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Chapter

Primary Progressive Aphasia

(PPA)
Yashaswini Channabasave Gowda and Hema Nagaraj

Abstract

Primary progressive aphasia (PPA) is a rare neurological condition that affects

an individual’s ability to communicate. PPA is a syndrome characterized by a
‘progressive worsening of language with preservation of the activities of daily
living and evidence of relatively normal non-verbal abilities on neuropsychological
testing’. It commonly appears initially as a disorder of speaking (an articulatory
problem) progressing to nearly total inability to speak in its most severe stage, while
comprehension remains relatively preserved. This chapter provides an overview of
the characteristic features of PPA, its classification, assessment, and rehabilitation
options.

Keywords: Primary Progressive Aphasia, neurology, speech-language, dementia,

assessment, management

1. Introduction

Primary Progressive Aphasia (PPA) is a type of dementia characterized by a loss

in one or more language functions in people under the age of 65 (and occasionally as
early as the 40’s). It is a type of dementia and the condition is caused due to degenera-
tion of nerve cells in the brain which control our ability to communicate [1].
PPA is a condition marked by a steady deterioration of language skills while
maintaining everyday activities and showing relatively normal nonverbal abilities
in neuropsychological testing. It frequently begins as a speech deficit (an articula-
tory problem), advancing to near total inability to speak in its most severe stage, but
understanding remains largely intact.
Other neurological illnesses, on the other hand, exist in which language degen-
eration is simply one aspect of a broader, progressive decrease in mental processes
such as memory, attention, visuospatial skills, reasoning, and the ability to
perform complicated physical activities. The generation of phonemic paraphasias
in naming was offered as a further criterion for separating PPA from progressive
aphasia in likely Alzheimer’s disease [2].

2. A retrospective delineation

Pick [3] described a patient who had a progressive language disorder that was
linked to left temporal lobe atrophy. This seminal paper was followed by a flood
of cases [1, 2], and it became clear that PPA can present in both fluent and non-
fluent people. Over the next few decades, there were intermittent incidents [1, 4].

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Aphasia Compendium

Mesulam’s describes PPA in the context of atrophy seen in the left focal perisylvian
region or temporal lobe [5]. Speech is grammatically structured and well-articu-
lated but gradually loses content words.
The study of patients with a progressive deficit in semantic memory began at the
same time as the study of cognitive issues linked with neurodegenerative disease [6,
7]. The rising anomia exhibited in these people, according to Warrington, was caused
by a fundamental loss of semantic memory, which impaired object recognition and
knowledge, as well as word discovery and comprehension. Following more evidence
of impaired nonverbal conceptual understanding (semantic memory) as well as
anomia in these patients, the term ‘SD’ was coined. The 1998 consensus declaration is
often quoted. Associative agnosia (difficulty recognizing/identifying items) and/or
prosopagnosia (difficulty recognizing/identifying known or renowned individuals)
were supported as criterion for SD in the widely recognized 1998 consensus state-
ment. PPA is currently included in the frontotemporal lobar degeneration, fronto-
temporal dementia, Pick’s disease, or Pick’s complex family of disorders.

3. Characteristics of PPA

Main characteristics of PPA [8] are as follows:

• Age of onset ranges from 40 to 75, with a mean of 59.3 years.

• The involvement of males predominates over females, with the ratio of 2:1.

• The duration of isolated language symptoms can range from 1 to 15 years with
a mean of 5.3 years.

• Autopsy findings reported for 14 cases disclosed; Pick’s a disease in 4,

Creutzfeld—Jakob’s disease in 3, Alzheimer’s disease in 3, focal spongiform
degeneration in 2, and nonspecific cellular changes in 2. The diversity of
pathology does not support the existence of a specific disease underlying
isolated language decline.

• Of the 47 cases with CT scan, 13 were normal, 5 showed a diffuse abnormality,

10 had greater left than right abnormality, and 19 had left hemisphere abnor-
mality only.

• Most of the reported cases had predominantly fluent, anomic, or Wernicke’s like
aphasia, but 12 cases with nonfluent or Broca’s aphasia have been described.

• The description of cases with PPA has aided in the long-running, more general
debate over whether a stage model or a subgroup model [9] might be more
appropriate for explaining the neuropsychological consequences of certain
degenerative brain diseases, as they appeared to support a subgroup model.

• Subgroup models do not presume that cognitive performance is disintegrated

uniformly. Rather, they assume that observed disparities between patients and
patient groups are qualitative in origin and represent impairments in various
cognitive modules.

• Stage models, on the other hand, assume a global progression of cognitive defi-
ciencies with solely quantitative changes over time, with observed disparities

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Primary Progressive Aphasia (PPA)
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between patients and patient groups explained by distinct points on a one-

dimensional time-axis.

Early symptoms:

• Difficulties recalling the names of people and objects,

• Difficulties expressing one’s thoughts orally and sometimes difficulty under-

standing others.

• Memory of recent events, attention/concentration, judgment and reasoning

abilities, and visuospatial abilities are relatively normal.

• Some patients tend to say or nod “yes” for “no” and vice versa.

• Gradual difficulty in one or more of the following language functions like

speaking, understanding, reading and writing.

• Inability to follow conversations or verbal instructions.

• Inability to read or write a letter.

• Decreasing content in speech or garbled speech to eventually becoming mute.

Later symptoms:

• As PPA progresses, it becomes increasingly difficult to communicate verbally

by any means.

• The capacity to comprehend what others are saying or reading deteriorates

as well.

• Inability to understand speech.

• Difficulty following conversations, especially in bigger groups.

• Requesting information to be repeated and misinterpreting what is said despite

normal hearing.

• Early in the course of the illness, speech may become empty of any genuine
information and difficult to grasp.

• Eventually, speech may be unable to communicate, leading to muteness.

• Math and calculating problems.

• Loss of ability to do even elementary mathematical processes.

Diagnostic criteria for PPA [10]:

• A distinctive feature is an insidious onset and slow advancement of word-

finding, object naming, or word understanding as shown during spontaneous
conversation or as tested through formal neuropsychological language tests.

3
Aphasia Compendium

• For at least 2 years following, the language impairment is responsible for all
limitations in daily living activities.

• Premorbid linguistic function is intact (except for developmental dyslexia).

• Within the first 2 years of the illness, there was no notable apathy, disinhibi-
tion, forgetfulness for recent events, visuospatial impairment, visual recogni-
tion deficiencies, or sensory-motor dysfunction.

• Even in the first 2 years, acalculia and ideomotor apraxia may be present (mild
constructional deficits and perseveration are also acceptable as long as neither
visuospatial deficits nor disinhibition influence daily living activities).

• Other domains may be impacted after the first 2 years, but the language
remains the most impaired function and deteriorates quicker than other
afflicted domains throughout the illness.

• Neuroimaging has revealed that there are no “particular” reasons such as

stroke or tumor.

4. Types of Primary Progressive Aphasia

The most common type of brain degeneration found after brain autopsy in
individuals with PPA (60% of cases) is Nonspecific degeneration (NSD) where there
is evidence of brain cell death, but no features of Alzheimer’s or Pick’s disease. Less
commonly, Pick or Alzheimer’s disease may be found.
Primary progressive aphasia, or PPA, is a group of Fronto-Temporal Dementia
(FTD) symptoms defined by a progressive loss of capacity to talk, read, write, and
understand what others are saying. When the following three conditions are met,
PPA is diagnosed:

1. There is a steady deterioration in verbal ability (not just speech).

2. At first, the sole disability is a linguistic barrier.

3. A neurological illness is the root reason.

PPA is further divided into three clinical subgroups based on the language abilities
that are most impacted, according to experts.

4.1 Semantic variant of PPA

The increasing loss of word meanings is a feature of semantic variant PPA

(svPPA). The disorder is sometimes known as semantic dementia if there are
extra substantial issues recognizing things or faces. Other language skills remain
unchanged, such as the ability to make speech and repeat words and sentences
uttered by others. However, while the affected person may continue to speak flu-
ently, their speech becomes hazy and difficult to comprehend due to the omission
or substitution of several words. People with svPPA (also known as PPA-S) may
demonstrate changes in behavior compared to those seen in behavioral variant of
Fronto-Temporal Dementia (bvFTD), such as disinhibition and rigid food prefer-
ences, as the disorder progresses.

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Signs and symptoms:

• Anomia—An inability to recall the names of objects; difficulty “finding the

right word.” (“car” for “truck.”)

• Reduced single-word comprehension—The person affected is unable to

recall what words mean, especially words that are less familiar or less fre-
quently used.

• Impaired object knowledge—Being unable to remember what a familiar

object is or how it is used.

• Surface dyslexia/dysgraphia—Reading and writing words that do not follow

standard pronunciation or spelling conventions can be difficult; such words
are spelled or spoken “as if ” they do. For example, the person might write “no”
instead of “know” or misread “wide” as “broad.”

4.2 Non-fluent variant of PPA

People with the non-fluent/agrammatic form of PPA (nfvPPA, also known

as PPA-G), also known as progressive non-fluent aphasia or PNFA, have trouble
speaking but, can remember the meanings of individual words. Degeneration of the
areas of the brain that regulate specific linked muscles causes them to lose their abil-
ity to make sounds with their lips and tongue; nevertheless, the muscles themselves
are intact. Apraxia of speech is the technical term for these issues (AOS). As a result,
their speech becomes labored and slow, and they may appear to be physically strug-
gling to speak. However, speech issues alone are not enough to diagnose PPA. When
speech rather than language is the primary difficulty, the diagnosis is progressive
AOS rather than PPA. The impairment of grammar is a distinctive characteristic.
When speaking, people with nfvPPA make a variety of errors, such as deleting small
grammatical terms, wrongly employing word ends and verb tenses, and/or messing
up word order in phrases. Some people may eventually develop swallowing dif-
ficulties as well as more generalized motor symptoms, comparable to those found in
movement-predominant FTDs such as corticobasal syndrome.
Signs and symptoms:

• Apraxia—difficulty making the necessary lip and tongue movements for

speech. This causes distorted or inaccurate speech sounds, as well as slow,
strained speech and groping facial and lip motions in an attempt to generate
the correct sound. The initial symptom is generally labored speaking. Words
with several syllables are the most difficult to create.

• Agrammatism—due to omissions and inaccuracies, speech becomes limited to

short, simple phrases that are difficult to understand by the listener.

• Difficulty understanding long or grammatically difficult sentences. Single-

word comprehension is unchanged, but the capacity to grasp long or gram-
matically difficult sentences is impaired. People with PPA may find it more
difficult to comprehend what they see as “too much” verbal information, such
as watching television or following a group conversation.

• Mutism

5
Aphasia Compendium

• Difficulty swallowing-This occurs later in the disease’s course.

• Movement impairments similar to Parkinson’s disease can arise. Slow, stiff

movement, loss of balance or falling easily, trouble moving an arm or leg, and
restricted up-and-down eye movement are all possible symptoms.

4.3 Logopenic variant of PPA

When speaking, people with logopenic variation PPA (lvPPA, also known as
PPA-L) have trouble choosing words. As a result, individuals may speak slowly and
pause frequently while searching for the appropriate word. They can still remember
the meanings of words, unlike persons with semantic variant PPA. Speech can be
absolutely fluent during the small discussion but become hesitant and halting when
the person has to be particular or use a more unfamiliar word, unlike persons with
agrammatic PPA. Speech is normally unforced and undistorted. The lvPPA type also
has a short attention span for words, which makes it difficult to repeat phrases and
sentences. As the condition advances, those who are affected may have difficulty
understanding complex sentences.
Signs and symptoms:

• Impaired single-word retrieval

○ Finding the correct word while speaking is difficult.

○ Due to the time required for word recovery, there are pauses and hesitations.

○ An extended description (circumlocution) can be used to replace a lost word.

• Impaired repetition-Longer phrases and sentences pose more difficulty.

• Phonological speech errors-Omissions and substitutions in speech sounds

are examples of mistakes. For instance, the affected individual may substitute
sounds made at the tip of the tongue, such as “t” or “d,” for sounds made near
the neck, such as “k” or “g,” as in “tup” instead of “cup” or “dap” instead of
“gap.” They can remove final consonants, such as “slee” rather than “sleep.”

• Phonological paraphasias-A non-word with some of the same sounds is

substituted for a real word. For example, the affected person might say “lele-
phone” instead of “telephone.”

• Poor comprehension of complex sentences-With single-word comprehen-

sion spared.

• Difficulty swallowing-This may develop later in the progression of the

disease.

5. Speech, language, and communication assessment of PPA

5.1 History

First, a thorough medical history is taken to determine whether a dementia con-

dition, as stated above, exists. Because the patient may be ignorant of the symptoms

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Primary Progressive Aphasia (PPA)
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(as in the case of memory loss or personality changes) or unable to describe them
owing to aphasia, family members or friends are frequently questioned about the
patient’s behavior.
Case history and interview:

• It is critical that a thorough case history be obtained.

• Document the initial presentation as well as the emergence of any additional

symptoms over time.

• Pay attention to characteristics of speech and language. At the time of evalu-

ation, the degree of linguistic versus cognitive or motoric impairment (e.g.,
word-finding difficulties, agrammatism, phonological errors)

• Nonverbal cognitive deficits that could worsen over time (e.g., episodic
memory impairment).

• Atypical behavioral symptoms (e.g., disinhibition, apathy, loss of empathy)

that can arise, particularly in semantic variant PPA, or motoric symptomatol-
ogy (e.g., limb apraxia, Parkinsonism, dysphagia) that can develop, most
commonly in nonfluent/agrammatic PPA. If not previously indicated in the
medical record, any family history of dementia or other pertinent medical
diagnoses (particularly neurodegenerative disorders) should be investigated.

• Disabilities in hearing and/or vision should be documented.

• Whether the person is monolingual, bilingual, or multilingual.

The therapist should enquire about existing functional communication

demands and constraints in order to inform therapy planning. It’s crucial, for
example, to figure out which communication contexts (work, home, commu-
nity), partners (family, friends, coworkers), and modes (telephone, face-to-
face, written) are most significant in the patient’s daily life. If possible, include
the individual’s primary communication partner(s) to establish a thorough and
accurate case history.

5.2 Neurological examination

A neurological examination is performed to see if there is evidence of dementia

on a simple mental function screening (the mental status assessment) as well as
signs of motor or sensory symptoms that indicate various forms of neurological
illnesses could be causing dementia. The neurologist may also arrange tests, such
as an MRI scan, to rule out the possibility of a stroke or tumor as the cause of the
symptoms.

5.3 Brain imaging studies

This can be done with a CAT scan or an MRI scan. Both of these technologies
create an image of the brain, allowing any structural abnormalities like a stroke,
tumor, or hydrocephalus—all of which can cause dementia-like symptoms—to be
recognized.
The CAT scan and MRI scans in the case of degenerative brain disease may
reveal “atrophy,” which indicates “shrinkage” of the brain tissue, or they may not

7
Aphasia Compendium

show anything at all, especially in the early stages. In fact, the report is frequently
returned as “normal.” However, this just implies that no evidence of a tumor or
stroke exists. It is unable to provide information on the microscopic degenerative
changes that have occurred.

5.4 Neuropsychological examination

It provides a more thorough assessment of mental health. This is especially

crucial in the early stages of sickness, when a standard screening examination may
fail to discover the patient’s difficulties. This takes several hours and consists of
tests of mental talents, such as attention and focus, language learning and memory,
visual perception, logic, and mood, conducted by paper and pencil or computer.
The results can reveal whether there are any irregularities in thought or behavior,
as well as their severity (mild, moderate, or severe). Because we normally test
memory by telling someone something and then asking them to repeat it later,
demonstrating that people with PPA have intact memory can be difficult. In an
individual with PPA, it may be impossible to repeat back the information because
of the aphasia.
As a result, it’s critical that testing is done adequately to ensure that there is not
an actual memory loss. To yet, the only way to objectively diagnose dementia is to
undergo a neuropsychological evaluation. There are currently no dementia-specific
blood tests or other physiological diagnostics available. This is especially helpful if
the symptoms are modest or in the early stages.

5.5 Psychiatric evaluation

Sometimes there will also be a need for a psychiatric evaluation. This may be the
case when it is not clear if the changes in behavior are due to depression or another
psychiatric disturbance.

5.6 Physiological evaluation

PPA impacts not just the person who is afflicted with the disorder, but also those
who are close to them. Relationships, the ability to continue working, the ability
to execute numerous ordinary tasks, and the ability to convey even the most basic
requirements are all affected by the disease. Although there are several resources
accessible for people with memory loss, there are far fewer resources available for
people with PPA, their relatives, and friends. A consultation with a social profes-
sional who is experienced with PPA can address these concerns and offer advice on
how to deal with daily frustrations and problems.

5.7 Speech and language evaluation

Because the primary symptom of PPA is a reduction in language abilities, it’s

critical to figure out which aspects of language use are most affected, how badly
they are affected, and what may be done to improve communication. A speech-
language pathologist (SLP) examines various areas of language in-depth and might
provide recommendations to improve communication. Family members should be
included in treatment sessions to learn how to communicate more effectively.

5.8 Formal assessment

The following tests can be used to assess various functions in patients with PPA:

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Primary Progressive Aphasia (PPA)
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• Mini-Mental State Examination [11]

• The Graded Naming Test [12]

• Wechsler Memory Scale—Revised [13]

• Rey–Osterrieth Complex Figure [14]

• The Visual Object and Space Perception [15]

• The Pyramids and Palm Trees Test [16]

In the Indian context, the following tests prove to be valid:

• The Western Aphasia Battery would help to classify the extent and type of
Aphasia in these patients but supplementary tests need to be used.

• Neuropsychological tests also help in arriving at a diagnosis.

• Model-based tests e.g., Psycholinguistic Assessments of Language Processing

in Aphasia (PALPA) provide information on underlying ‘processing’ deficits.

5.9 Informal assessment

5.9.1 Assessment of cognitive and linguistic functions:

1. Semantic memory:

• Recognition of familiar faces.

• Draw or color objects from memory, classify photographs using semantic

criteria or match pictures of objects based on semantic similarity.

• Executive functions, verbal fluency, and behavior: Impaired verbal fluency is fre-
quently associated with executive function deficits such as abstraction (interpre-
tation of proverbs, cognitive estimates, explaining similarities and differences),
response inhibition, or motor sequencing (e.g. alternating hand movements).

• The ability to create a list of common animals (“category fluency”) or words

beginning with a nominated letter (“phonological” or “phonemic fluency”)
can be used to measure verbal fluency.

• Progressive Nonfluent Aphasia (PNFA) is indicated by decreased letter

fluency.

• The amount of words produced in one minute is used to determine the score.

2. Spontaneous speech:

• The patient can be asked to describe a scene in a photograph or drawing,

and the system will analyze an extended sample of the patient’s spontaneous
(propositional) speech.

• This is preferable to asking the patient to describe a typical day in their life.

9
Aphasia Compendium

• Observing the patient’s overall demeanor and demeanor during the clinical
interview.

• Frontal dementia is a type of dementia that affects people in a passive way.

• PNFA is a group of people who are frustrated by their issues and try to
compensate by using a lot of nonverbal gestures.

• Semantic dementia is characterized by a constant stream of circumlocu-

tory speech.

3. Naming:

• Naming should be assessed both in response to pictorial items (confron-

tational naming) and in response to verbal descriptions (e.g., “a large grey
animal with a trunk”).

• The performance of naming should be evaluated for both high and low-
frequency words (e.g., “shoe” versus “moat”).

• Examine if phonological (initial letter) or semantic (related item) cueing

improves performance.

• On confrontational naming tasks, visual perception deficits emerge as

“visual” errors.

• Circumlocutory reactions, semantic and phonological paraphasias can

be noted.

• Cueing with the first letter of the target word may help people with primary
word retrieval and phonological encoding impairments (rather than primary
verbal store faults).

• Personal names might be particularly challenging.

4. Speech comprehension:

• Word-finding and language output difficulties coexist.

• Can be measured at the level of individual words, which is dependent on

both intact perceptual systems and the verbal knowledge store (vocabulary),
and sentences, which is dependent on the ability to maintain verbal informa-
tion online and understand grammatical relationships between words.

5. Single-word comprehension:

• Can be proven at the bedside by testing phoneme pair discrimination (for

example, “pat—tap,” and “gat—cat”).

• Noun comprehension—asking the patient to point to items mentioned or

described by the examiner, to provide a definition or other information regard-
ing a target word (e.g., “What is a squirrel?”), or to choose between possible
synonyms for a target word (e.g., does “trench” mean “hedge” or “ditch”?).

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Primary Progressive Aphasia (PPA)
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• Asking the patient to classify items according to predetermined criteria (for

example, “Is a lion a mammal?”) can be used to dig further.

• When more fine-grained classifications are impossible, meaning is usually

kept for broad categories of nouns.

• Comprehension of verbs can also be tested by having the patient choose an

appropriate description of the examiner’s actions (“pushing” versus “pull-
ing”, “catching” versus “throwing,” etc.) or by asking them to do acts sug-
gested by the examiner.

• Gestures can also be utilized as an assessment technique.

6. Sentence comprehension:

• A short sequence of acts following unique grammatical norms (e.g., “put the
paper beneath the pen that is on the book,” “you take up the watch, then give
me the book”) could be used to examine this.

• The patient could also be asked to recognize an image based on a syntactical

sentence description (for example, “point to the boy being chased by the dog”).

• Grammar comprehension can be separated into two types: syntactical (word

relationships) and morphological (word changes in response to grammatical
context), each with its own neurological foundations.

• You can test this by having the patient look for grammatical errors in written
sentences.

• An early selective deficit in comprehending grammatical relations may be

found in PNFA whereas in SD, comprehension of syntactical constructions is
typically intact within the limitations of reduced vocabulary.

7. Speech repetition:

• Patients with impaired speech processing (such as word deafness) as well as

those with impaired speech output are at risk.

• It’s possible to assess it at the word and sentence level.

• Single-word repetition is frequently preserved in SD, although sentence

repetition is influenced by understanding level.

• When individual words are lost, phonemes may “migrate” between words
(for example, “the flag was colored bright red” may become “the blag was
fullered with a right breg”), implying that the utterance is encoded as an
extended sequence of phonemes (and thus susceptible to re-ordering),
rather than a series of meaningful units).

8. Reading, writing, and spelling:

• The patient should be asked to read a piece aloud that contains both irregular
and non-words (e.g. proper nouns).

11
Aphasia Compendium

• When reading a passage aloud, the sorts of errors produced convey informa-
tion about the underlying reading fault.

• Rather than a core language impairment, patients who demonstrate letter-

by-letter reading have a problem processing visual word forms: a syndrome
of higher-order visual perception (the input to the verbal lexicon).

• Patients with verbal knowledge store deficits (in particular, SD) frequently
“regularize” irregular words (e.g., reading “yacht” as “yatched”): this is a
“surface dyslexia” [15, 17], in which reading is based on superficial rules for
translating written words to speech sounds, rather than a learned vocabulary
that governs the pronunciation.

• Regularization issues are particularly obvious for lower-frequency phrases.

• The loss of spelling vocabulary is a defining feature of the SD condition.

Despite appropriate noun rendering, phonological dysgraphia (impaired
spelling by sound) produces difficulties writing grammatical function words
and non-words in PNFA [18].

• Written expression is often better retained with fewer errors than speech in
patients with primary speech production impairments (for example, early in
the course of PNFA).

9. Sentence generation and completion:

• Once other linguistic functions have been established, this level can be
judged with confidence.

• Tasks that demand the development of novel verbal thought, such as the pro-
duction of a statement including a target word (e.g. “boat”) or the comple-
tion of an unfinished phrase, can be used to test the deficiency.
10. Motor assessment:

• It is helpful to distinguish these from any linguistic loss, as well as to progress

the clinical diagnosis in general.

• The patient can be instructed to repeat a single syllable quickly (for example,
“pa, pa, pa...”) [19, 20]

• Dysarthric patients’ performance will be wrong if their rate or rhythm var-

ies, although AOS patients’ performance is normally normal.

• AOS has a higher rate of sequencing mistakes.

5.9.2 Assessments designed for differential diagnosis and tracking severity in PPA

• Nine neuropsychological evaluations were created or altered expressly for the

diagnosis or characterization of PPA, according to a recent systematic review.
Several of these can be used to distinguish between clinical variations, and two
of them were created expressly to assess PPA severity and progression.

• The Sydney Language Battery (SydBat) is a short battery of tests (image nam-
ing, word comprehension, semantic association, and repetition) that can be
used to distinguish between PPA subtypes (80% accuracy).
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• The Repeat and Point Test requires patients to repeat 10 multisyllabic words
and point to the goal among semantic and phonological distractors in order to
distinguish between semantic and nonfluent versions (100% accuracy).

• The Progressive Aphasia Severity Scale (PASS) is a tool used to assess symptoms
and follow progression in people with PPA. On a three-point scale, clinicians rate
the severity of speech and language deficits (articulation, fluency, syntax/gram-
mar, word retrieval/expression, repetition, auditory comprehension phrases/
sentences, single-word comprehension, reading, writing, and functional com-
munication) as well as pragmatic aspects of communication. After an informant
fills out a questionnaire and a structured interview with both the patient and the
informant, the SLP completes the scale. Table 1 describes PASS.

• The Progressive Aphasia Language Scale (PALS) includes clinician ratings

of speech-language features (motor speech and grammatical features in
spontaneous speech, naming, single word repetition and comprehension, and
sentence repetition and comprehension), but it is based on signs observed dur-
ing a prescribed set of speech-language tasks rather than symptoms reported
via interview or questionnaire. PPA participants were subtyped by variation
using an algorithm based on four essential aspects from this evaluation (motor
speech impairment, grammar, single-word comprehension, and sentence
repetition) (relative to expert clinical diagnosis).

• Clinical Dementia Rating (CDR), a dementia severity rating scale based on

a semi-structured interview and clinical judgment, now includes a language
domain, which improves sensitivity (relative to the original CDR) for detecting
and tracking symptoms and functional impairments in language-prominent
dementias like PPA.

5.9.3 Quality of life assessment

1. Communication Activities of Daily Living—3rd edition (CADL-3)

2. Assessment for Living with Aphasia (ALA)

3. Quality of Communication Life Scale (ASHA QCL)

4. American Speech-Language-Hearing Association Functional Assessment of

Communication Skills for Adults (ASHA FACS)

5.10 Probable intervention strategies

5.10.1 Care, support, and treatment team of PPA

1. Neurologist (specialists in brain disorders who make the diagnosis and moni-
tors its symptoms)

2. Neuropsychologist (evaluate cognitive abilities through specialized paper and

pencil tests).

3. Psychiatrist (assist with behavioral and mood symptoms).

4. Social worker (help families navigate the difficult decisions related to their
diagnosis).

13
14

Aphasia Compendium
PASS Domain 0 = Normal 0.5 = Questionable or very mild 1 = Mild impairment 2 = Moderate 3 = Sever impairment
impairment impairment

FLUENCY-Degree to which speech Normal flow of Speech contains occasional blank Speech is in short phrases Dysfluencies in most Severely dysfluent
flows easily, or it is interrupted speech pauses or use fillers; reduced WPM interrupted by pauses or utterances; phrase length speech, phrase length
by hesitations, pauses, fillers; and/or phrase length groping for words but it is rarely exceeds three rarely exceeds one
reduced fluency is accompanied by occasional words. word. May not speak.
decreased phrase length and words
per minute.
SYNTAX AND GRAMMAR: Use No difficulty in the Occasional agrammatism or Frequent agrammatism; Utterances contain mostly Single word utterances
of word forms, function words, use of grammar and pragmatism (i.e odd sentence sentence structures are content words with rare or no speech or
and word order when forming syntax structure such as “I my car drive simple; frequent misuse or use of syntactic words, writing.
phrases and sentences in most used in your house’) may complain it omission of grammatical grouping; functional
modality (speech or writing) is effortful to combine words into words or sentences words or morphological
phrases or sentences markers

SINGLE WORD No difficulty in Occasional difficulty in Display lack of word Understands some high Minimal
COMPREHENSION: Ability to an understanding understanding, low-frequency comprehension frequency and / familiar comprehension
understand spoken or written a a single word in words (eg: cork); may question the several times in a brief words. Questions the of single word
single word. conversation or meaning of words (what?…) conversation but able meaning of many words comprehension.
text. to carry out an easy in conversation.
reasonable meaningful
conversation.

Table 1.
Three representative domains of the progressive aphasia severity scale (PASS) [21].
Primary Progressive Aphasia (PPA)
DOI: http://dx.doi.org/10.5772/intechopen.104291

5. Speech and language pathologist (provide strategies to maximize communica-

tion abilities).

6. Physical and occupational therapists (help optimize movement and activities

of daily living).

The goal is effective communication, not perfection as PPA is a progressive disorder.

Early treatment, a specific plan, partner training, and long-term planning are all critical
aspects of proper speech therapy treatment. Speech-language pathologists are uniquely
competent to assess the present impact on communication, establish patient priorities,
and build a treatment plan based on compensatory methods with a home program to
preserve abilities as long as possible. We cannot stop PPA from progressing, but speech-
language pathologists can help clients and families adjust their lives, compensate for
their impairments, and keep the highest function and quality of life possible.

5.10.2 Tips for treating primary progressive aphasia

5.10.2.1 Practice priority vocabulary

• Practice only what is important to your client.

• Carryover does not occur.

• Create opportunities for your client to practice important words and phrases.

• Use of strategies like “cue cards”.

5.10.2.2 Teach scripts for primary progressive aphasia

• If the client’s main progressive aphasia is still in its early stages, have the family and
the client pay attention to everyday routines and words. Request that a family mem-
ber jots down your ideas. You’ll now have a list of what’s significant in the client’s
daily life, and you can use these phrases as scripts to practice as the PPA progresses.

• Speaking scripts can be used for a variety of situations, including:

A typical discussions.

• Making a restaurant reservation.

• Calls that are made on a regular basis.

• Written scripts/models can be used for the following:

• Email

• Texts

• Check-writing

• Guidelines for writing effective scripts:

Depending on the severity of the PPA, vary the number and complexity of the
scripts—the fewer and simpler the scripts, the more severe the PPA. Concentrate on

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Aphasia Compendium

clarity and reduce frustration. When in doubt, keep the script simple—you are not
trying to teach something new; instead, you are bolstering and compensating. Set
priorities based on the client’s priorities.

5.10.2.3 Train compensatory strategies for primary progressive aphasia

Identifying and applying communication strengths can help you decide successful
compensating measures and increase confidence, just as it can with aphasia or demen-
tia. Modify hobbies and activities so that the person can compensate for linguistic
difficulties. Labeling items in the environment is sometimes all that is required.
Clients should practice employing techniques, alternative communication, and
scripts with the SLP, their family, and close friends in comfortable contexts. It will
be easier to use them in public as a result of this.

5.10.2.4 Create a PPA business card

Individualized information cards may be beneficial to clients who are still

self-sufficient in the community. This card can be used to inform people that the
individual has aphasia, as well as what tactics can help and who to contact in an
emergency. This can come in handy if the individual is having trouble at the grocery
store or library, or if they have a run-in with the cops. Cards can also be used as a
script for ordering a cup of coffee or checking in for tea time.

• To make contacts with new communication partners easier, hand out a cus-
tomized card that describes what PPA is.

• Make sure the card is in a familiar position for the client, such as their pocketbook
or purse, adjacent to their driver’s license or photo ID. The card should include any
relevant information that the person feels comfortable revealing, such as:

○ Name

○ Explanation of the communication difficulty or diagnosis (consider includ-

ing that they are not drunk or on drugs, as is often assumed)

○ Quick communication tip strategy that can help (e.g. “use short phrases”,
“speak slowly”, “allow extra time to understand”, “be patient”).

○ Emergency contact person’s name and phone number.

5.10.2.5 Train nonverbal communication

Nonverbal skills become even more critical as PPA advances and communication
abilities (especially verbal skills) deteriorate. Nonverbal communication is fre-
quently easier to comprehend and produce. Teach care partners how to use gestures,
eye contact, and body language and how to pay attention to them. Allow for more
thinking time, have them write keywords, use visuals (photos, drawings, objects),
shorten their phrases, and so on.

5.10.2.6 Voice banking and AAC

When words fail, care partners can use AAC apps to help communicate their
message. Early on in therapy, you can try out different AAC apps to discover which

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Primary Progressive Aphasia (PPA)
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one or style the client enjoys, and then start customizing and training them before
they are needed.

5.10.2.7 Plan for the future

From the first day of treatment, the focus should be on a home program and com-
pensating methods. Set up routines and experiment with multimodal and alternative
communication as soon as possible, even before the client requires it. It will be easier
to understand and acquire new strategies and routines early in the disease process,
while more language and cognitive skills are still intact. Clients and families have
more time to become comfortable with communication options and select what they
like and do not like with earlier involvement. If at all possible, follow up with the
client after discharge or check in with them every few months. PPA’s home program
and communication tactics will need to evolve as his or her life progresses.

5.10.3 Phased treatment

Clinicians need to consider important differences when managing speech and

language impairments in the context of progressive disease [22].
In aphasia caused by stroke, clients show a static or gradually improving commu-
nication profile. But clients with PPA will eventually become mute or speak largely in
jargon—using words that have some meaning to them but not to the listener.
Ultimately, with the disease’s progression, patients will show increasing cogni-
tive, behavioral, and motor deterioration.
Given this trajectory, PPA treatment should address a client’s current profile of
spared and impaired abilities, while also addressing the client’s inevitable decline in
communication and, in later stages, other domains.
Because of the evolving nature of PPA, a phased treatment approach, with
recurring assessment and repeated treatment “doses” tailored to the client’s current
communication needs and challenges is recommended.

5.10.3.1 Phase 1—restitutive treatment in mild PPA

• A crucial part of the SLP’s role during the early stages of PPA is to thoroughly
evaluate and document the patient’s communication. This initial evaluation
serves as the baseline for the progression of difficulties and helps to identify
targets for treatment.

• Clinician often focuses on rebuilding impaired communicative abilities via

recitative treatments.

• Treatment during mild stages is critical for helping clients relearn lost skills.

5.10.3.2 Phase 2—toward aided communication in moderate PPA

• Treatment shifts toward multimodal communication and the use of augmenta-

tive and alternative communication (AAC).

• Select functional phrases and words to include.

• Strategies to maximize conversational success.

• Also provides counseling regarding PPA.

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Aphasia Compendium

5.10.3.3 Phase 3—environmental support and communication partner training in

moderate-severe to severe PPA

• Treatment focuses on communication partner training and environmental

modifications.

• This phased treatment approach allows clinicians to draw from their experi-
ence treating aphasia while taking into account the inevitable decline associ-
ated with PPA.

5.10.4 Promoting self-cueing strategies

SLPs should focus on self-cueing strategies for lexical retrieval in the mild
phases of PPA, which take advantage of the individual’s residual skills [4]. During
the examination, semantic, orthographic, and phonological self-cueing should
all be tested in order to discover which method is most effective in prompting
retrieval.
Family members can be taught to use cueing tactics like “Tell me about it”
or “Can you think of what letter it starts with?” to help initiate self-cueing. The
ultimate goal is for the person to be able to self-cue at the conversational level on
their own. The SLP must be aware, however, when the family member is working
too hard or seeing all interactions in therapy sessions.

5.10.5 Script training

Researchers have shown that conversational scripts can help people with
stroke-induced aphasia improving their functional communication [23, 24].
The advantage of script training is the automaticity with which patients acquire
skills by recalling memories of context-bound, skilled performance. When the
same stimuli from the practice environment are available in a functional context,
repeated practice of a specific task might improve automatic retrieval. The key to
script training is to make sure that each person practices consistently and thor-
oughly. The SLP should consider the individual’s requirements and interests, the
style of the script (dialog or monolog), the number and duration of conversational
turns, as well as the grammatical complexity and vocabulary choices when writing
a script [25]. Families may purchase a script computer program such as Aphasia
Scripts.
Examples of possible script topics include:

• Talking to a family member or friend (e.g. by phone).

• Scheduling an appointment; asking for directions.

• Stating personal information or explaining PPA.

• Giving a lecture or speech

• Saying prayers.

SLPs can frame a written goals based on the accuracy and rate of production
(e.g., timing how long it takes to read the script while counting the number of
errors). If the person is not motivated to practice the script outside of therapy ses-
sions, he or she may not be a good candidate for script training.

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5.10.6 Facilitating receptive language

Facilitating the use of Receptive Language Family training on ways to aid

comprehension is critical for individuals with receptive language difficul-
ties. Automatically speaking communication partners can make a difference in
comprehension.

• Talk softly, face the person, and give them your undivided attention.

• Augmenting speech with motions.

• Only giving one command at a time.

• Employing grammatical structures and terminology that are easy to under-

stand, or.

• Removing distractions from crucial conversations (e.g., turning off the

TV/radio).

5.10.7 Low-tech AAC

The SLP’s job is to determine the book’s proper format, font size, and
word/page length, and then instruct the individual and family members to keep
adding pages.

• When creating a communication book, SLPs should think about a few things.

• SLPs can laminate small photographs so that the patient can attach them to a
key ring and carry them in his or her pocket.

• SLPs should utilize plastic page protectors in a binder to create a more thor-
ough book.

• Using images from the Internet or the person’s own photographs, SLPs can
personalize pages.

• SLPs can break the book into sections using tabs.

• SLPs should ask family members to record words and ideas that have led to
communication breakdowns. They should add these to the book.

Because some individuals with PPA may not independently use the book, train-
ing the family members on appropriate cueing strategies to initiate book use and
facilitate navigation also should be targeted during treatment sessions.

5.10.8 High-tech AAC devices

Despite the lack of evidence on AAC intervention options for people with PPA,
SLPs have effectively used high-tech AAC devices to help people with severe aphasia
caused by stroke communicate [26]. Because people with PPA’s other cognitive
abilities deteriorate with time, it’s crucial to keep in mind that a person’s capacity to
learn to utilize high-tech equipment may be limited and eventually hampered. The
SLP’s job is to figure out which devices if any, will best enable the client to convey

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Aphasia Compendium

his or her needs and allow for social communication. The SLP should ask a series of
questions to determine if a high-tech AAC is a good fit for someone with PPA.

• Is the person enthusiastic about using a high-tech ACC to communicate?

Otherwise, it is unlikely to succeed.

• Can the person operate other types of technology independently, such as a

computer, microwave, TV remote, or phone? If not, this could suggest a loss of
fine motor skills or an inability to sequence, indicating that you are not a good
candidate for an ACC.

• Has the family member(s) been involved in the planning, training, and
achievement of the objectives? If not, invite family members to several sessions
to ensure that the plan is implemented successfully.

• Has the person and their family been taught how to utilize the device in
everyday situations? Have the trials attained their objectives? If not, consider
additional training and a long trial.

• In conclusion, people with PPA have a variety of choices for increasing com-
munication. Patients and their families are advised to follow the following
guidelines:

• In the early stages of the disease, a comprehensive evaluation of speech,

language, and other cognitive abilities should be sought.

• Follow-up evaluations are required on a regular basis to evaluate patterns of

language deterioration.

• Treatment should begin as soon as possible, focusing on the areas of speech

and language that are affected.

• As language abilities deteriorate, the treatment focus should be modified.

• Early on, augmentative communication strategies should be implemented.

Patients who are given these tactics later in their language loss may find it dif-
ficult to learn how to use them.

• Involving family members or other people with whom the patient talks are
critical not just for increasing awareness of effective communication tactics,
but also for practicing them with the patient.

• As patients’ ability to communicate fades, they will rely increasingly on aug-

mentative communication tools. With individual patients, some techniques may
be more effective than others, and some patients may utilize more than one.

• Treatment will not stop the aphasia from progressing, but it will considerably
improve communication abilities.

6. General suggestions for communicating with individuals with PPA

*Communication is usually most successful in a peaceful, calm, relaxed

situation. Avoid talks when the television is on, and avoid circumstances where

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numerous individuals are talking at the same time. Avoiding social circumstances is
not necessary; rather, such activities should be encouraged. Maintain usual activi-
ties as much as feasible. When people are not tired, communication is usually the
most effective.

Communication partner Individual with PPA

Be patient—give your partner time to Remember, your partner is trying, so be patient.

communicate.
The main goal is the communication The main goal is to communicate—not speech or perfection.
of information—not speech or Use whatever methods you can to get your message across—
perfection. Once information has been speech, writing, drawing, gesture and/or communication
communicated, move on. notebook. Ask for help when needed.
Be careful not to speak too quickly. Take your time.

Ask questions one at a time, pausing Try to give as much relevant information as possible. Focus
between each. Ask yes/no questions, or on expressing the “main points.”
multiple choice questions.
Repeat what you understand to clarify. Ask your partner to repeat if you do not understand what
was said. Use facial expressions to indicate when you
misunderstand.

Treat the individual as an adult. Do not

talk down. Intelligence is generally intact;
communication is the problem.

Author details

Yashaswini Channabasave Gowda1 and Hema Nagaraj2*

1 Junior Research Fellow, Department of Speech-Language Sciences, All India

Institute of Speech and Hearing, Mysore, India

2 Assistant Professor, Department of Speech-Language Sciences, All India Institute

of Speech and Hearing, Mysore, India

*Address all correspondence to: hema_chari2@yahoo.com

© 2022 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms
of the Creative Commons Attribution License (http://creativecommons.org/licenses/
by/3.0), which permits unrestricted use, distribution, and reproduction in any medium,
provided the original work is properly cited.

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Aphasia Compendium

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