Table 1.

Primary progressive aphasia 2011 diagnostic consensus criteria algorithm

PPA clinical diagnostic criteria
Inclusion criteria
(1) Most prominent clinical feature is difficulty with language
(2) Aphasia should be the most prominent deficit at symptom onset and for the initial phases of the disease
(3) These deficits are the principal cause of impaired daily living activities
Exclusion criteria
(1) Pattern of deficits is better accounted for by other nondegenerative nervous system or medical disorders
(2) Cognitive disturbance is better accounted for by a psychiatric diagnosis
(3) Prominent initial episodic memory, visual memory, and visuoperceptual impairments
(4) Prominent initial behavioural disturbance
	Nonfluent/Agrammatic variant PPA	Semantic variant PPA	Logopenic variant PPA
Clinical diagnosis	Core features: (at least 1) (1) Agrammatism in language production (2) Effortful, halting speech with inconsistent speech sound errors and distortions (apraxia of speech) Supporting features: (at least 2) (1) Impaired comprehension of syntactically complex sentences (2) Spared single-word comprehension (3) Spared object knowledge	Core features: (both) (1) Impaired confrontation naming (2) Impaired single-word comprehension Supporting features: (at least 3) (1) Impaired object knowledge, particularly for low frequency or low familiarity items (2) Surface dyslexia or dysgraphia (3) Spared repetition (4) Spared speech production (grammar and motor speech)	Core features: (both) (1) Impaired single-word retrieval in spontaneous speech and naming (2) Impaired repetition of sentences and phrases Supporting features: (at least 3) (1) Speech (phonologic) errors in spontaneous speech and naming (2) Spared single-word comprehension and object knowledge (3) Spared motor speech (4) Absence of frank agrammatism
Imaging supported diagnosis (both present)	(1) Clinical diagnosis of nfvPPA	(1) Clinical diagnosis of svPPA	(1) Clinical diagnosis of lvPPA
	(2) Imaging: (at least 1)	(2) Imaging: (at least 1)	(2) Imaging: (at least 1)
	(a) Predominant left posterior frontoinsular atrophy on MRI	(a) Predominant anterior temporal lobe atrophy on MRI	(a) Predominant left posterior perisylvian or parietal atrophy on MRI
	(b) Predominant left posterior frontoinsular hypoperfusion or hypometabolism on SPECT or PET	(b) Predominant anterior temporal hypoperfusion or hypometabolism on SPECT or PET	(b) Predominant left posterior perisylvian or parietal hypoperfusion or hypometabolism on SPECT or PET
PPA with definite diagnosis	Clinical diagnosis fulfilled
	AND
	(1) Histopathologic evidence of a specific neurodegenerative disorder (e.g. FTLD-tau, FTLD-TDP, AD, other)
	OR
	(2) Presence of a known pathogenic mutation

Adapted with permission from [8,9]. AD, Alzheimer’s disease; FTLD-tau, frontotemporal lobar degeneration-tau; FTLD-TDP, frontotemporal lobar degeneration–TAR DNA-binding protein; lvPPA, logopenic variant primary progressive aphasia; nfvPPA, nonfluent/agrammatic primary progressive aphasia; PPA, primary progressive aphasia; svPPA, semantic variant primary progressive aphasia.