Journal of Neurology

, Volume 264, Issue 1, pp 121–130 | Cite as

Amyloid pet in primary progressive aphasia: case series and systematic review of the literature

  • Alberto Villarejo-GalendeEmail author
  • Sara Llamas-Velasco
  • Adolfo Gómez-Grande
  • Verónica Puertas-Martín
  • Israel Contador
  • Pilar Sarandeses
  • Marta González-Sánchez
  • Rocío Trincado
  • Patrick Pilkington
  • Sebastián Ruiz-Solis
  • David A. Pérez-Martínez
  • Alejandro Herrero-San Martín
Original Communication


Primary progressive aphasia (PPA) is considered a heterogeneous syndrome, with different clinical subtypes and neuropathological causes. Novel PET biomarkers may help to predict the underlying neuropathology, but many aspects remain unclear. We studied the relationship between amyloid PET and PPA variant in a clinical series of PPA patients. A systematic review of the literature was performed. Patients with PPA were assessed over a 2-year period and classified based on language testing and the International Consensus Criteria as non-fluent/agrammatic (nfvPPA), semantic (svPPA), logopenic variant (lvPPA) or as unclassifiable (ucPPA). All patients underwent a Florbetapir (18-F) PET scan and images were analysed by two nuclear medicine physicians, using a previously validated reading method. Relevant studies published between January 2004 and January 2016 were identified by searching Medline and Web of Science databases. Twenty-four PPA patients were included (13 women, mean age 68.8, SD 8.3 years; range 54–83). Overall, 13/24 were amyloid positive: 0/2 (0%) nfvPPA, 0/4 (0%) svPPA, 10/14 (71.4%) lvPPA and 3/4 (75%) ucPPA (p = 0.028). The systematic review identified seven relevant studies, six including all PPA variants and one only lvPPA. Pooling all studies together, amyloid PET positivity was 122/224 (54.5%) for PPA, 14/52 (26.9%) for nfvPPA, 6/47 (12.8%) for svPPA, 101/119 for lvPPA (84.9%) and 12/22 (54.5%) for ucPPA. Amyloid PET may help to identify the underlying neuropathology in PPA. It could be especially useful in ucPPA, because in these cases it is more difficult to predict pathology. ucPPA is frequently associated with amyloid pathology.


PET Amyloid Primary progressive aphasia PIB Florbetapir 


Compliance with ethical standards

Conflicts of interest

The authors declare that they have no conflict of interest.

Ethical standard

The Ethics Committee for medical research of Hospital Universitario Doce de Octubre has approved this study.


Drs. Llamas-Velasco (CM13/00079) and González-Sánchez (CM15/00222) have been supported by a Rio Hortega grant for clinical research (Instituto de Salud Carlos III, Spain).


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Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  • Alberto Villarejo-Galende
    • 1
    • 4
    • 5
    Email author
  • Sara Llamas-Velasco
    • 1
    • 4
  • Adolfo Gómez-Grande
    • 2
  • Verónica Puertas-Martín
    • 1
    • 4
  • Israel Contador
    • 3
  • Pilar Sarandeses
    • 2
  • Marta González-Sánchez
    • 1
    • 4
  • Rocío Trincado
    • 1
    • 4
  • Patrick Pilkington
    • 2
  • Sebastián Ruiz-Solis
    • 2
  • David A. Pérez-Martínez
    • 1
    • 4
    • 5
  • Alejandro Herrero-San Martín
    • 1
    • 4
  1. 1.Department of NeurologyHospital Universitario Doce de OctubreMadridSpain
  2. 2.Department of Nuclear MedicineHospital Universitario Doce de OctubreMadridSpain
  3. 3.Department of Basic Psychology, Psychobiology and Methodology of Behavioral ScienceUniversity of SalamancaSalamancaSpain
  4. 4.Imas12. CIBERNEDMadridSpain
  5. 5.Universidad Complutense de MadridMadridSpain

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