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. Author manuscript; available in PMC: 2018 May 1.
Published in final edited form as: Neurol Clin. 2017 May;35(2):339–374. doi: 10.1016/j.ncl.2017.01.008

Table 3. Diagnostic criteria for nonfluent/agrammatic variant PPA.

(From Gorno-Tempini ML, Hillis AE, Weintraub S, et al. Classification of primary progressive aphasia and its variants. Neurology. 2011)

I. Clinical diagnosis of nonfluent/agrammatic variant PPA:
At least one of the following core features must be present:

  1. Agrammatism in language production

  2. 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:

  1. Impaired comprehension of syntactically complex sentences

  2. Spared single-word comprehension

  3. Spared object knowledge

II. Imaging-supported nonfluent/agrammatic variant diagnosis
Both of the following criteria must be present:

  1. Clinical diagnosis of nonfluent/agrammatic variant PPA

  2. Imaging must show one or more of the following results:
    1. Predominant left posterior frontoinsular atrophy on MRI or
    2. Predominant left posterior frontoinsular hypoperfusion or hypometabolism on SPECT or PET
III. Nonfluent/agrammatic variant PPA with definite pathology
Clinical diagnosis (criterion A below) and either criterion B or C must be present:

  1. Clinical diagnosis of nonfluent/agrammatic variant PPA

  2. Histopathologic evidence of a specific neurodegenerative pathology (e.g., FTLD-tau, FTLD-TDP, AD, other)

  3. Presence of a known pathogenic mutation