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Primary Progressive Aphasia

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Handbook on the Neuropsychology of Aging and Dementia

Part of the book series: Clinical Handbooks in Neuropsychology ((CHNEURO))

Abstract

Primary progressive aphasia (PPA) is a form of frontotemporal dementia that is characterized by progressive language decline. Language impairment is the primary feature in the initial stages and the main cause of impairment in activities of daily living. In recent years, three main behaviorally defined variants of PPA have been identified: (1) semantic, (2) logopenic, and (3) nonfluent/agrammatic. While the neuropathological features are heterogeneous, biomarkers and patterns of brain atrophy have been identified in association with the various subtypes. This chapter describes the clinical features of PPA, the most recent classification system, and the clinical pathologic correlates. In addition, a suggested language battery with specific tests for different aspects of language is provided to aid the clinician in differential diagnosis. A case study is included that demonstrates a classic presentation of PPA and how the test battery is used for diagnostic differentiation.

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References

  1. Mesulam MM. Primary progressive aphasia. Ann Neurol. 2001;49(4):425–32.

    Article  Google Scholar 

  2. Gorno-Tempini ML, et al. Classification of primary progressive aphasia and its variants. Neurology. 2011;76(11):1006–14.

    Article  Google Scholar 

  3. Pick A. Ueber die Beziehungen der senilen Hirnatrophie zur Aphasie. Prager Medicinische Wochenschrift. 1892;17:165–7.

    Google Scholar 

  4. Sérieux P. Sur un cas de surdité verbale pure. Revue De Medecine. 1983;13:733–50.

    Google Scholar 

  5. Gorno-Tempini ML, et al. Cognition and anatomy in three variants of primary progressive aphasia. Ann Neurol. 2004;55(3):335–46.

    Article  Google Scholar 

  6. Onyike CU, Diehl-Schmid J. The epidemiology of frontotemporal dementia. Int Rev Psychiatry. 2013;25(2):130–7.

    Article  Google Scholar 

  7. Johnson JK, et al. Frontotemporal lobar degeneration: demographic characteristics of 353 patients. Arch Neurol. 2005;62(6):925–30.

    Article  Google Scholar 

  8. Knopman DS, Roberts RO. Estimating the number of persons with frontotemporal lobar degeneration in the US population. J Mol Neurosci. 2011;45(3):330–5.

    Article  Google Scholar 

  9. Grossman M. Primary progressive aphasia: clinicopathological correlations. Nat Rev Neurol. 2010;6(2):88–97.

    Article  Google Scholar 

  10. Goodglass H, Kaplan E, Barresi B. Boston diagnostic aphasia examination (BDAE-3). Austin, TX: Pro-Ed; 2001.

    Google Scholar 

  11. Kertesz A. Western aphasia battery-revised. 2006th ed. New York: Grune & Stratton; 2007.

    Google Scholar 

  12. Howard D, Patterson K. The pyramid and palms trees test. London: Pearson; 1992.

    Google Scholar 

  13. Reynolds CK, Kamphaus RW. Test of irregular word reading efficiency. Lutz, FL: Psychological Assessment Resources, Inc; 2008.

    Google Scholar 

  14. Benson DF. Fluency in aphasia: correlation with radioactive scan localization. Cortex. 1967;3:373–94.

    Article  Google Scholar 

  15. Greenwald ML, Nadeau SE, Gonzalez Rothi LJ. Fluency. In: Nadeau SE, Gonzalez Rothi LJ, Crosson B, editors. Aphasia and language: theory to practice. New York: The Guilford Press; 2000.

    Google Scholar 

  16. Caso F, et al. In vivo signatures of nonfluent/agrammatic primary progressive aphasia caused by FTLD pathology. Neurology. 2014;82(3):239–47.

    Article  Google Scholar 

  17. Gorno-Tempini ML, et al. Anatomical correlates of early mutism in progressive nonfluent aphasia. Neurology. 2006;67(10):1849–51.

    Article  Google Scholar 

  18. Binder JR, Desai RH. The neurobiology of semantic memory. Trends Cogn Sci. 2011;15(11):527–36.

    Article  Google Scholar 

  19. Hodges JR, Patterson K. Semantic dementia: a unique clinicopathological syndrome. Lancet Neurol. 2007;6(11):1004–14.

    Article  Google Scholar 

  20. Patterson K, Hodges JR. Deterioration of word meaning: implications for reading. Neuropsychologia. 1992;30(12):1025–40.

    Article  Google Scholar 

  21. Bonner MF, et al. Reversal of the concreteness effect in semantic dementia. Cogn Neuropsychol. 2009;26(6):568–79.

    Article  Google Scholar 

  22. Knibb JA, Hodges JR. Semantic dementia and primary progressive aphasia: a problem of categorization? Alzheimer Dis Assoc Disord. 2005;19(Suppl 1):S7–14.

    Article  Google Scholar 

  23. Baddeley A. Working memory and language: an overview. J Commun Disord. 2003;36(3):189–208.

    Article  Google Scholar 

  24. Gorno-Tempini ML, et al. The logopenic/phonological variant of primary progressive aphasia. Neurology. 2008;71(16):1227–34.

    Article  Google Scholar 

  25. Mesulam M. Primary progressive aphasia: a dementia of the language network. Dement Neuropsychol. 2013;7(1):2–9.

    Article  Google Scholar 

  26. Davies RR, et al. The pathological basis of semantic dementia. Brain. 2005;128(Pt 9):1984–95.

    Article  Google Scholar 

  27. Mesulam M, et al. Alzheimer and frontotemporal pathology in subsets of primary progressive aphasia. Ann Neurol. 2008;63(6):709–19.

    Article  Google Scholar 

  28. Rohrer JD, et al. Progressive logopenic/phonological aphasia: erosion of the language network. NeuroImage. 2010;49(1):984–93.

    Article  Google Scholar 

  29. Mesulam MM, et al. Words and objects at the tip of the left temporal lobe in primary progressive aphasia. Brain. 2013;136(Pt 2):601–18.

    Article  Google Scholar 

  30. Rohrer JD, et al. Patterns of cortical thinning in the language variants of frontotemporal lobar degeneration. Neurology. 2009;72(18):1562–9.

    Article  Google Scholar 

  31. Whitwell JL, et al. Longitudinal patterns of regional change on volumetric MRI in frontotemporal lobar degeneration. Dement Geriatr Cogn Disord. 2004;17(4):307–10.

    Article  Google Scholar 

  32. Scheltens P, et al. Structural magnetic resonance imaging in the practical assessment of dementia: beyond exclusion. Lancet Neurol. 2002;1(1):13–21.

    Article  Google Scholar 

  33. Borruat F-X. Posterior cortical atrophy: review of the recent literature. Curr Neurol Neurosci Rep. 2013;13(12):406.

    Article  Google Scholar 

  34. Crutch SJ, et al. Posterior cortical atrophy. Lancet Neurol. 2012;11(2):170–8.

    Article  Google Scholar 

  35. Lomen-Hoerth C, Anderson T, Miller B. The overlap of amyotrophic lateral sclerosis and frontotemporal dementia. Neurology. 2002;59(7):1077–9.

    Article  Google Scholar 

  36. Bozeat S, et al. Non-verbal semantic impairment in semantic dementia. Neuropsychologia. 2000;38(9):1207–15.

    Article  Google Scholar 

  37. Benton AL, Hamsher KD, Sivan AB. Multilingual aphasia examination: MAE. Lutz, FL: Psychological Assessment Resources, Inc.; 1994.

    Google Scholar 

  38. PsychologicalCorporation. WIAT III: Wechsler individual achievement test. [Assessment instrument]. San Antonio, TX: Psychological Corp; 2009.

    Google Scholar 

  39. Woodcock RW, McGrew KS, Mather N. Woodcock-Johnson III. Itasca, IL: Riverside Publishing; 2001.

    Google Scholar 

  40. Bier N, Paquette G, Macoir J. Smartphone for smart living: using new technologies to cope with everyday limitations in semantic dementia. Neuropsychol Rehabil. 2018;28:734–54.

    Article  Google Scholar 

  41. Evans WS, et al. Relearning and retaining personally-relevant words using computer-based flashcard software in primary progressive aphasia. Front Hum Neurosci. 2016;10:561.

    Article  Google Scholar 

  42. Jokel R, et al. Group intervention for individuals with primary progressive aphasia and their spouses: who comes first? J Commun Disord. 2017;66:51–64.

    Article  Google Scholar 

  43. Tippett DC, Hillis AE, Tsapkini K. Treatment of primary progressive aphasia. Curr Treat Options Neurol. 2015;17(8):362.

    Article  Google Scholar 

  44. Douglas JT. Adaptation to early-stage nonfluent/agrammatic variant primary progressive aphasia: a first-person account. Am J Alzheimers Dis Other Demen. 2014;29(4):289–92.

    Article  Google Scholar 

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Correspondence to Sara J. Swanson .

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Swanson, S.J., Pillay, S.B., Elverman, K.H., Umfleet, L.G. (2019). Primary Progressive Aphasia. In: Ravdin, L.D., Katzen, H.L. (eds) Handbook on the Neuropsychology of Aging and Dementia. Clinical Handbooks in Neuropsychology. Springer, Cham. https://doi.org/10.1007/978-3-319-93497-6_31

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