Primary progressive aphasia (PPA) is a rare neurological syndrome that causes a gradual decline in language abilities, primarily affecting individuals over age 50. There are three main variants of PPA - non-fluent/agrammatic PPA, semantic dementia, and logopenic PPA. Non-fluent PPA is characterized by effortful and halting speech with agrammatism, while semantic dementia involves loss of word comprehension and knowledge over time. Logopenic PPA causes word-finding difficulties and reduced verbal output with relatively spared grammar. Neuroimaging often shows left-sided temporal and frontal lobe atrophy depending on the variant. A diagnosis of PPA requires language impairment to be the primary deficit, with progressive decline and

1. Primary progressiveaphasia(PPA)
PRESENTER
Renju.P.R
2nd MASLP

2.  Primary progressive aphasia (PPA) is a gradually worsening disorder of
speech with an insidious onset.
Marsel Mesulam 1982.
 Initially he referred it to as “ slowly progressive aphasia ” and later it
was termed as primary progressive aphasia.
 Primary progressive aphasia is a rare neurological syndrome that
impairs language capabilities, primarily in people over the age of 50.

3. Causes of PPA
 The most common type of brain degeneration found after
brain autopsy in individuals with PPA (60% of cases) is
Nonspecificdegeneration.
 Nonspecific degeneration (NSD) – 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.

4. Neuroanatomical Correlates of PPA
 Most of the MRI studies (56%) have reported anomalies in
the left hemisphere only, other studies (43%) reported
bilateral anomalies.
 The most commonly reported sites of anomaly among the
MRI scans which revealed evidence of exclusively left
hemisphere degeneration were the sylvian fissure and the
temporal lobe.
 The sylvian fissure and the temporal lobe are reported to be
the common sites of degeneration.

5. Data from PET and SPECT scan studies of PPA
patients reported anomalies in the blood flow.
PET and SPECT scans reveal a lower rate of bilateral
involvement. The temporal lobe is reported to be the
site of anomaly in 90% of the patients with PPA.

6. Signs and symptoms
Symptoms can begin as early as the 40's, but can appear any time
afterwards. Initially, the symptoms of PPA are confined exclusively to
the area of language functions. The types of language difficulties that
occur differ between patients, but generally involve things such as the
following:
 Increased difficulty thinking of words which results in, Substituting
the wrong word , Mistakes in pronunciation , Talking around the
word .
 Problems reading or writing , Difficulty following written directions,
reading signs , No longer able to write cheques, letters

7.  Reduced ability to understand speech , Trouble following
conversations, especially in larger groups , Asking for repetition often,
misunderstanding things that are said (hearing normal)
 Decreased use of language, Initially fluent speech but with word
finding difficulties. Speech may become garbled or empty of any real
information and difficult to comprehend early in the course of the
illness , Eventually may be unable to use speech to communicate,
becoming mute Agrammatism
 Problems in arithmetic and calculations, May lose ability to perform
even simple mathematical operations , No longer able to handle
money

8.  The preservation of the cognitive function is the one which separates PPA
from dementia.
 In general early symptoms of primary progressive aphasia involve
difficulty finding the right words during conversations or in writing.
 Language problems gradually worsen with time.
 People with this disorder often become mute and may eventually lose the
ability to understand written or spoken language. This generally happens
within 10 years of diagnosis. As the disease progresses, other mental skills
may become impaired. Many people with this disorder eventually will
need help with all their day-to-day care.

9. Types of PPA
 In the classical Mesulam criteria for PPA there are 2 variants:
-Non-fluent type ( progressive non-fluent aphasia or
PNFA)
-Fluent aphasia (semantic dementia or SD).

10.  Recently a third variant of PPA is also suggested, called
logopaenic PPA (Gorno - Tempini et al, 2004), characterized
clinically by spared articulation, reduced phrase length
despite grammatical output and impaired syntactic
comprehension due to auditory working memory deficits.
 Lesion: Atrophy in the posterior superior temporal and
inferior parietal regions of the dominant hemisphere.

11. • Snowden et al (1992), reporting on their extensive clinical
experience with this disorder, divided progressive aphasia into
three varieties –
fluent,
non fluent and
mixed.

12. Progressive Non-fluent Aphasia
 Resembles descriptions of Broca’s aphasia. It is defined as an insidious
and progressive language abnormality with reduced phrase length,
agrammatism or effortful and halting speech.
 Studies have suggested involvement of the left inferior frontal region
(Rosen et al., 2002b).
 Similar to stroke patients with nonfluent aphasia have atrophy (Gorno-
Tempini et al, 2004) and reduced metabolic activity in the anterior insula.

13. Features of PNFA: presenting characteristics, neuropsychological profile
and language characteristics
Perception Preserved
Episodic & Semantic memory preserved
Planning & Judgment preserved
Comprehension preserved
Verbal Expression impaired agrammatic, stuttering
speech
Paraphasias Present, phonemic

14. Repetition impaired
Word retrieval impaired
Reading paralexias
Writing telegrammatic
Abstraction Impaired secondary to language
disorder
Calculation preserved early
Spatial ability & navigation skills preserved early
Motor abilities preserved early
Social skills preserved
Behaviour Frustration and irritability

15. SemanticDementia
SD resembles Wernicke’s aphasia in that it is associated with normal
fluency, comprehension deficits, and left temporal lobe lesions
(McClelland et al, 2001).
 As the disease progresses, patients often suffer a breakdown of
semantic knowledge that usually manifests as loss of single word
comprehension.
 Patients with SD have a predominance of left temporal lobe atrophy
on neuroimaging (Garrand & Hodges, 2000).

16.  Features of Semantic Dementia: presenting characteristics,
neuropsychologicalprofileand language characteristics
Perception Impairedobjectand face recognition
Episodic memory preserved
Semantic memory Impaired
Comprehension Loss of word meaning, good
understanding of syntax
Spontaneous speech impaired Fluent, effortless, semantic
Paraphasias and preserved syntax
Paraphasias Present, phonemic
Repetition Relatively preserved

17. Naming Profoundimpairment, semantic errors,
no benefit with cueing, letter fluency
better than category fluency
Reading Fluent reading, impaired comprehension
for written text
Writing Fluent
Abstraction Compromised
Calculation preserved till late
Spatial abilities and constructional skills preserved
Behaviour Irritability, apathy, disinhibition &
altered eating behaviour

18. LOGOPENICPPA
• “logopenic” (from Greek, meaning “lack of words”)
• overall paucity of verbal output (An insufficient quantity
or number/ shortage)
• relative sparing of grammar, phonology, and motor
speech.
• word-finding difficulties
• Decreased verbal output,

19. • speak slowly, with syntactically simple but largely correct sentences.
• phonological deficits - temporo-parietal junction damage
• Impaired naming and sentence repetition
• difficulty with comprehension and repetition of sentences-
phonological working memory impairment.
• frequent ‘‘RETRACINGS’’ - patient stops what they are saying, and
goes back to phrase the sentence differently- verbal working memory
impairment
• Reading errors- pseudo word reading
• Spelling impairment

20. Behavioral changes
• irritability,
• anxiety,
• Agitation (extreme emotional disturbance, not calm)

21. NONFLUENTPPA FLUENTPPA
• Evidence of a left frontal involvement on
neurological examination.
• Patients with nonfluent PPA develop more
dysarthria & impaired letter fluency than
category fluency consistent with a left
frontal nonfluent syndrome.
• A high proportion of phonological errors
than fluent patients.
• Evidence of left temporal lobe abnormalities
on neuroimaging.
• Patients with fluent PPA performed worse
with confrontation naming of objects than
nonfluent PPA patients, which has been
linked to lesions of the left temporal lobe.
• A higher proportion of semantic anomia &
semantic paraphasic errors, resulting from
the disruption of lexical-semantic networks
in the left temporal lobe.

22. • Phonemic paraphasic errors (exclusively)
indicating involvement of the superior
temporal gyrus (Hickok et al, 2004).
Impaired in repetition and phrase
comprehension.
• Nonfluent PPA differs from classic Broca’s
aphasia. Agrammatism is a common
feature of Broca’s aphasia which is not
markedly evident in PPA.
• Impaired repetition and disturbed single
word comprehension due to disturbance in
acoustic-phonetic processing, auditory
lexical access, or semantic access (Hodges
and Miller, 2001)
• Both Wernicke’s aphasia and PPA are
associated with reduced naming,
comprehension & repetition with spared
fluency.

23. ASSESSMENT

24. Three core criteria to diagnose as PPA
(Mesulam, 2001).
1. patient should have an aphasic disorder of recent onset manifested by
distortions of word usage or comprehension that cannot be attributed to
more elementary motor or perceptual deficits.
2. language impairment should constitute the most salient
neurobehavioural deficit and the chief impediment to the pursuit of
customary daily living activities during the initial stages of the illness
3. underlying disease should be neurodegenerative and, therefore,
progressive

25. Steps involved:-
1. History:
 First, a careful history is taken to establish that a condition of
dementia, as defined above, exists.
 This often requires that family members or friends be
questioned about the patient’s behavior because sometimes the
patient is unaware of the symptoms (as in the case of memory
loss or personality changes) or may be unable to describe them
due to aphasia.

26. 2. Neurological Examination :
 A neurological examination is done to determine if there are
signs of dementia on a simple screening of mental functions
(the mental status examination) and also if there are signs of
motor or sensory symptoms that indicate other types of
neurological disorders might be causing the dementia.
 The neurologist will also order tests, such as a magnetic
resonance imaging (MRI) scan, to make sure that the
symptoms are not due to factors such as stroke or tumor.

27. 3. Neuropsychological Examination :
 Provides a more detailed evaluation of mental functioning.
 This is especially important in the very early stages of illness when a
routine screening evaluation may not detect the problems the patient
is experiencing.
 This requires several hours and consists of paper and pencil or
computer administered tests of mental abilities, including attention
and concentration, language learning and memory, visual perception,
reasoning and mood.

28.  The results can indicate if there are abnormalities of thinking and
behavior and also their degree-mild moderate or severe.
 It is often difficult to demonstrate that individual with PPA have intact
memory since we usually test memory by telling a person some
information and then asking them to repeat it later on.
 In an individual with PPA, it may be impossible to repeat back the
information because of the aphasia. Therefore, it is important that
testing is done properly to make sure that it is not a true loss of
memory.
 Currently, there are no blood tests or other physiological tests that are
specific for dementia.
 This is especially important when symptoms are in the very early
stages or mild.

29. 4. Speech andLanguage Evaluation :
 Since a decline in language abilities is the primary symptom of PPA, it
is important to determine which components of language use are most
affected, how severely affected they are, and what can be done to
improve communication.
 A SLP evaluates different aspects of language in detail and can make
recommendations to improve communication.
 Family members should be included in the treatment sessions to
educate them how to facilitate communication.

30. INFORMALASSESSMENT
1. Semantic memory :
 Recognition of familiar faces
 Draw or colour objects from memory, classifypictures based
on semantic criteria or match pictures of objects according to
semanticrelatedness
 Spontaneousspeech :
 Systematic analysis of an extended sample of the patient's
spontaneous(propositional) speech.
 The patient can be asked to describe a scene ina photograph
or drawing. This is preferable to asking the patient to recount
an event in theirdaily routine.

31.  Naming :
 Naming should be tested directly both in response to pictured items
(confrontational naming) and from verbal description (e.g. ‘a large grey
animal with a trunk’).
 Naming performance should be assessed for words of both high and low
frequency(e.g. ‘shoe’ versus ‘moat’)
 Sentence comprehension:
 Can be assessed by asking the patient to perform a short sequenceof actions
according to different syntactic rules (e.g. ‘putthe paper underneath the pen
that is on the book’, ‘youpick up the watch and then give me the book’).
 Alternatively, the patient can be asked to identify a picture based on a
syntactical sentence description (e.g. ‘point to the boy being chasedby the
dog’).

32. 5. Reading, writing and spelling :
The patient should be asked to read aloud a passage that
includes both irregular words and non-words (e.g. proper
nouns).
Patients who exhibit letter-by-letter reading have a defectin
processing visual word forms: a syndrome of higher order
visual perception (the input to the verbal lexicon) rather
thana primary language deficit.

33. FORMAL ASSESSMENT
The following tests can be used to assess various functions in
patients with PPA:
 Mini-Mental State Examination (Folstein et al., 1975)
 The Graded Naming Test(McKenna and Warrington, 1983)
 Wechsler Memory Scale—Revised (Wechsler,1987)
 Rey–OsterriethComplex Figure (Rey, 1964)
 The Visual Object and Space Perception(VOSP, Warrington
and James, 1986)
 The Pyramids and Palm Trees Test (PPT, Howard and
Patterson, 1992

34. 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., PALPA (Psycholinguistic Assessments
of Language Processing in Aphasia) provide information on
underlying ‘processing’ deficits.

35. TREATMENT

36.  The primary goal of treatment for language impairments in
individuals with PPA is to improve the ability to
communicate. Because the type of language problem
experienced by patients with PPA may vary, the focus of
treatment for improving communication ability will also vary.
 At present there is no cure for the degenerative diseases that
causes PPA. Medical treatments are generally in the realm of
managing behavioral symptoms such as depression, anxiety,
or agitation, which may occur later in the course of the illness.

37. Fromstudies on aphasia therapy :
 Treatment targeting the ‘lexical route’ , Repeated exposure to and practice
of target stimuli strengthens the orthographic representations and also aids
in the restoration of word-specific information
 Strengthening of a general procedure takes place by which entries in the
visual-word recognition system are checked.
 Picture cues/imagery: strengthens links between semantic system; ease of
retrieval and depth of processing.
 Treatment targeting the ‘phonological route’ . DePartz (1986); Cardell &
Chenerey (1999); Kiran (2005) proposed phonological retraining and
segmentation skills to strengthen the phonological route, which could be
time consuming and rigorous in nature.

38. Spell-study-spelltraining procedure (Rapp& Kane, 2002)
 Their subject was a right handed, 63 year old, college educated,
professional artist diagnosed with fluent PPA.
 The patient exhibited significant deterioration in spoken and written
language production as well as in her artistic ability and aspects of
praxis. With regard to her dysgraphia, the patient suffered from
deficits at the level of the orthographic lexicon and the graphemic
buffer, exhibiting significant effects of lexical frequency and word
length.
 The study was conducted in four phases: pre-training, training, post-
training and follow-up. biweekly sessions for 7 weeks distributed over 2
months. follow-up evaluations after 5 months

39.  Stimuli consisted of four sets of words which were matched for
frequency, letter length, and concreteness.
 For trained words (training phase), at every session, the patient was
asked to spell each word to dictation, study the word, and then
attempt to spell it again if it had been spelled incorrectly. This
procedure was repeated until the word was correctly spelled.
 The authors found that there were both short-term and long-term
benefits of behavioural intervention.
 In the short-term, accuracy on treated words increased relative to
baseline and relative to all other word types. In the longer term,
treated words were protected from significant deterioration. These
findings are the first to provide empirical support for the potential of
behavioural therapy as a means of prolonging language abilities in
primary progressive aphasia.

40. CART (Copy & Recall Technique, modified fromBeeson, 1999)
 The stimuli here is a set of irregular words (treated vs.
untreated words)
 Each word is presented on index cards
 The patient is asked to write the previously presented word
from memory (immediate) , Similarly, words are practiced
within the session and at home (2 weeks at a time minimally)
Repeated baselines of treated and untreated words are
obtained.

41. Copy and delayed recall (modified fromRapp & Kane, 2002)
 This strategy is similar to CART except for introduction of a
30-45 sec. delay in the retrieval of the word
 In the intervening period, the clients are asked to actively
rehearse the word. This strategy aims at strengthening the
GOB through articulatory rehearsal.
 After rehearsing the word, the target word should be recalled.
 Repeated assessments of treated + untreated words are
obtained to assess generalization of treated to untreated
words.

42. Drug Trials
 No current rationale for pharmacological treatment of PPA.
 The drugs approved for treating amnesic dementia related to
Alzheimer’s disease (AD) are cholinergic agents, targeting the
cholinergic loss that has been shown in Alzheimer’s disease.
 The likelihood of the pathology in PPA being similar to
Alzheimer’s disease is very low.
 Therefore there is no scientific evidence to support the
administration of AD drugs to PPA patients.
 One rationale for treatment of PPA may come from studies of the
treatment of aphasia resulting from stroke.

43. Approaches to treatment
2 basic approaches:
- Focus treatment directly on the impaired language skills.
- Provide AAC strategies or devices.
 Recommend that both treatment approaches be used with PPA
patients.
 Beginning in early stages of the disease, treatment should be provided
to enhance verbal language skills. For example, treatments focused on
word retrieval skills may be helpful.
 Treatment focused on the use of AAC strategies also should be
provided, even in the early stages of PPA.

44. There are strategies that either enhance verbal
communication or replace it. Suggested that the
patient (and family members) be trained in AAC
strategies such as
use of communication notebook
use of gestures
use of drawing

45. • Results of the speech and language evaluation will
determine which strategy (or strategies) is the best and
some practice in using them will be provided during
the evaluation. However, follow up treatment with a
speech language pathologist is important in order to
further develop to strategy and provide in using it.

46. Primary progressive aphasia: clinicopathological correlations
Murray Grossman
Nature Reivews Neurology 6, 88-97 (February 2010)
Primary progressive aphasia (PPA) is a disorder of declining
language that is a frequent presentation of neurodegenerative
diseases such as frontotemporal lobar degeneration. Three
variants of PPA are recognized: progressive nonfluent
aphasia, semantic dementia, and logopenic progressive
aphasia. In an era of etiology-specific treatments for
neurodegenerative conditions, determining the
histopathological basis of PPA is crucial

47. • Clinicopathological correlations in PPA emphasize the contributory role of
dementia with Pick bodies and other tauopathies, TDP-43 proteinopathies,
and Alzheimer disease.
• These data suggest an association between a specific PPA variant and an
underlying pathology, although many cases of PPA are associated with an
unexpected pathology.
• Neuroimaging and biofluid biomarkers are now emerging as important
adjuncts to clinical diagnosis.
• There is great hope that the addition of biomarker assessments to careful
clinical examination will enable accurate diagnosis of the pathology
associated with PPA during a patient's life, and that such findings will
serve as the basis for clinical trials in this spectrum of disease

48. Role of AAC Intervention for Adults in Middle Stage PPA.
King, J. (2007, September)
Paper Presented at the 2007 Clinical AAC Research Conference, Lexington,
KY.
• Primary Progressive Aphasia (PPA) is an atypical form of
dementia characterized by core memory functions remaining
intact even as deficits in language appear and worsen
(Mesulam, 2003).
• Mesulam (2003) and Kertesz and colleagues (2003) have
described language changes associated with PPA but limited
evidence is available to guide clinical intervention practice as
the disorder progresses over time.

49. • The purpose of this study was to identify how
often and what type of intervention was required
to maximize outcomes of treatment for two
adults with a medical diagnosis of PPA or
probable PPA.
• Results summarize outcomes of staging
communication intervention as well as the AAC
intervention approach used with each person.

50. Foreign accent syndrome as the initial sign of primary progressive aphasia
S Luzzi, G Viticchi, M Piccirilli, K Fabi, M Pesallaccia, M Bartolini, L Provinciali,
J S Snowden
J Neurol Neurosurg Psychiatry 2008 : 79-81
• Foreign accent syndrome (FAS) is a rare speech disorder
characterised by the emergence of a new accent, perceived by
listeners as foreign. FAS has usually been described following
focal brain insults, such as stroke.
• They describe the unusual case of a woman presenting with
FAS as the earliest symptom of progressive degenerative
brain disease.

51. • At presentation, she showed no language or other cognitive impairment,
and functional and structural brain imagings were normal.
• Follow-up 1 year later revealed the emergence of mild expressive language
problems.
• Repeat functional neuroimaging showed mild hypoperfusion of the
perisylvian speech area of the left hemisphere, and structural imaging
showed mild left perisylvian atrophy.
• They interpret the case as an unusual presentation of primary progressive
non-fluent aphasia. The case provides further evidence of the variable and
circumscribed nature of the clinical presentation of focal cerebral
degeneration.