Event Abstract

Back to Event

Quantifying the Differentiated Trajectory of the Wernicke’s-like Presentation of Logopenic Variant Primary Progressive Aphasia (lvPPA)

Jeanne Gallée1*, Jessica Collins2, 3, Claire Cordella2, 3, Rania Ezzo2, Shalom Kim2, Bradford C. Dickerson2, 3 and Megan Quimby2
  • 1 Harvard University, United States
  • 2 Massachusetts General Hospital, Harvard Medical School, United States
  • 3 Harvard Medical School, United States

Primary Progressive Aphasia (PPA) is a neurodegenerative aphasic syndrome with devastating effects on language. The current study focuses on the logopenic variant of PPA (lvPPA), typically characterized by significantly impaired word-retrieval and auditory verbal working memory with relatively intact single word comprehension and grammar (Gorno-Tempini et al., 2008; Mesulam et al., 2009; Leyton et al., 2011; Sapolsky et al., 2011). However, multiple cases have been documented where patients diagnosed with lvPPA developed speech patterns more similar to that of Wernicke’s aphasia, such as press of speech, excessive jargon, and impaired comprehension (Rohrer et al., 2009; Knels & Danek, 2010; Caffarra et al., 2013; Bodner, Domahs, & Benke, 2015). The primary aim of this study was to create a quantifiable comparison between the progressions of “typical” and “Wernicke’s-like” (WlvPPA) lvPPA presentations. To accomplish this, we tracked domains of speech and language longitudinally. Data was collected from individuals with “typical” lvPPA profiles (N = 12; mean age: 72.5, range: 64-82) and Wernicke’s-like lvPPA presentations (N = 3; mean age: 72.33, range: 67 - 83) over multiple timepoints. Participants were recruited from the Frontotemporal Disorders Unit at Massachusetts General Hospital (MGH). Structured speech samples describing the Western Aphasia Battery Picnic Scene (WAB; Kertesz, 1982) were recorded at every assessment. The audio files were processed using a MATLAB-based program, Speech Pause Analysis (SPA), to algorithmically estimate speech and pause segments in continuous speech (Green, Beukelman, & Ball, 2004). Participants completed a battery of standardized language assessments at every visit. Measures of interest for the speech samples included speech rate and proportion of speech per sample. Cortical thickness measures were derived from language-network regions of interest using each patient’s most recent structural MRI. Mixed effects linear regression analyses support that WlvPPA participants had significantly higher speech rates (t = 5.01, p <.001) and proportions of speech per sample (t = -2.97, p = .012) over time. Generalized Estimating Equations (GEEs) models were used for the longitudinal analysis of the language domains. Repetition (𝜒2𝜈 = 8.13, p = .004), auditory comprehension (𝜒2𝜈 = 4.09, p <.000), functional communication (𝜒2𝜈 =15.86, p <.000), and global language ability (𝜒2𝜈 = 9.96, p = .001) were all significantly predicted by the WlvPPA classification, whereas confrontation naming (𝜒2𝜈 = 1.4, p = .24) and single word comprehension (𝜒2𝜈 = 0.53, p = .467) were not. A whole brain GLM comparing cortical thickness for a single patient with WlvPPA to that of 12 patients with canonical lvPPA revealed that accentuated cortical thinning in the left temporal pole may contribute to this patient's clinical phenotype. Our results support that quantifiable measures of speech samples, such as rate and proportion of speech, contribute to the distinct profiles of the lvPPA and WlvPPA presentations. Further measures that distinguish between these presentations include performance on standardized assessments of language. Finally, cortical atrophy may provide further insight on the neurological basis of Wernicke’s-like characteristics. Our observations suggest that further research is needed to flesh out the heterogeneity of the course of patients with lvPPA.

Figure 1
Image

Acknowledgements

This research was supported by the NIH-NIDCD grant R01DC014296 and the NIH-NIDCD training grant 5T32DC000038-27 (supporting JG).

References

Bodner, T., Domahs, F., & Benke, T. (2015). Primäre progressive Aphasie: kanonische und nicht-kanonische Formen? Ein Fallbericht. Zeitschrift für Neuropsychologie. Caffarra, P., Gardini, S., Cappa, S., Dieci, F., Concari, L., Barocco, F., ... & Prati, G. D. R. (2013). Degenerative jargon aphasia: Unusual progression of logopenic/phonological progressive aphasia?. Behavioural neurology, 26(1-2), 89-93. Cordella, C., Dickerson, B. C., Quimby, M., Yunusova, Y., & Green, J. R. (2017). Slowed articulation rate is a sensitive diagnostic marker for identifying non-fluent primary progressive aphasia. Aphasiology, 31(2), 241-260. Gorno‐Tempini, M. L., Dronkers, N. F., Rankin, K. P., Ogar, J. M., Phengrasamy, L., Rosen, H. J., ... & Miller, B. L. (2004). Cognition and anatomy in three variants of primary progressive aphasia. Annals of Neurology: Official Journal of the American Neurological Association and the Child Neurology Society, 55(3), 335-346. Gorno-Tempini, M. L., Hillis, A. E., Weintraub, S., Kertesz, A., Mendez, M., Cappa, S. F., Ogar, J., Rohrer, J., Black, S. Boeve, B.F., Manes, F., Drokers, N.F., Vandenberghe, R., Rascovsky, K., Patterson, K., Miller, B.L., Knopman, D.S., Hodges, J.R., Mesulam, M.M. & Grossman, M. (2011). Classification of primary progressive aphasia and its variants. Neurology, 76(11), 1006- 1014. Gorno-Tempini, M. L., Brambati, S. M., Ginex, V., Ogar, J., Dronkers, N. F., Marcone, A., Perani, D., Garibotto, V., Cappa, S.F., & Miller, B. L. (2008). The logopenic/phonological variant of primary progressive aphasia. Neurology, 71(16), 1227-1234. Green, J. R., Beukelman, D. R., & Ball, L. J. (2004). Algorithmic estimation of pauses in extended speech samples of dysarthric and typical speech. Journal of Medical Speech- Language Pathology, 12(4), 149. Fischl, B., Van Der Kouwe, A., Destrieux, C., Halgren, E., Ségonne, F., Salat, D. H., ... & Caviness, V. (2004). Automatically parcellating the human cerebral cortex. Cerebral cortex, 14(1), 11-22. Kertesz, A. (1982). Western Aphasia Battery. New York: Grune and Stratton. Leyton, C. E., Villemagne, V. L., Savage, S., Pike, K. E., Ballard, K. J., Piguet, O., ... & Hodges, J. R. (2011). Subtypes of progressive aphasia: application of the international consensus criteria and validation using β-amyloid imaging. Brain, 134(10), 3030-3043. Knels, C., & Danek, A. (2010). Loss of word-meaning with spared object semantics in a case of mixed primary progressive aphasia. Brain and language, 113(2), 96-100. Mesulam, M., Wieneke, C., Rogalski, E., Cobia, D., Thompson, C., & Weintraub, S. (2009). Quantitative template for subtyping primary progressive aphasia. Archives of neurology, 66(12), 1545-1551. Rohrer, J. D., Rossor, M. N., & Warren, J. D. (2009). Neologistic jargon aphasia and agraphia in primary progressive aphasia. Journal of the neurological sciences, 277(1-2), 155-159. Sapolsky, D., Bakkour, A., Negreira, A., Nalipinski, P., Weintraub, S., Mesulam, M. M., Caplan, D., & Dickerson, B. C. (2010). Cortical neuroanatomic correlates of symptom severity in primary progressive aphasia. Neurology,75(4), 358-366. Sapolsky, D., Domoto-Reilly, K., Negreira, A., Brickhouse, M., McGinnis, S., & Dickerson, B. C. (2011). Monitoring progression of primary progressive aphasia: current approaches and future directions. Neurodegenerative Disease Management, 1(1), 43-55. Wilson, S. M., Henry, M. L., Besbris, M., Ogar, J. M., Dronkers, N. F., Jarrold, W., ... & Gorno- Tempini, M. L. (2010). Connected speech production in three variants of primary progressive aphasia. Brain, 133(7), 2069-2088.

Keywords: primary progressive aphasia, lvppa, speech rate, Frontotemporal Dementia, Wernicke's aphasia

Conference: Academy of Aphasia 57th Annual Meeting, Macau, Macao, SAR China, 27 Oct - 29 Oct, 2019.

Presentation Type: Platform presentation

Topic: Eligible for student award

Citation: Gallée J, Collins J, Cordella C, Ezzo R, Kim S, Dickerson BC and Quimby M (2019). Quantifying the Differentiated Trajectory of the Wernicke’s-like Presentation of Logopenic Variant Primary Progressive Aphasia (lvPPA). Front. Hum. Neurosci. Conference Abstract: Academy of Aphasia 57th Annual Meeting. doi: 10.3389/conf.fnhum.2019.01.00075

Copyright: The abstracts in this collection have not been subject to any Frontiers peer review or checks, and are not endorsed by Frontiers. They are made available through the Frontiers publishing platform as a service to conference organizers and presenters.

The copyright in the individual abstracts is owned by the author of each abstract or his/her employer unless otherwise stated.

Each abstract, as well as the collection of abstracts, are published under a Creative Commons CC-BY 4.0 (attribution) licence (https://creativecommons.org/licenses/by/4.0/) and may thus be reproduced, translated, adapted and be the subject of derivative works provided the authors and Frontiers are attributed.

For Frontiers’ terms and conditions please see https://www.frontiersin.org/legal/terms-and-conditions.

Received: 06 May 2019; Published Online: 09 Oct 2019.

* Correspondence: Mx. Jeanne Gallée, Harvard University, Cambridge, United States, jgallee@g.harvard.edu