a dual-stream, cortical organiza-

melody, cadence, and tempo.4 The right tion of speech processing similar to that
hemisphere, in an analogous organiza- found for visual processing,8 and a role
tion to the language representation in for the cerebellum and subcortical struc-
the left hemisphere, mediates both pros- tures in the temporal processing of
ody and the interpretation of gesture.4 speech.9 Identifying the nodes and path-
The connectionist model of language ways of these distributed global net-
function, however, does not fully ex- works will advance understanding of how
plain how words are organized into sen- both focal lesions and neurodegenera-
tences. Therefore, the connectionist tive disorders affect the networks in-
model, which is based on lesion studies, volved in language function,10 especially
has recently come under scrutiny. Func- since different neurodegenerative dis-
tional brain imaging5 suggests language orders preferentially affect different large-
function is mediated by larger scale, dis- scale brain networks.11
tributed global networks in the brain,
explaining why many patients with aphasia NONCLASSIC APHASIAS
do not fit well into one of the clas- In addition to the classic aphasia syn-
sic connectionist aphasia syndromes.1 dromes, neurologists commonly encounter
The new models of the neuroanatomic a number of other aphasia syndromes.
basis of language are still under devel-
opment6 and will not be reviewed in Subcortical Aphasias
detail in this article. Some of the emerging Thalamic aphasia. Thalamic aphasia
concepts include subregions of Broca has been identified after left thalamic
area that serve different language func- hemorrhage and ischemic stroke (often

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 Language Dysfunction

KEY POINTS
h Aphasia can develop left tuberothalamic artery stroke). The monotonous speech pattern and de-
after injury to either aphasia that occurs after thalamic creased use of gesture.13 A classification
cortical or subcortical injury is variable and has been de- schema for the aprosodias analogous to
structures. scribed as a fluent aphasia with normal the classic aphasias has been proposed,4
h Deficits in prosody can repetition and more preserved compre- and evidence exists that the primary pro-
be mistaken for hension than that seen in Wernicke gressive aphasias (PPAs) can affect both
affective disorders and aphasia. In this way, thalamic aphasia receptive and expressive aspects of pros-
can be disabling. can resemble transcortical sensory ody. Deficits in prosody can be mis-
h Due to the nature of the aphasia. Perhaps due to involvement taken for affective disorders and can be
language network of other areas of the thalamus, this disabling, underscoring the importance
representation in the aphasia has been associated with a of nonverbal aspects of language. Def-
brain, deficits in written fluctuating course in not only language icits in prosody have also been impli-
versus spoken function, but also level of conscious- cated in both autism spectrum disorders
expressive language can ness, alternating between an alert state and schizophrenia.
occur; for this reason, with fairly normal speech and a lethargic
writing should be tested state with dysarthria, paraphasic errors, Alexias
separately in patients and hypophonia.3,12 Alexias (inability to read) can be divided
with aphasia. Striatal-capsular aphasia. This apha- into the following three types:
sia has also been associated with both Alexia with agraphia. Alexia with agra-
hemorrhages as well as infarctions that phia is essentially acquired illiteracy. In
involve the head of the left caudate nu- patients who have alexia with agraphia,
cleus, putamen, and surrounding white reading and writing are both impaired,
matter, including the internal capsule. whereas other language functions are
Due to internal capsule involvement, a spared with the possible exception of
right-sided hemiparesis is often associ- paraphasias and naming errors. Alexia
ated with this type of aphasia. Depend- with agraphia is associated with a lesion
ing on which structures are affected, in the left inferior parietal lobule, involv-
this aphasia can affect different parts of ing the angular gyrus. For this reason, it
language, but a common syndrome is the is often associated with Gerstmann syn-
anterior subcortical aphasia syndrome drome (in which patients exhibit agraphia,
associated with lenticulostriate artery acalculia, finger agnosia, and right-
stroke, which produces a nonfluent apha- left disorientation) and other features
sia often associated with dysarthria and of the angular gyrus syndrome such as
paraphasic errors. Lesions of the body anomia, constructional apraxia, and right
and tail of the caudate and putamen are inferior quadrantanopia.
not thought to cause aphasia.12 Alexia without agraphia (sometimes
In general, a common, although not called pure alexia). Alexia without agra-
universal, distinguisher between thalamic phia is associated with an inability to
aphasia and striatal-capsular aphasia is read, but with an intact writing ability,
that thalamic aphasias are fluent, whereas ironically causing patients to be unable
striatal-capsular aphasias are nonfluent. to read what they have just written. As-
sociated symptoms include difficulty with
Aprosodias color naming and right hemianopia. Some
Injury to the right hemisphere can im- patients also experience anterograde
pair the production, comprehension, and amnesia difficulties since the causative
repetition of prosody, but leave the other lesion in the left medial occipitotemporal
aspects of language intact.4 Lesions to junction next to or involving the splenium
the right frontal operculum can cause of the corpus callosum can impact epi-
an expressive aprosody leading to a flat, sodic memory networks.

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 Alexia associated with aphasia. Alexia referred to as paraphasias, are often
associated with aphasia is the alexia found identified during observation of
as part of an aphasia syndrome due to speech and are the production of
left frontal lesions causing Broca apha- unintended phonemes, morphemes,
sia or global aphasia. words, or phrases. These are
generally placed into the two
Pure Word Deafness categories of phonemic and semantic
Pure word deafness, a type of agnosia, is paraphasic errors, although other
the inability to understand speech despite classification schemes exist. A
otherwise intact language function. Pa- phonemic paraphasic error occurs
tients are generally aware of their deficit when a word is substituted with
and will sometimes describe speech as a nonword that retains a significant
sounding like a foreign language. Be- proportion (often over one-half)
cause this deficit is often seen with bi- of the intended word’s morphemes
lateral superior temporal gyrus injury from (the nonword sounds similar to
stroke (or, rarely, only left-sided lesions) the intended word). A semantic
it can be accompanied by an auditory ag- paraphasic error occurs when the
nosia, which is the inability to identify intended word is replaced by
nonspeech sounds. Pure word deafness another word that is inappropriate
is distinguished from cortical deafness in contextually, but often is semantically
which the patients report that they are related (replacing ‘‘horse’’ for ‘‘cow’’).
unable to recognize or differentiate any & Comprehension (verbal and written):
sounds. Cortical deafness is an uncom- Start with one-step midline
mon disorder.14 commands (‘‘close your eyes’’);
progress to distal one-step
ASSESSMENT OF LANGUAGE commands (‘‘hold up your left
FUNCTION hand’’); then progress to complex
Bedside Testing commands (‘‘point to the door
All neurologists should be able to assess after you point to the window’’).
the key aspects of language efficiently & Repetition: Start with short complete
and acutely at the bedside. A number of sentences and progress to an
published bedside language assessments open-ended phrase of at least five
exist15,16; however, as the bedside assess- words in length, such as one used
ment of language function is often more in the Boston Diagnostic Aphasia
qualitative than quantitative, many exam- Examination (‘‘near the table in
iners use one of their own creation. The the dining room’’).17
following is a suggested approach to the & Naming: Start with whole items. Ask,
bedside language examination: ‘‘What is this?’’ and point to the
& Observation: Listen to the patient’s object (eg, pen, watch), then
spontaneous speech to assess progress to parts (eg, watchband
articulation of words, fluency, and cuff of shirt). In patients who either
prosody. If the patient produces cannot perform these tasks or are
little spontaneous verbal output, ask nonfluent, test receptive naming
him or her to describe a picture by stating, ‘‘Point to the pen,’’ and
such as the cookie theft picture from hold out a pen and a watch.
the Boston Diagnostic Aphasia & Writing: Have the patient write
Examination, although any picture a sentence spontaneously. If the
showing action may be used. patient cannot produce a sentence
Paraphasic errors, sometimes spontaneously, have him or her

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 Language Dysfunction

KEY POINTS
h Bedside testing of try to write by dictation. Because language (including reading), repetition,
language function can written expression can be affected naming, verbal fluency, and, in some cases,
be done efficiently, but separately from verbal expression, prosody.18 Examples of comprehensive
for a comprehensive writing should be tested in language measures include the Boston
assessment of language, addition to testing verbal output. Diagnostic Aphasia Examination, Third
neuropsychological In addition, testing for an apraxia of Edition and the Western Aphasia Bat-
testing may be required. speech should be part of the evaluation tery.17,19 The Boston Naming Test20 is a
h Neuropsychological process for patients with a progressive widely used instrument to detect impair-
testing provides a aphasia. An apraxia of speech is a motor ments in naming associated with left
comprehensive and speech disorder characterized by a slow temporal dysfunction. Whereas bedside
objective assessment of
rate of speech and distorted speech assessment of naming usually relies on
multiple cognitive
sounds. To test for apraxia of speech, naming actual body parts and readily avail-
domains, including
expressive and receptive have the patient attempt to alternate able objects, the Boston Naming Test
language function, between labial (produced by the lips), uses pictures as the naming stimulus and
repetition, naming, verbal lingual (produced by the tongue), and provides robust normative data. Table 3-3
fluency, and prosody. guttural (produced by the soft palate provides an overview of common neuro-
h Neuropsychological and other throat structures) sounds psychological tests used to assess lan-
testing allows for the by saying words such as ‘‘patty-cake’’ guage function.
precise assessment of or ‘‘irresponsibility.’’
language by using DISORDERS AFFECTING LANGUAGE
normative data to Neuropsychological Testing Aphasia can occur after any injury to the
control for various Although the neurologist can test lan- portions of the brain’s language network.
demographic factors. guage function at the bedside, formal Stroke is a common cause of aphasia, and
neuropsychological testing allows for a the common ischemic stroke syndromes
more comprehensive assessment of lan- associated with aphasia are summarized
guage with normative data corrected for in Table 3-4. Case 3-1 gives an example
age and a variety of demographic vari- of the effects of an internal carotid artery
ables, such as gender, race, and educa- occlusion on language function.
tional level. Ample data support the Other disorders causing structural le-
utility of neuropsychological tests for sions such as intracerebral hemorrhage,
both localizing organic brain dysfunction traumatic brain injury, and brain tumors
and providing some degree of predictive may also produce aphasias. Since these
validity regarding the patient’s functional lesions do not typically damage a pre-
ability in daily life (Case 3-1) (Figure 3-3) dictable vascular territory, their associ-
(Table 3-2).16 Neuropsychological eval- ated neurologic features vary widely.
uations are comprehensive, assess mul- In addition to structural lesions, which
tiple cognitive domains, account for the can cause acute language dysfunction,
patient’s premorbid cognitive status, and many neurodegenerative disorders pro-
can be performed with the help of an gressively degrade language. Understand-
interpreter for individuals whose primary ing the distinguishing features of these
language is not English. In addition to a neurodegenerative diseases aids the neu-
primary emphasis on language, the eval- rologist in making the proper diagnosis
uation provides assessment of memory, and implementing appropriate treatment.
attention and executive functions, and While many neurodegenerative disorders
visual perceptual skills. affect language, this section will provide
Neuropsychological evaluation of lan- an overview of disorders that have a lan-
guage includes assessment of expressive guage disturbance as a defining feature
language (including writing), receptive early in the clinical presentation.

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 Case 3-1
A 34-year-old right-handed woman sustained an unexplained, spontaneous left internal carotid artery
dissection, which resulted in a large left middle cerebral artery stroke. Acutely, the patient evidenced
dysphagia, global aphasia, right hemiplegia, left gaze preference (right neglect), and was following less
than 25% of commands. Follow-up imaging several months later revealed large focal encephalomalacia
along the left middle cerebral artery territory, particularly affecting the inferior division (Figure 3-3).
On neuropsychological testing 18 months poststroke, the patient demonstrated impaired naming,
vocabulary, reading, writing, auditory repetition, and spelling capabilities (Table 3-2). Her speech
included decreased fluency and paraphasic errors. Examples of semantic paraphasias included
‘‘saxophone’’ for ‘‘harmonica,’’ and ‘‘buffalo’’ for ‘‘rhinoceros.’’ Phonemic paraphasias were also present such
as ‘‘tactus, actus, and cascus’’ for ‘‘cactus.’’ Her verbal output was greatly reduced as she did not speak
phrases longer than two to three words. She often provided one-word automatic statements, such as
‘‘sorry.’’ She was able to read several key words in a passage, but because her reading was so nonfluent
she had difficulty comprehending the context of the passage. The patient’s repetition was also impaired.
For example, the phrase, ‘‘When you go climbing, watch for falling rocks,’’ was repeated as, ‘‘Outside, up,
falling out.’’ Basic receptive language skills were more functionally intact, such as understanding test
instructions and following basic commands. Evidence of decreased insight and judgment was also present,
contributing to concern about her ability to live independently and capacity for decision making. For
example, the patient threw a lit cigarette into the trash can (which started a fire) in an attempt to avoid
being caught smoking by her mother.
Neuropsychological test results from 18 months poststroke provided strong evidence of localization to the
left hemisphere, including frontal (expressive), frontotemporal (receptive/comprehension), angular gyrus
(reading), and temporal (auditory memory) brain regions. The arcuate fasciculus and other perisylvian
connections were also affected given her impairments in repetition. Because of her severe deficits in
language, impairment in executive functioning (eg, decreased insight and judgment), and conflicted family
dynamics (eg, being subject to undue influence), she was determined to lack decision-making capacity by
her family physician and neuropsychologist. A court concurred, deeming the patient incompetent to
manage her affairs, and appointed the patient’s sister as her legal guardian.

FIGURE 3-3 Axial noncontrast CT of the patient in Case 3-1 showing encephalomalacia in the left
middle cerebral artery territory. A shows encephalomalacia of the inferior frontal
lobe and parietal lobes. B shows full thickness injury to the left temporal lobe.
Courtesy of Christopher Schaeffer, MD, PhD, Borg & Ide Imaging, Rochester, New York.

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 Language Dysfunction

Continued from page 635

TABLE 3-2 Neuropsychological Test Results for the Patient in Case 3-1

Ability Measure Performance Description

Expressive
Naming Boston Naming Test Severe impairment
Single word production F-A-S Test Severe impairment
Animals Severe impairment
Fluency Clinical observation Severe impairment
Semantic knowledge Wechsler Adult Intelligence Scale, Severe impairment
Fourth Edition (WAIS-IV) vocabulary subtest
Writing Qualitatively assessed Moderate impairment
Receptive
Comprehension Peabody Picture Vocabulary Test, Severe impairment
Fourth Edition
Boston Diagnostic Aphasia Severe impairment
Examination Complex Ideational
Reading Test of Premorbid Functioning Severe impairment
Wechsler Individual Achievement Severe impairment
Test (WIAT), Third Edition Word Reading
Pseudoword Decoding Severe impairment
Other Language
Repetition WIAT Sentence Repetition subtest Severe impairment
Prosody Qualitatively assessed Functionally intact
Syntax Qualitatively assessed Impaired
Other Cognitive
Visual perceptual WAIS-IV Perceptual Reasoning Index Average
Visuoconstruction Rey Osterrieth Complex Figure Test Average
Attention WAIS-IV Digit Span Subtest Severe impairment
Problem solving Wisconsin Card Sorting Test Mild impairment
Memory, auditory Wechsler Memory Scale (WMS-IV) Moderate impairment
Logical Memory I and II
Delayed Recall from Hopkins Average
Verbal Learning Test, Revised
Memory, visual WMS-IV Visual Reproduction I and II Low average to average
Rey Osterrieth Complex Figure Test Average

Comment. This case illustrates several important points. First, neuropsychological testing can
delineate various aspects of language functioning associated with a particular diagnosis, such as a
stroke. Second, the evaluation can provide increased specificity of localization. Additionally, testing
can provide diagnostic clarity, particularly over time, as individuals may improve to some extent. In this
case, the patient acutely demonstrated global aphasia, which improved to primarily Broca aphasia
with less severe receptive language deficits at 18 months poststroke. Third, the case demonstrates that
severe language deficits are often accompanied by other areas of cognitive dysfunction, such as
impaired memory and executive functioning (eg, decreased insight and judgment, being subject to
undue influence). Fourth, objective cognitive test data can be used to determine that a patient lacks
decision-making capacity. Finally, the case illustrates the devastating effects that global aphasia
has, even in an atypically young patient.

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 a
TABLE 3-3 Suggested Neuropsychological Tests of Language

Ability Sample Tests Brief Description of the Test

Expressive
Naming Boston Naming Test Sixty drawings of common objects are presented with increasing
difficulty. Early items include scissors and a camel, with more difficult
items being tongs and a trellis.
Single word Controlled Oral Word The patient has 60 seconds to orally list words that start
production Association Test with the letters ‘‘F, A, and S;’’ proper nouns and repetitions are
(F-A-S Test, animals) not counted.
Delis-Kaplan Executive The D-KEFS uses F-A-S Test and animals, but provides additional
Function System subtests for assessing semantic fluency, including boys’ names and a
(D-KEFS) verbal fluency category-switching test (ie, alternating between fruits and furniture).
subtest
Fluency Cookie theft picture The patient orally describes what is happening in a scene,
from the Boston which the examiner records or writes down verbatim.
Diagnostic Aphasia
Examination (BDAE)
Writing Thurstone Word Five minutes are given to write down words that start with ‘‘S’’
Fluency Test and 4 minutes are given to write four-letter words that start with ‘‘C.’’
Spontaneous writing Patients can be asked to write about any topic.
and writing to dictation
Receptive
Comprehension Token Test Verbal commands of increasing complexity are provided using
tokens of various shapes and colors.
BDAE complex Patient responds to a series of yes/no questions after hearing
ideational a short story.
Naming Peabody Picture Assesses receptive vocabulary through a multiple-choice, nonverbal
Vocabulary Test, response format. The examiner says a word, and the examinee points to
Fourth Edition the corresponding picture from an array of four illustrations.
Reading Test of Premorbid These four tests assess reading recognition by having patients read a list of
Functioning words aloud. The words are irregularly spelled (eg, cough) to minimize
Wide Range Achievement patients’ ability to apply standard pronunciation rules and maximize
Test 4 assessment of their prior learning of the word. Scores are often used
to assess premorbid intellectual functioning.
National Adult
Reading Test
Wechsler Individual
Achievement Test,
Third Edition (WIAT-III)
Other Language
Functions
Repetition Western Aphasia Battery The patient repeats single words of increasing complexity,
repetition subtest followed by short sentences. Points are deducted for
phonemic paraphasias and errors in word sequence.
WIAT-III sentence The patient is asked to repeat sentences of
repetition subtest increasing complexity.

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 Language Dysfunction

a
TABLE 3-3 Suggested Neuropsychological Tests of Language Continued from page 637

Ability Sample Tests Brief Description of the Test

Prosody Advanced Clinical The patient listens to brief recordings and is asked to match the speaker’s
Solutions Social tone of voice with an emotion (eg, happy, sad, angry, afraid,
Perception Test surprised, disgusted, neutral, sarcastic).
Syntax Northwestern A picture and corresponding word cards are presented in random
Anagrams Test order, requiring the patient to use all the words to make a sentence
about the picture.
Semantic Wechsler Adult The patient orally defines various words of increasing complexity.
knowledge Intelligence Scale,
Fourth Edition
vocabulary subtest
Pyramid and Palm Forced choice format is used to assess the degree to which a patient can
Trees Test access semantic meaning from pictures and words. Six different
versions of the test are possible by using either pictures or written
or spoken words to change the modality of stimulus or
response items.
a
For a detailed review of neuropsychological assessment of language, including screening tests and comprehensive language batteries,
see Lezak MD, et al, 2004.16

a
TABLE 3-4 Stroke Syndromes Causing Aphasia

Associated
Arterial Territory Aphasia Syndrome Associated Signs Behavioral Symptoms
Superior division of Broca Right hemiparesis, Depressed affect, frustration
middle cerebral artery possible right
hemisensory loss
Superior division of middle Transcortical motor Right leg more than Abulia (lack of motivation)
cerebral artery or anterior arm weakness
cerebral artery
Inferior division of Wernicke Right superior Agitation, anosognosia
middle cerebral artery quadrantanopia
Watershed infarct Transcortical Variable Variable
sensory
Proximal middle Global aphasia Right hemiparesis, Variable
cerebral artery or hemisensory loss,
internal carotid artery and hemianopia
Branch of inferior or Conduction Right hemiparesis, No typical symptoms
superior division of aphasia right hemisensory
middle cerebral artery loss, apraxia
Many Anomic aphasia Variable No typical symptoms
a
Presumed left hemisphere dominance.

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 KEY POINTS
Alzheimer Disease are not well categorized by the current h The clinical syndrome of
Memory impairment and anomia are classification system.25 The variants agPPA primary progressive
often the earliest cognitive symptoms in and svPPA are currently thought to be a aphasia can be caused
Alzheimer disease, although primary lan- subtype of frontotemporal lobar degen- by Alzheimer disease or
guage presentations occur, as is discussed eration (FTLD) and share underlying frontotemporal lobar
in the section on the logopenic variant of pathology, most often tau-positive or degeneration pathology.
primary progressive aphasia. Confronta- TAR-DNA binding protein 43 (TDP-43)Y h Agrammatic variant of
tion naming can be impaired early in the positive neuronal inclusions, whereas primary progressive
disease process and markedly worsens as lvPPA often has underlying Alzheimer aphasia is characterized
the disease progresses. Alzheimer disease disease pathology.26 The PPA variants by loss of grammar
also impacts verbal fluency, affecting se- leading to progressively
have corresponding patterns of regional
nonfluent speech.
mantic fluency (eg, ability to name animals atrophy affecting dominant hemisphere
in 1 minute) greater than phonemic flu- language networks. Disease progression
ency (eg, ability to name words begin- is associated with more widespread left
ning with ‘‘F’’ in 1 minute).21 However, hemisphere atrophy and increasing in-
verbal fluency has not been found to be volvement of the right hemisphere. There-
a strong predictor of progression from fore, cognitive deficits evolve beyond
mild cognitive impairment to Alzheimer language dysfunction, including the devel-
disease, and semantic fluency deficits have opment of behavioral abnormalities.
not been consistently found to discrim- Agrammatic variant of primary pro-
inate Alzheimer disease from other de- gressive aphasia. Patients with agPPA,
mentias such as patients with dementia sometimes referred to as progressive
from Parkinson and Huntington dis- nonfluent aphasia or the nonfluent/
ease.22 Advanced Alzheimer disease agrammatic variant of PPA, have progres-
devastates multiple cognitive processes sive impairment of grammar leading to a
resulting in aphasia, agnosia, and apraxia nonfluent style of expressive communi-
as well as amnesia.18 cation. Some patients exhibit an associ-
ated apraxia of speech. Both speech and
Primary Progressive Aphasias writing become nonfluent and effortful,
The primary progressive aphasias (PPAs) with phonological and grammatic errors
are progressive neurodegenerative apha- progressively increasing. Comprehension
sia syndromes that generally occur with- of words and repetition remain intact
out other significant cognitive impairment except for syntactically complex sentences.
early in the disease course. In fact, many Speech articulation can be disturbed if
patients remain dementia-free for at least there is an associated apraxia of speech.
2 years and as long as 10 years,23 although On imaging, agPPA is commonly asso-
almost all progress to a dementia syn- ciated with atrophy and hypometabolism
drome as the disease impacts other cog- of the left posterior inferior frontal and
nitive domains. Three variants of PPAs insular cortices.25,27 Pathologically, agPPA
have been described: agrammatic variant is associated with frontotemporal degen-
of primary progressive aphasia (agPPA), eration with tau-positive neuronal in-
semantic variant of primary progressive clusions, in particular when there is an
aphasia (svPPA), and logopenic variant of associated apraxia of speech; however,
primary progressive aphasia (sometimes some patients with agPPA may have
called logopenic phonological aphasia) other underlying pathologies including
(lvPPA). Diagnostic criteria have been progressive supranuclear palsy, corticobasal
proposed for the different variants,24 al- degeneration, and tau-negative fronto-
though a number of patients with PPA temporal degeneration.28

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 Language Dysfunction

Semantic variant of primary progres- to comprehend the meaning of words,

sive aphasia. Patients with the svPPA, although the word may seem familiar.
sometimes referred to as semantic de- Grammar and syntax are often spared.
mentia, have progressive anomia and Unlike other dementias, recent memo-
impairment in single word compre- ries may be better preserved than
hension. Although patients present remote memories.29 On imaging, atro-
with word-finding difficulties, the most phy and hypometabolism of the ante-
striking finding is impaired knowledge rior left temporal lobe is common,
of word meaning, identified on test- particularly the inferior and middle
ing as a single word comprehension temporal gyri. The amygdala and hippo-
deficit (Case 3-2). svPPA patients fail campus may also be affected.25,27,30 The

Case 3-2
An 86-year-old woman presented for ‘‘memory loss,’’ but she could not relay her history because of frequent
word-finding problems and difficulty with comprehension. The patient had begun referring to everyone as
‘‘she’’ and had been referring to words in a strange way. During a discussion about a hotel, she asked,
‘‘What is Holiday Inn?’’ More recently, the patient’s children had to take over managing all of her daily
activities. She lost the ability to use the microwave and television remote control, and she also became
obsessed with household cleaning and spent all day performing the same cleaning activity multiple times.
Despite having no personal history of stroke, she was previously diagnosed with vascular dementia
based on MRI findings that had been interpreted to be due to a stroke (Figure 3-4). Her examination was
dominated by a comprehension deficit with frequent paraphasic errors. Some errors were phonemic, such
as saying ‘‘pretzel’’ when asked to name a picture of a cello, but some responses were nonsensical, as
when she said ‘‘for rain, wine, onion’’ when asked to name a picture of a saw. She did not have an apraxia of
speech. The patient’s neuropsychological testing is shown in Table 3-5.

FIGURE 3-4 Axial fluid-attenuated inversion recovery (FLAIR) MRI of the patient in
Case 3-2 showing left anterior temporal lobe atrophy with surrounding gliosis (A, B).
Note atrophy of both the amygdala and hippocampus (left greater than right) in panel B.
Courtesy of Christopher Schaeffer, MD, PhD, Borg & Ide Imaging, Rochester, New York.

Continued on page 641

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 Continued from page 640

TABLE 3-5 Neuropsychological Test Results for the Patient in Case 3-2

Ability Measure Performance Description

Expressive
Naming Boston Naming Test Impaired
Single word production F-A-S Test Impaired
Animals Impaired
Fluency Clinical observation Fluent with paraphasic errors
Semantic knowledge Pyramids and Palm Trees Test Impaired
Writing Qualitative assessment Fluent with paraphasic errors
Receptive
Naming Peabody Picture Vocabulary Test, Impaired
Fourth Edition
Comprehension Qualitative assessment Impaired
Reading Test of Premorbid Functioning Low average
Other Language
Repetition Qualitative assessment Impaired
Prosody Qualitative assessment Normal
Syntax Northwestern Anagrams Test Normal
Other Cognitive
Visuospatial Complex figure copy and judgment Average
of line orientation subtests of the
Repeatable Battery for Assessment
of Neuropsychological Status (RBANS)
Attention Digit Span Subtest of RBANS Impaired
Executive function Trail Making Test A Impaired
Memory, auditory List learning task and story memory Impaired
subtests of RBANS
Memory, visual Complex figure recall from RBANS Impaired

Comment. The patients’ case highlights several common issues that arise in patients with
semantic variant of primary progressive aphasia (svPPA). First, patients are often referred for memory
dysfunction, but this concern is often an overgeneralization, and identifying the actual initial presenting
symptom is critical to making a correct diagnosis. Next, the patient’s MRI findings of marked
asymmetric anterior temporal lobe atrophy with surrounding gliosis is suggestive of svPPA, but these
findings, not uncommonly, will be misread as a stroke or traumatic brain injury. Next, patients with svPPA
have a single word comprehension deficit that makes them unable to follow even simple sentences
and causes them to refer to words in the abstract, suggesting that the link to semantic knowledge of
an item is disrupted. These deficits in receptive and expressive naming in svPPA often manifest on
neuropsychological testing as difficulty understanding instructions for cognitive testing, leading to a
pattern of diffuse impairment. Lastly, typical of patients with svPPA, the patient’s language deficits
progressed and affected other aspects of cognition and behavior seen in the behavioral variant of
frontotemporal degeneration.

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 Language Dysfunction

KEY POINTS
h Semantic variant of consistent finding of anterior tem- leading some to refer to lvPPA as the
primary progressive poral lobe involvement in svPPA has ‘‘unihemispheric’’ or ‘‘language’’ pre-
aphasia is characterized suggested this area is part of a semantic sentation of Alzheimer disease.
by single word memory network. Pathologically, svPPA
comprehension deficits. is associated with non-tau neuronal Language Impairment of
h Logopenic variant of inclusions, most often TDP-43 neuronal Unexplained Etiology
primary progressive inclusions.31,32 (Functional or Psychogenic)
aphasia is characterized Logopenic variant of primary pro- Slowed speech with an atypical halting
by nonfluent, effortful gressive aphasia. Patients with lvPPA, or stuttering quality may be suggestive
speech with an anomia, sometimes referred to as logopenic pho- of a functional or psychogenic aphasia.
but without a single word nological aphasia, have speech that is The absence of a medical condition to
comprehension deficit. nonfluent, effortful, and have frequent account for the abnormal language symp-
h The current clinical word-finding pauses resulting in an toms is an important initial clue for con-
classification systems for anomia without a single word compre- sidering a functional aphasia. For example,
patients with primary hension deficit. Patients may also a concussion or mild traumatic brain
progressive aphasia do exhibit difficulty with repetition, partic- injury resulting in significant focal lan-
not permit accurate ularly of sentences and may make guage deficits (eg, impaired articulation,
prediction of phonological errors, which involve pat- nonfluent speech, agrammatism, aproso-
underlying pathology.
terns of sound errors. For example, dia) is highly atypical and should raise
h Detecting a psychogenic patients may substitute sounds made suspicion of a psychogenic origin.36
language disorder is a in the back of the mouth like ‘‘K’’ and Fluency impairment in conversational
rigorous process of ‘‘G’’ for those in the front of the mouth speech (as opposed to verbal fluency tests)
exclusion and requires
like ‘‘T’’ and ‘‘D’’ (eg, saying ‘‘tup’’ for following uncomplicated mild traumatic
understanding the
‘‘cup’’ or ‘‘das’’ for ‘‘gas’’).33 In addi- brain injury should alert clinicians to
psychosocial factors
that contribute to
tion to aphasia, patients have reduced assess motivation, financial, psychoso-
symptom production. digit span and, usually, impaired ver- cial, and psychiatric issues perpetuating
bal episodic memory. On imaging, symptom maintenance.37 Other poten-
patients with lvPPA demonstrate corti- tial indicators of psychogenic language
cal atrophy in the left posterior superior disorders after mild traumatic brain injury
temporal, inferior parietal, medial tem- include experiencing late-onset language
poral, and posterior cingulate gyri.34 As difficulties (eg, months postinjury),
the disease progresses, atrophy de- reporting deficits worsening over time,
velops in the left anterior temporal and exhibiting an inconsistent abnor-
and frontal cortices, moving to the right mality in speech patterns. An example
hemisphere, where it affects the of language inconsistency is an inconsis-
temporoparietal junction, posterior cin- tent paraphasic error for a certain sound,
gulate, and medial temporal lobe. On such as incorrectly reproducing sounds,
fluorodeoxyglucose positron emission like saying ‘‘torry’’ for ‘‘sorry’’ but later
tomography (FDG-PET) imaging, lvPPA correctly saying the word ‘‘scene.’’
patients have the bilateral tempo- Stuttering acquired in adulthood can
roparietal hypometabolism pattern also be indicative of a psychogenic cause
typical of Alzheimer disease as well and is more likely to be so if it does not
as left lateral frontal and medial improve in situations that usually improve
parietal lobe hypometabolism.27 Re- fluency, such as speaking or singing in
cent evidence suggests that lvPPA unison or when performing over-learned
includes Alzheimer disease pathology recitation tasks (eg, saying the days of
in a high proportion of cases and the week or counting). Inconsistencies
may be the most common aphasia can also be associated with psychogenic
phenotype of Alzheimer disease, 35 stuttering. This includes periods of time

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 KEY POINTS
with no stuttering, marked differences Noninjury factors also play an impor- h Mild traumatic brain
between conversation and reading, and tant role in a patient’s recovery, including injury is not typically
worsening of stuttering when performing premorbid intellectual and educational associated with significant
less difficult tasks.38 levels and cognitive reserve, personality focal language
Individuals with psychogenic language characteristics, emotional stability, level disturbance, and,
disorders often present with consider- of family support, and accessibility of ser- therefore, focal language
able distress about their condition while vices. Patients with more diffuse cog- dysfunction after mild
minimizing any potential psychological nitive dysfunction in addition to focal traumatic brain injury
language deficits will experience other should raise concern for a
issues they may be experiencing. Com-
barriers to recovery. For example, pa- psychogenic cause.
mon risk factors for conversion disorder
include history of psychological trauma tients with preaphasia frontal lobe dysfunc- h Treatment of language
tion may experience decreased insight impairment varies based
and histrionic personality disorder fea-
into their deficits as well as personality on etiology but often
tures associated with attention seeking,
and mood changes that may adversely requires addressing
somatic symptom disorders, and sec- physical, cognitive, and
ondary gain incentives (eg, litigation, impact recovery. Patients with preaphasia
emotional factors
disability claims, and increased atten- temporal lobe dysfunction may experience
through a comprehensive
tion from caretakers). memory impairment that may limit their rehabilitation program
Evaluation of a functional or psy- ability to utilize compensatory strategies. targeting restorative and
chogenic language disorder includes Length of recovery from focal dam- compensatory approaches.
a thorough physical examination and age to language areas varies but gener-
neurologic workup to ensure the proper ally ranges from 1 to 2 years postinjury.
differential diagnosis. Obtaining collateral While the most dramatic spontaneous
information from the patient’s primary improvements tend to occur in the first
care provider and family members or several months, the use of physical,
caregivers is necessary to provide a frame- speech, and occupational therapies can
facilitate restorative gains and compen-
work for understanding the patient’s
satory adjustments well beyond that point.
functioning. In many cases, referral for
Referrals to speech therapy are ap-
neuropsychological evaluation is useful
propriate for focal language deficits and
to assess motivation, effort, and language
other cognitive dysfunction. Acute ther-
skills. Psychotherapy is the treatment of
apy emphasizes brain stimulation to re-
choice for conversion and somatic symp- store functioning and recruit new neuronal
tom disorders, including functional or pathways. Patients can also benefit from
psychogenic language disorders. returning to outpatient speech therapy
and cognitive rehabilitation several years
TREATMENT/RECOVERY
after their injury to develop and fine-
Recovery from acute causes of language tune compensatory strategies to adjust
disturbance, such as stroke or traumatic for persistent deficits. There has been
brain injury, is dependent on multiple in- some support for the efficacy of speech
jury factors such as the size and location therapy to treat language disturbance
of the area injured. There is evidence for in PPA; however, studies suggest that
neuroplasticity as a mechanism for recov- improvements on specific language therapy
ery. After ischemic stroke, areas of the brain exercises are neither readily generaliz-
recruited during performance of language able to other language tasks, nor main-
vary for distinct language tasks and also tained for more than several months after
across individuals with similar size and site treatment discontinuation.40
of lesions. However, individuals with ex- In addition to speech therapy, some
tensive lesions in Broca area are less able support exists for the use of donepezil
to recover language function.39 to treat poststroke aphasia and memory
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 Language Dysfunction

dysfunction.41 Due to the lack of evi- Neuroimage 2012;62(2):816Y847.

doi:10.1016/j.neuroimage.2012.04.062.
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and memantine in neurodegenerative 7. Amunts K, Lenzen M, Frederici AD, et al.
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