Primary progressive aphasia

Czech version

Authors: Z. Cséfalvay ^1,5; R. Bajtošová ²; J. Keller ^3,4; E. Straková ⁵; R. Matěj ⁶; R. Rusina ^2,5
Authors‘ workplace: Katedra logopedie, Pedagogická, fakulta, UK, Bratislava, Slovensko ¹; Neurologická klinika 3. LF UK a Thomayerova nemocnice, Praha, Česká, republika ²; Neurologická klinika 3. LF UK a FN, Královské Vinohrady, Praha, Česká, republika ³; Oddělení radiologie, Nemocnice, Na Homolce, Praha, Česká republika ⁴; Neurologická klinika a Centrum klinických neurověd, 1. LF UK a VFN, Praha, Česká republika ⁵; Ústav patologie a molekulární, medicíny, 3. LF UK a Thomayerova, nemocnice, Praha, Česká republika ⁶
Published in: Cesk Slov Neurol N 2020; 83/116(3): 226-239
Category: Minimonography
doi: https://doi.org/10.14735/amcsnn2020226

Overview

Primary progressive aphasia (PPA) results from selective neurodegeneration mainly in the areas of the language-dominant hemisphere, disrupting processes whose proper functioning is ensured by a complex language network in the brain, localized mainly in the cortex but also in subcortical and deeper brain areas. Speech/language deficits are the first and long-term dominant problem in the initial stage of the disease, causing significant impairment in activities of everyday life in PPA patients. We summarize the most important aspects of PPA with special emphasis on the characteristics of three PPA variants – the nonfluent/agrammatic, semantic and logopenic variants –and primary apraxia of speech are described in terms of linguistic deficits and co-occurring cognitive disorders (neuropsychological aspects) and neuropsychiatric disorders, especially behavioral disorders. We provide information that can help to identify key symptoms more rapidly in clinical practice. The clinical picture of PPA is complemented by localization of brain atrophy on MRI and also by summarizing specific PPA neuropathology.

Keywords:

primary progressive aphasia – nonfl uent agrammatic variant – semantic variant – logopenic variant – primary apraxia of speech – frontotemporal lobar degeneration – Alzheimer’s disease

Sources

1. Cséfalvay Z. Progresivní afázie. In: Rusina R, Matěj R (eds). Neurodegenerativní onemocnění, 2. přepracované a doplněné vydání. Praha: Mladá fronta 2019: 177–191.

2. Mesulam MM. Slowly progressive aphasia without generalized dementia. Ann Neurol 1982; 11 (6): 592–598. doi: 10.1002/ana.410110607.

3. Kirshner HS. Frontotemporal dementia and primary progressive phasia, a review. Neuropsych Dis Treat 2014; 10: 1045–1055. doi: 10.2147/NDT.S38821.

4. Grossman M. Primary progressive aphasia: clinicopathological correlations. Nat Rev Neurol 2010; 6 (2): 88–97. doi: 10.1038/nrneurol.2009.216.

5. Gorno-Tempini ML, Hillis AE, Weintraub S et al. Classification of primary progressive aphasia and its variants. Neurology 2011; 76 (11): 1006–1014. doi: 10.1212/WNL.0b013e31821103e6.

6. Miller BL. Frontotemporal dementia. Oxford, UK: Oxford University Press 2014.

7. Rosen HJ, Allison SC, Ogar JM et al. Behavioral features in semantic dementia vs other forms of progressive aphasias. Neurology 2006; 67 (10): 1752–1756. doi: 10.1212/01.wnl.0000247630.29222.34.

8. Banks SJ, Weintraub S. Neuropsychiatric symptomsin behavioural variant frontotemporal dementia and primary progressive aphasia. J Geriatr Psychiatry Neurol 2008; 21 (2): 133–141. doi: 10.1177/0891988708316856.

9. Park MH, Kim EJ, Park KW et al. Behavioural and neuropsychiatric disturbance in three clinical subtypes of frontotemporal dementia: A Clinical Research Center for Dementia of South Korea-FTD Study. Australas J Ageing 2017; 36 (1): 46–51. doi: 10.1111/ajag.12374.

10. Midorikawa A, Kumfor F, Leyton CE et al. Characterisation of „positive“ behaviours in primary progressive aphasia. Dement Geriatr Cogn Disord 2017; 44 (3–4): 119–128. doi: 10.1159/000478852.

11. Finney GR, Heilman KM. Artwork before and after onset of progressive nonfluent aphasia. Cogn Behav Neurol 2007; 20 (1): 7–10. doi: 10.1097/WNN.0b013e31802b6c1f.

12. Green HA, Patterson K. Jigsaws-a preserved ability in semantic dementia. Neuropsychologia 2009; 47 (2): 569–576. doi: 10.1016/j.neuropsychologia.2008.10.015.

13. Wu TQ, Miller ZA, Adhimoolam B et al. Verbal creativity in semantic variant primary progressive aphasia. Neurocase 2015; 21 (1): 73–78. doi: 10.1080/13554794.2013.860179.

14. Mechl M, Tintěra J, Žižka J et al. Protokoly MR zobrazování. Praha: Galén 2014: 40–43.

15. Modirrousta M, Price BH, Dickerson BC. Neuropsychiatric symptoms in primary progressive aphasia: phenomenology, pathophysiology, and approach to assessment and treatment. Neurodegener Dis Manag 2013; 3 (2): 133–146. doi: 10.2217/nmt.13.6.

16. Bruun M, Koikkalainen J, Rhodius-Meester HF et al. Detecting frontotemporal dementia syndromes using MRI biomarkers. Neuroimage Clin 2019; 22: 101711. doi: 10.1016/j.nicl.2019.101711.

17. Canu E, Agosta F, Imperiale F et al. Added value of multimodal MRI to the clinical diagnosis of primary progressive aphasia variants. Cortex 2019; 113: 58–66. doi: 10.1016/j.cortex.2018.11.025.

18. Battistella G, Henry M, Gesierich B et al. Differential intrinsic functional connectivity changes in semantic variant primary progressive aphasia. Neuroimage Clin 2019; 22: 101797. doi: 10.1016/j.nicl.2019.101797.

19. Rusina R, Cséfalvay Z, Kovacs GG et al. Globular glial tauopathy type I presenting as atypical progressive aphasia, with comorbid limbic-predominant age-related TDP-43 encephalopathy. Front Aging Neurosci 2019; 11: 336. doi: 10.3389/fnagi.2019.00336.

20. Šutovský S, Králová M, Kollár B et al. Frontotemporálna lobárna degenerácia z pohľadu nových klinicko-patologických korelácií. Cesk Slov Neurol N 2013; 76/109 (6): 679–689.

21. Josephs KA, Duffy JR, Strand EA et al. Characterizing a neurodegenerative syndrome: primary progressive apraxia of speech. Brain 2012; 135 (Pt 5): 1522–1536. doi: 10.1093/brain/aws032.

22. Grossman M, Irwin DJ. Primary Progressive aphasia and stroke aphasia. Continuum (Minneap Minn) 2018; 24 (3): 745–767. doi: 10.1212/CON.0000000000000618.

23. Kopeček M. Psychomotorické tempo, rychlost řeči a myšlení. Psychiat Praxi 2007; 8: 213–215

24. Bettcher B. Neuropsychological assesment of primary progressive aphasia. Perspect Neurophysiol Neurogenic Speech Lang Disord 2014; 24 (4): 128–136. doi: 10.1044/nnsld24.4.128.

25. Tippett DC, Thompson CB, Demsky C et al. Differentiating between subtypes of primary progressive aphasia and mild cognitive impairment on a modified version of the Frontal Behavioral Inventory. PLoS One 2017; 12 (8): e0183212. doi: 10.1371/journal.pone.0183212.

26. Montembeault M, Brambati SM, Gorno-Tempini ML et al. Clinical, anatomical, and pathological features in the three variants of primary progressive aphasia: a review. Front Neurol 2018; 9: 692. doi: 10.3389/fneur.2018.00692.

27. Menšíková K, Tučková L, Kaňovský P. Atypický parkinsonizmus a frontotemporální demence – klinické, patologické a genetické aspekty. Cesk Slov Neurol N 2016; 79/112 (3): 275–286. doi: 10.14735/amcsnn2016275

28. Barkhof F, Fox NC, Bastos-Leite AJ et al. Neuroimaging in dementia. Heidelberg: Springer 2011.

29. Warrington EK. The selective impairment of semantic memory. Q J Exp Psychol 1975; 27 (4): 635–657. doi: 10.1080/14640747508400525.

30. Snowden JS, Goulding PJ, Neary D. Semantic dementia: a form of circumscribed cerebral atrophy. Behav Neurol 1989; 2 (3): 167–182.

31. Miller ZA, Miller BL. Artistic creativity and dementia. Prog Brain Res 2013; 204: 99–112. doi: 10.1016/B978-0-444-63287-6.00005-1.

32. Seeley WW, Bauer AM, Miller BL et al. The natural history of temporal variant frontotemporal dementia. Neurology 2005; 64 (8): 1384–1390. doi: 10.1212/01.WNL.0000158425.46019.5C.

33. Shany-Ur T, Rankin KP. Personality and social cognition in neurodegenerative disease. Neurology 2011; 24 (6): 550–555. doi: 10.1097/WCO.0b013e32834cd42a.

34. D’Anna L, Mesulam MM, Thiebaut de Schotten M et al. Frontotemporal networks and behavioral symptoms in primary progressive aphasia. Neurology 2016; 86 (15): 1393–1399. doi: 10.1212/WNL.0000000000002579.

35. Mesulam MM, Weintraub S. Spectrum of primary progressive aphasia. In: Rossor MN (ed). Unusual dementias. London: BaillièreTindall 1992: 583–609.

36. Gorno-Tempini ML, Dronkers NF, Rankin KP et al. Cognition and anatomy in three variants of primary progressive aphasia. Ann Neurol 2004; 55 (33): 335–346. doi: 10.1002/ana.10825.

37. Tee BL, Gorno-Tempini ML. Primary progressive aphasia: a model for neurodegenerative disease. Curr Opin Neurol 2019; 32 (2): 255–265. doi: 10.1097/WCO. 0000000000000673.

38. Marshall CR, Hardy CJ, Volkmer A et al. Primary progressive aphasia: a clinical approach. J Neurol 2018; 265 (6): 1474–1490. doi: 10.1007/s00415-018-8762-6.

39. Gorno-Tempini ML, Brambati SM, Ginex V et al. The logopenic/phonological variant of primary progressive aphasia. Neurology 2008; 71 (16): 1227–1234. doi: 10.1212/01.wnl.0000320506.79811.da.

40. Rabinovici GD, Jagust WJ, Furst AJ et al. Abeta amyloid and glucose metabolism in three variants of primary progressive aphasia. Ann Neurol 2008; 64 (4): 388–401. doi: 10.1002/ana.21451.

41. Leyton CE, Villegamne LS, Savage S. Subtypes of progressive aphasia: application of the international consensus criteria and validation using b-amyloid imaging. Brain 2011; 134 (Pt 10): 3030–3043. doi: 10.1093/brain/awr216.

42. Sajjadi SA, Patterson K. Logopenic, mixed, or Alzheimer-related aphasia? Neurology 2014; 82 (13): 1127–1131. doi: 10.1212/WNL.0000000000000271.

43. Butts AM, Machulda MM, Duffy JR. Neuropsychological profiles differ among the three variants of primary progressive aphasia. J Int NeuropsycholSoc 2015; 21 (6): 429–435. doi: 10.1017/S1355617715000399.

44. Rohrer JD, Warren, JD. Phenomenology and anatomy of abnormal behaviours in primary progressive aphasia. J Neurol Sci 2010; 293 (1–2): 35–38. doi: 10.1016/j.jns.2010.03.012.

45. Josephs KA, Duffy JR, Strand EA et al. The evolution of primary progressive apraxia of speech. Brain 2014; 137 (Pt 10): 2783–2795. doi: 10.1093/brain/awu223.

46. Fernandez YM, Frucht J. Primary progressive apraxia: an unusual ideomotor syndrome. J Clin Mov Dis 2017; 4: 17. doi: 10.1186/s40734-017-0064-0.

47. Singh TD, Duffy JR, Strand EA et al. Neuropsychiatric symptoms in primary progressive aphasia and apraxia of speech. Dement Geriatr Cogn Disord 2015; 39 (3–4): 228–238. doi: 10.1159/000369062.

48. Deramecourt V, Lebert F, Debachy B et al. Prediction of pathology in primary progressive language and speech disorders. Neurology 2010; 74 (1): 42–49. doi: 10.1212/WNL.0b013e3181c7198e.

49. Boxer AL, Gold M, Feldman H et al. New directions in clinical trials for frontotemporal lobar degeneration: methods and outcome measures. Alzheimers Dement 2020; 16 (1): 131–143. doi: 10.1016/j.jalz.2019.06.4956.

50. Rusina R, Cséfalvay Z. Behaviorální a řečové poruchy u primární progresivní afázie. Neurol praxi 2018; 19: 411–416.

51. Croot K, Nickels L, Laurence F et al. Impairment and activity/participation-directed interventions in progressive language impairment: clinical and theoretical issues. Aphasiology 2009; 23 (2): 125–160. doi: 10.1080/02687030801943179.

52. Tippett DC, Hillis AE, Tsapkin K. Treatment of primary progressive aphasia. Curr Treat Options Neurol 2015; 17 (8): 362. doi: 10.1007/s11940-015-0362-5.

53. Henry ML, Hubbard HI, Grasso SM et al. Retraining speech production and fluency in non-fluent/agrammatic primary progressive aphasia. Brain 2018; 141 (6): 1799–1814. doi: 10.1093/brain/awy101.