Primary progressive aphasia

Diagnosis

Clinical criteria for PPA (Gorno-Tempini 2011)

1. Most prominent clinical feature is difficulty with language
2. Deficits are the principle cause of impaired daily living activities
3. Aphasia should be the most prominent deficit at symptom onset and for the initial phases of the disease
4. The pattern of deficit must not be better accounted for by other nondegenerative nervous system or medical disorders
5. Cognitive disturbance is not better accounted for by a psychiatric diagnosis
6. There is not prominent initial episodic memory, visual memory, or visuoperceptual impairments
7. There is not prominent initial behavioral disturbance

Variants:

Logopenic variant primary progressive aphasia

Nonfluent / agrammatic variant primary progressive aphasia

Semantic variant primary progressive aphasia

References

Gorno-Tempini, M. L. et al. Classification of primary progressive aphasia and its variants. Neurology 76, 1006–1014 (2011). PubMed link