Primary Progressive Aphasia

Atrophy Patterns

• Atrophy patterns in PPA start relatively focal before becoming more diffuse over time

• not limited to the vasculature as with stroke

PPA Classification Basis

• Speech production features (grammar, motor speech, sound errors, word- finding pauses)

• Repetition

• Single word/syntax comprehension

• Reading/spelling

• Naming

• Semantic knowledge

PPA Diagnosis

● A type of dementia where language processes affected first

● At least two years of isolated language impairment

● Memory problems, visuospatial deficits, and behavioral/personality change must not be present during this time

● Activities of daily living are limited only by the language problem

● There must be no focal lesion (e.g., stroke) that could have caused the language problem

Semantic Variant (svPPA)

● Impairment of semantic memory

● Naming & single-word comprehension severely impaired

● Impaired object knowledge, particularly for low frequency or low familiarity items

● Reduced category fluency

● Surface dyslexia/dysgraphia (letter-by-letter reading)

● Relative spared grammatical and phonological elements

● Atrophic regions primarily in bilateral (but L>R) anterior temporal lobes

Relevant svPPA Assesments

• Impaired confrontation naming

  • Boston Naming test

• Impaired object knowledge, particularly for low frequency or low familiarity items

  • Pyramids and palm trees

  • may use very general language in sample

  • synonym judgement

• Surface dyslexia/dysgraphia

  • inability to spell irregularly spelled words (damaged orthographic lexicon) relying on phonological system

Nonfluent/Agrammatic Variant (nvPPA)

● Effortful. Halting speech, hesitations, speech-sound errors

● Reduced length of utterances

● Poor sentence construction

● Comprehension may be spared early on

● Motor speech disorders may be present (e.g., apraxia of speech),

● Atrophic regions primarily in anterior perisylvian region – left posterior fronto- insular atrophy.

Relevant nvPPA Assessments

• Motor control tasks

  • will perform poorly on tasks requiring motor control of articulators

• picture description task

  • will reveal effortful, halting, agrammatic speech

Logogenic Variant (lvPPA)

● Anomia

● Phonological errors, leading to impaired repetition

● Slowed speech rate

● Spared motor speech

● Spared single word comprehension and object knowledge

● Absence of frank agrammatism

● Atrophy primarily in the left temporo-parietal

region, including:

○ Posterior temporal cortex

○ Supramarginal and angular gyri

Relevant lvPPA Assessments

• Impaired confrontation naming

  • Boston Naming test

• Impaired repetition of sentences and phrases

  • especially for unpredictable phrases, as they can’t hold information in phonological working memory

• Phonological errors in spontaneous speech and naming

  • phonological paraphasias

  • difficulty with phoneme blending tasks

PPA Progression

First complaint:

• Often anomia: “I know what it is, but I just can’t say the name of it...”

• Impaired confrontation naming

• Word-finding difficulty in conversation

• Occasionally slurring speech or reduced verbal production

From there, progresses fluent or non-fluent aphasia

• Possible presentation of more than one variant time as symptoms progress?

• Impairment in other cognitive domains may begin to emerge

End stage: mutism and global cognitive impairment

Treatment for PPA

• Common aphasia treatments may be implemented for individuals with PPA

• People with PPA can improve communication through speech-language therapy

• Improvements in communication can be maintained for months following treatment

• Possibly, (lots) of practice can help preserve communication despite the inevitability of progression

• Trained items may be preserved longer than generalized treatment effects

• Always plan for future decline and adjust treatments as needed