Primary progressive aphasia
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At the time the article was created Frank Gaillard had no recorded disclosures.View Frank Gaillard's current disclosures
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Primary progressive aphasia is a group of neurodegenerative disorders mainly characterized by increasing language impairment. The group is clinically and pathologically heterogeneous and includes two clinical variants usually associated with frontotemporal lobar degeneration pathology and one clinical variant usually associated with Alzheimer disease pathology.
Primary progressive aphasia is characterized by gradual impairment of language that predates, usually by many years, the onset of more global cognitive deficits.
Inclusion criteria 2:
- most prominent clinical feature is difficulty with language
- these deficits are the principal cause of impaired daily living activities
- aphasia should be the most prominent deficit at symptom onset and for the initial phases of the disease
Exclusion criteria 2:
- pattern of deficit is better accounted for by other nondegenerative nervous system or other disorders
- cognitive disturbance is better accounted for by a psychiatric diagnosis
- prominent initial episodic memory, visual memory, and visuoperceptual impairments
- prominent, initial behavioral disturbance
There are three clinical variants 2:
- nonfluent (agrammatic) variant: characterized by motor speech deficit with difficulty articulating phonemes and also agrammatism
- semantic variant (semantic dementia): characterized by deficits in single-word object naming and comprehension
- logopenic variant (logopenic dementia): characterized by deficits in repetition and single-word retrieval, resulting in frequent word-finding pauses
The nonfluent and semantic variants are considered clinical syndromes of frontotemporal dementia, while the logopenic variant is not.
The nonfluent and semantic variants are usually associated with frontotemporal lobar degeneration pathology, while the logopenic variant is usually associated with Alzheimer pathology 1,2,4. It is, therefore, important to note that this group is not only clinically heterogeneous but also is the result of a number of pathological processes1,4.
History and etymology
Primary progressive aphasia was first described in 1982.
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- 2. Gorno-Tempini ML, Hillis AE, Weintraub S et-al. Classification of primary progressive aphasia and its variants. Neurology. 2011;76 (11): 1006-14. Neurology (full text) - doi:10.1212/WNL.0b013e31821103e6 - Free text at pubmed - Pubmed citation
- 3. Neary D, Snowden JS, Gustafson L et-al. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology. 1998;51 (6): 1546-54. Neurology (full text) - doi:10.1212/WNL.51.6.1546 - Pubmed citation
- 3. Madhavan A, Whitwell JL, Weigand SD, Duffy JR, Strand EA, Machulda MM, Tosakulwong N, Senjem ML, Gunter JL, Lowe VJ, Petersen RC, Jack CR, Josephs KA. FDG PET and MRI in logopenic primary progressive aphasia versus dementia of the Alzheimer's type. PloS one. 8 (4): e62471. doi:10.1371/journal.pone.0062471 - Pubmed