| 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
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| 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:
a. Predominant left posterior fronto-insular atrophy on MRI b. 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
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