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Diagnostic criteria for semantic variant PPA

Diagnostic criteria for semantic variant PPA
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 3 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 1 below) and either criterion 2 or 3 must be present:
  1. Clinical diagnosis of semantic variant PPA
  2. Histopathologic evidence of a specific neurodegenerative pathology (eg, FTLD-tau, FTLD-TDP, AD, other)
  3. Presence of a known pathogenic mutation
AD: Alzheimer disease; FTLD: frontotemporal lobar degeneration; 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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