Diagnostic Criteria for Primary Progressive Aphasia (Mesulam, 2003, NEJM)
- There is an insidious onset and gradual progressive impairment of word finding, object naming, grammar, or word comprehension manifested during conversation or assessed with the use of standard neuropsychological tests of language. Isolated motor speech disorders (such as dysarthria, apraxia of speech, aphemia) are not by themselves sufficient for making the diagnosis.
- All major limitations in activities of daily living can be attributed to the language impairment for at least two years after onset.
- Premorbid language function (except for developmental dyslexia) is known to be intact.
- Prominent apathy, disinhibition, loss of memory of recent events, visuospatial impairment, visual-recognition deficits, are absent during the initial phase of illness, as indicated by the history, evaluation of activities of daily living or neuropsychological testing.
- Acalculia (inability to perform simple mathematical calculations) and ideomotor apraxia (inability to pantomime movement as instructed by an examiner) can be present even in the first two years of illness.
- Other cognitive functions may be affected after the first two years of illness, but language remains the most impaired function throughout the course of the illness and deteriorated faster than other affected functions.
- Specific causes of aphasia, such as stroke or tumor, as ascertained by neuroimaging, are absent.
Current subtype classifications and criteria can be found in Gorno-Tempini, et al., 2011.