Primary Progressive Aphasia (PPA)

Primer

Primary Progressive Aphasia (PPA) is a neurological syndrome associated with certain types of dementia. Alzheimer’s Disease and frontotemporal dementia are the most common underlying forms of dementia in which PPA occurs. PPA typically begins as gradual, subtle language deficits that progresses to a nearly complete inability to speak.

Variants

There are three variants of PPA:

  1. Semantic (the dominant type and is present in most instances of PPA)
  2. Logopenic (LPA), due to atrophy in the left posterior temporal cortex and inferior parietal lobule
  3. Non-fluent/agrammatic (stumbling for words, grammar is very poor)

Non-fluent/Agrammatic

The Non-fluent/Agrammatic subtype is characterized by distortion of word and sentence construction. This includes abnormal order of words (i.e. - syntax), distortion of word endings, misuse of pronouns, and a lack of grammatical words such as articles (words that define a noun as specific or unspecific, e.g. - a, an, the) and prepositions (these are words show the relationship between the noun and pronoun in a sentence, e.g. - at, for, in, off, on, over, under, into, upon, onto, out of, from within)

At least 1 of 2:

  1. Agrammatism
  2. Apraxia of speech

At least 2 of 3:

  1. Impaired complex comprehension
  2. Spared single-word comprehension
  3. Spared object knowledge

Semantic

Both of:

  1. Impaired confrontation naming (i.e. - showing an object or a line drawing of an object (e.g. - a spoon) to a patient and requesting the correct verbal label for that object)
  2. Impaired single word comprehension

At least 3 of 4:

  1. Impaired object knowledge
  2. Surface dyslexia/dysgraphia
  3. Spared repetition
  4. Spared speech production

Logopenic

Both of:

  1. Impaired single word retrieval
  2. Impaired repetition

At least 3 of 4:

  1. Speech errors in spontaneous speech and naming
  2. Spared single-word comprehension and object knowledge
  3. Spared motor speech
  4. Absence of frank agrammatism

Diagnostic Criteria

Rating Scales

Progressive Aphasia Severity Score (PASS)

  • Articulation
  • Fluency
  • Syntax and grammar
  • Word retrieval and expression
  • Repetition
  • Auditory comprehension
  • Single word comprehension
  • Reading
  • Writing
  • Functional communication