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geri:dementia:primary-progressive-aphasia-ppa [on September 16, 2018]
geri:dementia:primary-progressive-aphasia-ppa [on June 29, 2020]
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 +====== Primary Progressive Aphasia (PPA) ======
 +{{INLINETOC}}
 +===== Primer =====
 +**Primary Progressive Aphasia (PPA)** is a neurological syndrome associated with certain types of dementia. [[geri:​dementia:​alzheimers|Alzheimer’s Disease]] and [[geri:​dementia:​frontotemporal|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.
  
 +<alert icon="​fa fa-arrow-circle-right fa-lg fa-fw" type="​success">​See also article: **[[neurology:​approach-aphasia|]]**</​alert>​
 +===== Variants =====
 +There are three variants of PPA:
 +  - **Semantic** (the dominant type and is present in most instances of PPA)
 +  - **Logopenic** (LPA), due to atrophy in the left posterior temporal cortex and inferior parietal lobule
 +  - **Non-fluent/​agrammatic** (stumbling for words, grammar is very poor)
 +
 +<WRAP group>
 +
 +<WRAP third column>
 +==== Semantic ====
 +**Both** of:
 +  - 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)
 +  - Impaired single word comprehension
 +At least ''​3''​ of 4:
 +  - Impaired object knowledge
 +  - Surface dyslexia/​dysgraphia
 +  - Spared repetition
 +  - Spared speech production
 +
 +</​WRAP>​
 +<WRAP third column>
 +==== Logopenic ====
 +**Both** of:
 +  - Impaired single word retrieval
 +  - Impaired repetition
 +
 +At least ''​3''​ of 4:
 +  - Speech errors in spontaneous speech and naming
 +  - Spared single-word comprehension and object knowledge
 +  - Spared motor speech
 +  - Absence of frank agrammatism
 +</​WRAP>​
 +
 +<WRAP third column>
 +==== 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:
 +  - Agrammatism
 +  - Apraxia of speech
 +At least ''​2''​ of 3:
 +  - Impaired complex comprehension
 +  - Spared single-word comprehension
 +  - Spared object knowledge
 +</​WRAP>​
 +</​WRAP>​
 +===== Diagnostic Criteria =====
 +  * [[https://​www.ncbi.nlm.nih.gov/​pmc/​articles/​PMC3059138/​|Gorno-Tempini,​ M. L., Hillis, A. E., Weintraub, S., Kertesz, A., Mendez, M., Cappa, S. E. E. A., ... & Manes, F. (2011). Classification of primary progressive aphasia and its variants. Neurology, WNL-0b013e31821103e6.]]
 +
 +===== 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