Skip to main content
    • Aa
    • Aa
  • Get access
    Check if you have access via personal or institutional login
  • Cited by 58
  • Cited by
    This article has been cited by the following publications. This list is generated based on data provided by CrossRef.

    Foxe, David Leyton, Cristian E. Hodges, John R. Burrell, James R. Irish, Muireann and Piguet, Olivier 2016. The neural correlates of auditory and visuospatial span in logopenic progressive aphasia and Alzheimer's disease. Cortex, Vol. 83, p. 39.

    Gorno-Tempini, Maria Luisa and Pressman, Peter 2016. Neurobiology of Language.

    Lee, Suzee E. and Miller, Bruce L. 2016. Non-Alzheimer's and Atypical Dementia.

    Botha, Hugo Duffy, Joseph R. Whitwell, Jennifer L. Strand, Edythe A. Machulda, Mary M. Schwarz, Christopher G. Reid, Robert I. Spychalla, Anthony J. Senjem, Matthew L. Jones, David T. Lowe, Val Jack, Clifford R. and Josephs, Keith A. 2015. Classification and clinicoradiologic features of primary progressive aphasia (PPA) and apraxia of speech. Cortex, Vol. 69, p. 220.

    Magnin, E. Teichmann, M. Martinaud, O. Moreaud, O. Ryff, I. Belliard, S. Pariente, J. Moulin, T. Vandel, P. and Démonet, J.-F. 2015. Particularités du variant logopénique au sein des aphasies progressives primaires. Revue Neurologique, Vol. 171, Issue. 1, p. 16.

    Matias-Guiu, Jordi A. Cabrera-Martín, María Nieves Moreno-Ramos, Teresa García-Ramos, Rocío Porta-Etessam, Jesús Carreras, José Luis and Matías-Guiu, Jorge 2015. Clinical course of primary progressive aphasia: clinical and FDG-PET patterns. Journal of Neurology, Vol. 262, Issue. 3, p. 570.

    Duffy, Joseph R. Strand, Edythe A. and Josephs, Keith A. 2014. Motor speech disorders associated with primary progressive aphasia. Aphasiology, Vol. 28, Issue. 8-9, p. 1004.

    Harciarek, Michał Sitek, Emilia J. and Kertesz, Andrew 2014. The patterns of progression in primary progressive aphasia—Implications for assessment and management. Aphasiology, Vol. 28, Issue. 8-9, p. 964.

    Kertesz, Andrew and Harciarek, Michał 2014. Primary progressive aphasia. Scandinavian Journal of Psychology, Vol. 55, Issue. 3, p. 191.

    Leyton, Cristian E. and Hodges, John R. 2014. Differential diagnosis of primary progressive aphasia variants using the international criteria. Aphasiology, Vol. 28, Issue. 8-9, p. 909.

    Sławek, Jarosław 2014. Neuroimaging in the Differential Diagnosis of Primary Progressive Aphasia – Illustrative Case Series in the Light of New Diagnostic Criteria. Polish Journal of Radiology, Vol. 79, p. 251.

    Harciarek, Michał and Cosentino, Stephanie 2013. Language, executive function and social cognition in the diagnosis of frontotemporal dementia syndromes. International Review of Psychiatry, Vol. 25, Issue. 2, p. 178.

    Konstantinopoulou, Eleni Aretouli, Eleni Ioannidis, Panagiotis Karacostas, Dimitrios and Kosmidis, Mary H. 2013. Behavioral disturbances differentiate frontotemporal lobar degeneration subtypes and Alzheimer's disease: evidence from the Frontal Behavioral Inventory. International Journal of Geriatric Psychiatry, Vol. 28, Issue. 9, p. 939.

    Etcheverry, Louise Seidel, Barbara Grande, Marion Schulte, Stephanie Pieperhoff, Peter Südmeyer, Martin Minnerop, Martina Binkofski, Ferdinand Huber, Walter Grodzinsky, Yosef Amunts, Katrin and Heim, Stefan 2012. The time course of neurolinguistic and neuropsychological symptoms in three cases of logopenic primary progressive aphasia. Neuropsychologia, Vol. 50, Issue. 7, p. 1708.

    Kambanaros, Maria and Grohmann, Kleanthes K. 2012. BATting multilingual primary progressive aphasia for Greek, English, and Czech. Journal of Neurolinguistics, Vol. 25, Issue. 6, p. 520.

    Lange, Inga Grande, Marion Willmes, Klaus Kastrau, Frank Fimm, Bruno Heim, Stefan and Huber, Walter 2012. Charakteristiken der flüssigen und der nicht-flüssigen primär progressiven Aphasie. Zeitschrift für Neuropsychologie, Vol. 23, Issue. 1, p. 7.

    Thompson, Cynthia K. Cho, Soojin Hsu, Chien-Ju Wieneke, Christina Rademaker, Alfred Weitner, Bing Bing Mesulam, M. Marsel and Weintraub, Sandra 2012. Dissociations between fluency and agrammatism in primary progressive aphasia. Aphasiology, Vol. 26, Issue. 1, p. 20.

    Wilson, Stephen M. Galantucci, Sebastiano Tartaglia, Maria Carmela and Gorno-Tempini, Maria Luisa 2012. The neural basis of syntactic deficits in primary progressive aphasia. Brain and Language, Vol. 122, Issue. 3, p. 190.

    Harciarek, Michał and Kertesz, Andrew 2011. Primary Progressive Aphasias and Their Contribution to the Contemporary Knowledge About the Brain-Language Relationship. Neuropsychology Review, Vol. 21, Issue. 3, p. 271.

    Lee, Suzee E. Rabinovici, Gil D. Mayo, Mary Catherine Wilson, Stephen M. Seeley, William W. DeArmond, Stephen J. Huang, Eric J. Trojanowski, John Q. Growdon, Matthew E. Jang, Jung Y. Sidhu, Manu See, Tricia M. Karydas, Anna M. Gorno-Tempini, Maria-Luisa Boxer, Adam L. Weiner, Michael W. Geschwind, Michael D. Rankin, Katherine P. and Miller, Bruce L. 2011. Clinicopathological correlations in corticobasal degeneration. Annals of Neurology, Vol. 70, Issue. 2, p. 327.

  • Journal of the International Neuropsychological Society, Volume 9, Issue 5
  • July 2003, pp. 710-719

Primary progressive aphasia: Diagnosis, varieties, evolution

  • DOI:
  • Published online: 01 July 2003

A referred cohort of 67 clinically defined PPA patients were compared to 99 AD patients with formal language and nonverbal cognitive tests in a case control design. Language fluency was determined at the first and last follow up visits. Quantitation of sulcal and ventricular atrophy on MRI was carried out in 46 PPA and 53 AD patients. Most PPA patients (57%) are relatively fluent when first examined. Visuospatial and memory functions are initially preserved. Aphemic, stuttering, “pure motor” presentation, or agrammatic aphasia are seen less frequently. Later most PPAs become logopenic and nonfluent, even those with semantic aphasia (dementia). In contrast, AD patients were more fluent and had relatively lower comprehension, but better overall language performance. MRI showed significant left sided atrophy in most PPA patients. Subsequent to PPA, 25 patients developed behavioral manifestations of frontotemporal dementia and 15 the corticobasal degeneration syndrome, indicating the substantial clinical overlap of these conditions. Language testing, particularly fluency scores supported by neuroimaging are helpful differentiating PPA from AD. The fluent–nonfluent dichotomy in PPA is mostly stage related. The aphemic-logopenic-agrammatic and semantic distinction is useful, but the outcomes converge. (JINS, 2003, 9, 710–719.)

Corresponding author
Reprint requests to: Dr. Andrew Kertesz, Department of Clinical Neurological Sciences, St. Joseph's Hospital, University of Western Ontario, London, ON N6A 4V2, Canada. E-mail:
Recommend this journal

Email your librarian or administrator to recommend adding this journal to your organisation's collection.

Journal of the International Neuropsychological Society
  • ISSN: 1355-6177
  • EISSN: 1469-7661
  • URL: /core/journals/journal-of-the-international-neuropsychological-society
Please enter your name
Please enter a valid email address
Who would you like to send this to? *