Treatment of Primary Progressive Aphasia
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Primary progressive aphasia (PPA) is a neurodegenerative disease that primarily affects language functions and often begins in the fifth or sixth decade of life. The devastating effects on work and home life call for the investigation of treatment alternatives. In this paper, we present a review of the literature on treatment approaches for this neurodegenerative disease. We also present new data from two intervention studies we have conducted, a behavioral one and a neuromodulatory one using transcranial direct current stimulation (tDCS) combined with written production intervention. We show that speech-language intervention improves language outcomes in individuals with PPA, and especially in the short term, tDCS augments generalization and maintenance of positive language outcomes. We also outline current issues and challenges in intervention approaches in PPA.
KeywordsDementia Primary progressive aphasia (PPA) Non-fluent agrammatic PPA Semantic variant PPA Logogpenic variant PPA Apraxia of speech (AOS) Transcranial direct current stimulation (tDCS) Neurodegeneration Neuromodulation
Some of the research reported in this paper, as well as the authors of the paper, were supported by NIDCD through R01 14129060, R01 DC011317, R01 DC03681, and R01 DC014475 and by the Science of Learning Institute of Johns Hopkins University.
Compliance with Ethics Guidelines
Conflict of Interest
Donna C. Tippett, Argye E. Hillis, and Kyrana Tsapkini declare no conflicts of interest.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
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- 11.•Mesulam MM, Wieneke C, Thompson C, Rogalski E, Weintraub S. Quantitative classification of primary progressive aphasia at early and mild impairment stages. Brain. 2012;135(Pt 5):1537–53. State-of-the art in quantifying symptoms of PPA and differential diagnosis of variants.PubMedCentralPubMedGoogle Scholar
- 15.Hillis AE, Heidler‐Gary J, Newhart M, Chang S, Ken L, Bak TH. Naming and comprehension in primary progressive aphasia: the influence of grammatical word class. Aphasiology. 2006;20(02-04):246–56.Google Scholar
- 27.Henry ML, Beeson PM, Alexander GE, Rapcsak SZ. Written language impairments in primary progressive aphasia: a reflection of damage to central semantic and phonological processes. J Cogn Neurosci. 2011.Google Scholar
- 29.••Tsapkini K, Hillis AE. Spelling intervention in post-stroke aphasia and primary progressive aphasia. Behav Neurol. 2013;26(1-2):55–66. Commonalities and differences in the treatment trajectory of spelling deficits between a case of post-stroke aphasia and a case of PPA.PubMedCentralPubMedGoogle Scholar
- 32.Ellis AW. Reading, writing and dyslexia: a cognitive analysis. Psychology Press, 1993.Google Scholar
- 33.Ellis AW, Young AW. Human cognitive neuropsychology. Hillsdale: Lawrence Erlbaum Associates; 1988.Google Scholar
- 34.Kiran S, Thompson CK, Hashimoto N. Training grapheme to phoneme conversion in patients with oral reading and naming deficits: a model-based approach. Aphasiology. 2001;15(9):855–76.Google Scholar
- 35.Kiran S. Training phoneme to grapheme conversion for patients with written and oral production deficits: a model-based approach. Aphasiology. 2005;19(1):53–76.Google Scholar
- 38.Best W, Nickels L. From theory to therapy in aphasia: where are we now and where to next? Neuropsychol Rehabil. 2000;10(3):231–47.Google Scholar
- 39.Bastiaanse R, Bosje M, Franssen M. Deficit-oriented treatment of word-finding problems: another replication. Aphasiology. 1996;10(4):363–83.Google Scholar
- 41.DeDe G, Parris D, Waters G. Teaching self-cues: a treatment approach for verbal naming. Aphasiology. 2003;17(5):465–80.Google Scholar
- 45.••Croot K, Nickels L, Laurence F, Manning M. Impairment‐and activity/participation-directed interventions in progressive language impairment: clinical and theoretical issues. Aphasiology. 2009;23(2):125–60. Examples and directions for treatment of aphasic symptoms in PPA.Google Scholar
- 51.••McNeil M, Small S, Masterson RJ, Fossett T. Behavioural and pharmacological treatment of lexical-semantic deficits in a single patient with primary progressive aphasia. A J Speech Lang Pathol. 1995;4(4):76–87. Effects of behavioral treatment of cueing hierarchy of predicative adjectives combined with dextroamphetamine treatment.Google Scholar
- 52.Schneider S, Thompson C, Luring B. Effects of verbal plus gestural matrix training on sentence production in a patient with primary progressive aphasia. Aphasiology. 1996;10(3):297–317.Google Scholar
- 54.••Newhart M, Davis C, Kannan V, Heidler‐Gary J, Cloutman L, Hillis AE. Therapy for naming deficits in two variants of primary progressive aphasia. Aphasiology. 2009;23(7-8):823–34. Variation of therapeutic approaches and results in a case of semantic and a case of logopenic PPA.Google Scholar
- 55.••Beeson PM, King RM, Bonakdarpour B, Henry ML, Cho H, Rapcsak SZ. Positive effects of language treatment for the logopenic variant of primary progressive aphasia. J Mol Neurosci. 2011;45(3):724–36. Improvement of oral and written naming after treatment in logopenic variant PPA.PubMedCentralPubMedGoogle Scholar
- 58.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.Google Scholar
- 60.••Fridriksson J, Richardson JD, Baker JM, Rorden C. Transcranial direct current stimulation improves naming reaction time in fluent aphasia: a double-blind, sham-controlled study. Stroke. 2011;42(3):819–21. Follow-up study on RT improvement in naming in post-stroke aphasia using tDCS.PubMedGoogle Scholar
- 62.••Boggio PS, Khoury LP, Martins DC, Martins OE, de Macedo EC, Fregni F. Temporal cortex direct current stimulation enhances performance on a visual recognition memory task in Alzheimer disease. J Neurol Neurosurg Psychiatry. 2009;80(4):444–7. tDCS contributed to longer lasting visual memories in AD than sham.PubMedGoogle Scholar
- 68.Meinzer M, Lindenberg R, Darkow R, Ulm L, Copland D, Floel A. Transcranial direct current stimulation and simultaneous functional magnetic resonance imaging. J Vis Exp. 2014; (86). doi(86): 10.3791/51730.
- 83.Polania R, Paulus W, Nitsche MA. Modulating cortico-striatal and thalamo-cortical functional connectivity with transcranial direct current stimulation. Hum Brain Mapp. 2011.Google Scholar
- 87.••Marangolo P, Marinelli CV, Bonifazi S, et al. Electrical stimulation over the left inferior frontal gyrus (IFG) determines long-term effects in the recovery of speech apraxia in three chronic aphasics. Behav Brain Res. 2011;225(2):498–504. tDCS improves outcome of apraxia of speech treatment more than sham in post-stroke aphasia.PubMedGoogle Scholar
- 90.Chrysikou EG, Hamilton RH. Noninvasive brain stimulation in the treatment of aphasia: exploring interhemispheric relationships and their implications for neurorehabilitation. Restor Neurol Neurosci. 2011.Google Scholar
- 92.••Datta A, Baker JM, Bikson M, Fridriksson J. Individualized model predicts brain current flow during transcranial direct-current stimulation treatment in responsive stroke patient. Brain Stimul. 2011;4(3):169–74. tDCS Electrode placement according to individualized computational models of current flow.PubMedCentralPubMedGoogle Scholar
- 96.Brunoni AR, Nitsche MA, Bolognini N, et al. Clinical research with transcranial direct current stimulation (tDCS): challenges and future directions. Brain Stimul. 2011.Google Scholar
- 100.••Tsapkini K, Frangakis C, Gomez Y, Davis C, Hillis AE. Augmentation of spelling therapy with transcranial direct current stimulation in primary progressive aphasia: preliminary results and challenges. Aphasiology. 2014;28(8-9):1112–30. tDCS improves spelling in PPA: effects are generalized and last longer than sham.PubMedCentralPubMedGoogle Scholar
- 107.••Trebbastoni A, Raccah R, de Lena C, Zangen A, Inghilleri M. Repetitive deep transcranial magnetic stimulation improves verbal fluency and written language in a patient with primary progressive aphasia-logopenic variant (LPPA). Brain Stimul. 2013;6(4):545–53. TMS improves verbal fluency and written language in PPA.PubMedGoogle Scholar