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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 2. Diagnostic Criteria for the semantic 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 semantic variant PPA
Both of the following core features must be present:

  1. Impaired confrontation naming

  2. Impaired single-word comprehension

At least three of the following other diagnostic features must be present:

  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)

II. Imaging-supported semantic variant PPA diagnosis
Both of the following criteria must be present:

  1. Clinical diagnosis of semantic variant PPA

  2. Imaging must show one or more of the following results:
    1. Predominant anterior temporal lobe atrophy
    2. Predominant anterior temporal hypoperfusion or hypometabolism on SPECT or PET
III. Semantic variant PPA with definite pathology
Clinical diagnosis (criterion A below) and either criterion B or C must be present:

  1. Clinical diagnosis of semantic 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