Table 1.

Diagnostic classification criteria for primary progressive aphasia and its variants.

A diagnosis of PPA requires all of the following features:

• The most prominent clinical feature is difficulty with language.

• The language deficits are the principal cause of impaired activities of daily living.

• Aphasia is the most prominent deficit at symptom onset and for the initial phases of disease.

In addition, the following four criteria must be answered negatively:

• Pattern of deficits is better accounted for by other nonneurodegenerative nervous system or medical disorders.

• Cognitive disturbance is better accounted for by a psychiatric diagnosis.

• There are prominent initial episodic memory, visual memory, and visuoperceptual impairments.

• There is a prominent initial behavioral disturbance.