Ethical and Practical Challenges of the Communication and Behavioral Manifestations of Primary Progressive Aphasia

Abstract

The communication and behavioral manifestations of primary progressive aphasia (PPA) present ethical and practical challenges for individuals with this clinical syndrome as well as for individuals who are involved closely in their care. In this article, cases representing all three PPA variants (logopenic variant, nonfluent agrammatic, semantic variant) are presented to illustrate commonly encountered situations in which self-determination is at risk in decisions about housing, driving, social interactions, finances, and treatment interventions. Potential approaches, including patient/family education, implementation of safeguards, redirection to meaningful activities, and protections against vulnerability in treatment decisions, are described to preserve autonomy in patients with this neurodegenerative clinical syndrome.

Keywords

primary progressive aphasia - logopenic variant PPA - nonfluent agrammatic PPA - semantic variant PPA

• References

• 1 Mesulam MM. Primary progressive aphasia. Ann Neurol 2001; 49 (04) 425-432
• 2 Montembeault M, Brambati SM, Gorno-Tempini ML, Migliaccio R. Clinical, anatomical, and pathological features in the three variants of primary progressive aphasia: a review. Front Neurol 2018; 9: 692
• 3 Gorno-Tempini ML, Hillis AE, Weintraub S. , et al. Classification of primary progressive aphasia and its variants. Neurology 2011; 76 (11) 1006-1014
• 4 Gorno-Tempini ML, Brambati SM, Ginex V. , et al. The logopenic/phonological variant of primary progressive aphasia. Neurology 2008; 71 (16) 1227-1234
• 5 Gorno-Tempini ML, Dronkers NF, Rankin KP. , et al. Cognition and anatomy in three variants of primary progressive aphasia. Ann Neurol 2004; 55 (03) 335-346
• 6 Ogar JM, Dronkers NF, Brambati SM, Miller BL, Gorno-Tempini ML. Progressive nonfluent aphasia and its characteristic motor speech deficits. Alzheimer Dis Assoc Disord 2007; 21 (04) S23-S30
• 7 Mandelli ML, Vilaplana E, Brown JA. , et al. Healthy brain connectivity predicts atrophy progression in non-fluent variant of primary progressive aphasia. Brain 2016; 139 (Pt 10): 2778-2791
• 8 Mandelli ML, Vitali P, Santos M. , et al. Two insular regions are differentially involved in behavioral variant FTD and nonfluent/agrammatic variant PPA. Cortex 2016; 74: 149-157
• 9 Hodges JR, Patterson K, Oxbury S, Funnell E. Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy. Brain 1992; 115 (Pt 6): 1783-1806
• 10 Spinelli EG, Mandelli ML, Miller ZA. , et al. Typical and atypical pathology in primary progressive aphasia variants. Ann Neurol 2017; 81 (03) 430-443
• 11 Acosta-Cabronero J, Patterson K, Fryer TD. , et al. Atrophy, hypometabolism and white matter abnormalities in semantic dementia tell a coherent story. Brain 2011; 134 (Pt 7): 2025-2035
• 12 Snowden J, Neary D, Mann D. Frontotemporal lobar degeneration: clinical and pathological relationships. Acta Neuropathol 2007; 114 (01) 31-38
• 13 Mesulam M, Wicklund A, Johnson N. , et al. Alzheimer and frontotemporal pathology in subsets of primary progressive aphasia. Ann Neurol 2008; 63 (06) 709-719
• 14 Hodges JR, Mitchell J, Dawson K. , et al. Semantic dementia: demography, familial factors and survival in a consecutive series of 100 cases. Brain 2010; 133 (Pt 1): 300-306
• 15 Harris JM, Jones M. Pathology in primary progressive aphasia syndromes. Curr Neurol Neurosci Rep 2014; 14 (08) 466
• 16 Giannini LAA, Irwin DJ, McMillan CT. , et al. Clinical marker for Alzheimer disease pathology in logopenic primary progressive aphasia. Neurology 2017; 88 (24) 2276-2284
• 17 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
• 18 Gómez-Tortosa E, Rigual R, Prieto-Jurczynska C. , et al. Behavioral evolution of progressive semantic aphasia in comparison with nonfluent aphasia. Dement Geriatr Cogn Disord 2016; 41 (1-2): 1-8
• 19 Van Langenhove T, Leyton CE, Piguet O, Hodges JR. Comparing longitudinal behavior changes in the primary progressive aphasias. J Alzheimers Dis 2016; 53 (03) 1033-1042
• 20 Tippett DC, Thompson CB, Demsky C, Sebastian R, Wright A, Hillis AE. Differentiating between subtypes of primary progressive aphasia and mild cognitive impairment on a modified version of the Frontal Behavioral Inventory. PLoS One 2017; 12 (08) e0183212
• 21 Beauchamp TL, Childress JF. Principles of Biomedical Ethics. 7th ed. New York: Oxford University Press; 2012
• 22 National Commission for the Protection of Human Subjects of Biomedical and Behavioral Research. The Belmont report: ethical principles and guidelines for the protection of human subjects of research. Published April 18, 1979. Available at: http://www.hhs.gov/ohrp/regulations-and-policy/belmont-report/read-the-belmont-report/index.html . Accessed January 23, 2020
• 23 Aldous K, Tolmie R, Worrall L, Ferguson A. Speech-language pathologists' contribution to the assessment of decision-making capacity in aphasia: a survey of common practices. Int J Speech Lang Pathol 2014; 16 (03) 231-241
• 24 Carr F. Financial and legal decision-making capacity in the aphasic population - a narrative review. Med Leg J 2016; 84 (04) 195-199
• 25 Sullivan WF, Heng J. Supporting adults with intellectual and developmental disabilities to participate in health care decision making. Can Fam Physician 2018; 64 (Suppl. 02) S32-S36
• 26 Teng C, Loy CT, Sellars M. , et al. Making decisions about long-term institutional care placement among people with dementia and their caregivers: systematic review of qualitative studies. Gerontologist 2019 . Doi: 10.1093/geront/gnz046 [Epub ahead of print]
• 27 Rodenburg-Vandenbussche S, Carlier I, van Vliet I, van Hemert A, Stiggelbout A, Zitman F. Patients' and clinicians' perspectives on shared decision-making regarding treatment decisions for depression, anxiety disorders, and obsessive-compulsive disorder in specialized psychiatric care. J Eval Clin Pract 2020; 26 (02) 645-658
• 28 Le Rhun E, Richard F, Pasquier F. Natural history of primary progressive aphasia. Neurology 2005; 65 (06) 887-891
• 29 Sajjadi SA, Patterson K, Arnold RJ, Watson PC, Nestor PJ. Primary progressive aphasia: a tale of two syndromes and the rest. Neurology 2012; 78 (21) 1670-1677
• 30 Wicklund MR, Duffy JR, Strand EA, Machulda MM, Whitwell JL, Josephs KA. Quantitative application of the primary progressive aphasia consensus criteria. Neurology 2014; 82 (13) 1119-1126
• 31 Sebastian R, Thompson CB, Wang NY. , et al. Patterns of decline in naming and semantic knowledge in primary progressive aphasia. Aphasiology 2018; 32 (09) 1010-1030
• 32 Henry ML, Meese MV, Truong S, Babiak MC, Miller BL, Gorno-Tempini ML. Treatment for apraxia of speech in nonfluent variant primary progressive aphasia. Behav Neurol 2013; 26 (1-2): 77-88
• 33 Henry ML, Hubbard HI, Grasso SM. , et al. Retraining speech production and fluency in non-fluent/agrammatic primary progressive aphasia. Brain 2018; 141 (06) 1799-1814 [Published correction appears in Brain 2018;141(7):e57]
• 34 Meyer AM, Tippett DC, Turner RS, Friedman RB. Long-term maintenance of anomia treatment effects in primary progressive aphasia. Neuropsychol Rehabil 2019; 29 (09) 1439-1463
• 35 Rogalski EJ, Saxon M, McKenna H. , et al. Communication bridge: a pilot feasibility study of Internet-based speech-language therapy for individuals with progressive aphasia. Alzheimers Dement (N Y) 2016; 2 (04) 213-221
• 36 Keator LM, Wright AE, Saxena S. , et al. Distinguishing logopenic from semantic & nonfluent variant primary progressive aphasia: patterns of linguistic and behavioral correlations. Neurocase 2019; 25 (3-4): 98-105
• 37 Watson CL, Possin K, Allen IE. , et al. Visuospatial functioning in the primary progressive aphasias. J Int Neuropsychol Soc 2018; 24 (03) 259-268
• 38 Tippett DC, Breining B, Goldberg E. , et al. Visuomotor figure construction and visual figure delayed recall and recognition in primary progressive aphasia. Aphasiology 2019;
• 39 Wise RJS. Language systems in normal and aphasic human subjects: functional imaging studies and inferences from animal studies. Br Med Bull 2003; 65: 95-119
• 40 Khayum B, Wieneke C, Rogalski E, Robinson J, O'Hara M. Thinking outside the stroke: treating primary progressive aphasia (PPA). Perspect Gerontol 2012; 17 (02) 37-49
• 41 Primary progressive aphasia. Orphanet. Available at: http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=95432 . Accessed October 13, 2019
• 42 Medina J, Weintraub S. Depression in primary progressive aphasia. J Geriatr Psychiatry Neurol 2007; 20 (03) 153-160
• 43 Banks SJ, Weintraub S. Cognitive deficits and reduced insight in primary progressive aphasia. Am J Alzheimers Dis Other Demen 2008; 23 (04) 363-371
• 44 Jokel R, Meltzer J. , D R J, et al. Group intervention for individuals with primary progressive aphasia and their spouses: Who comes first?. J Commun Disord 2017; 66: 51-64
• 45 Mooney A, Beale N, Fried-Oken M. Group communication treatment for individuals with PPA and their partners. Semin Speech Lang 2018; 39 (03) 257-269
• 46 Miller ZA, Miller BL. Artistic creativity and dementia. Prog Brain Res 2013; 204: 99-112
• 47 Tippett DC, Sugarman J. Discussing advance directives under the Patient Self-Determination Act: A unique opportunity for speech-language pathologists to help persons with aphasia. Am J Speech Lang Pathol 1996; 5 (02) 31-34
• 48 Gorno-Tempini ML, Ogar JM, Brambati SM. , et al. Anatomical correlates of early mutism in progressive nonfluent aphasia. Neurology 2006; 67 (10) 1849-1851
• 49 Rogalski EJ, Khayum B. A life participation approach to primary progressive aphasia intervention. Semin Speech Lang 2018; 39 (03) 284-296