Diagnostic criteria for nonfluent/agrammatic variant PPA

I. Clinical diagnosis of nonfluent/agrammatic variant PPA

At least one of the following core features must be present:

Agrammatism in language production
Effortful, halting speech with inconsistent speech sound errors and distortions (apraxia of speech)

At least 2 of 3 of the following other features must be present:

Impaired comprehension of syntactically complex sentences
Spared single-word comprehension
Spared object knowledge

II. Imaging-supported nonfluent/agrammatic variant diagnosis

Both of the following criteria must be present:

Clinical diagnosis of nonfluent/agrammatic variant PPA
Imaging must show one or more of the following results:
1. Predominant left posterior fronto-insular atrophy on MRI
2. Predominant left posterior fronto-insular hypoperfusion or hypometabolism on SPECT or PET

III. Nonfluent/agrammatic variant PPA with definite pathology

Clinical diagnosis (criterion 1 below) and either criterion 2 or 3 must be present:

Clinical diagnosis of nonfluent/agrammatic variant PPA
Histopathologic evidence of a specific neurodegenerative pathology (eg, FTLD-tau, FTLD-TDP, AD, other)
Presence of a known pathogenic mutation

AD: Alzheimer disease; FTLD: frontotemporal lobar degeneration; MRI: magnetic resonance imaging; PET: positron emission tomography; PPA: primary progressive aphasia; SPECT: single-photon emission computed tomography; TDP: TAR DNA-binding protein 43.

Reproduced with permission from: Gorno-Tempini ML, Hillis AE, Weintraub S, et al. Classification of primary progressive aphasia and its variants. Neurology 2011; 76:1006. DOI: 10.1212/WNL.0b013e31821103e6. Copyright © 2011 American Academy of Neurology. Unauthorized reproduction of this material is prohibited.

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Diagnostic criteria for nonfluent/agrammatic variant PPA