Skip to main content Accessibility help
×
Home
Hostname: page-component-99c86f546-md8df Total loading time: 0.24 Render date: 2021-12-08T01:49:22.675Z Has data issue: true Feature Flags: { "shouldUseShareProductTool": true, "shouldUseHypothesis": true, "isUnsiloEnabled": true, "metricsAbstractViews": false, "figures": true, "newCiteModal": false, "newCitedByModal": true, "newEcommerce": true, "newUsageEvents": true }

Neuropsychological Profiles of Patients with Progressive Apraxia of Speech and Aphasia

Published online by Cambridge University Press:  22 July 2021

Angelina J. Polsinelli
Affiliation:
Department of Neurology, Indiana University School of Medicine, Indianapolis, IN, USA
Mary M. Machulda
Affiliation:
Department of Psychiatry and Psychology, Division of Neuropsychology, Mayo Clinic, Rochester, MN, USA
Peter R. Martin
Affiliation:
Department of Biomedical Statistics & Informatics, Mayo Clinic, Rochester, MN, USA
Joseph R. Duffy
Affiliation:
Department of Neurology, Mayo Clinic, Rochester, MN, USA
Heather M. Clark
Affiliation:
Department of Neurology, Mayo Clinic, Rochester, MN, USA
Alissa M. Butts
Affiliation:
Department of Neurology, Division of Neuropsychology, Medical College of Wisconsin, Madison, WI, USA
Hugo Botha
Affiliation:
Department of Neurology, Mayo Clinic, Rochester, MN, USA
Val J. Lowe
Affiliation:
Department of Neuroradiology, Mayo Clinic, Rochester, MN, USA
Jennifer L. Whitwell
Affiliation:
Department of Neuroradiology, Mayo Clinic, Rochester, MN, USA
Keith A. Josephs
Affiliation:
Department of Neurology, Mayo Clinic, Rochester, MN, USA
Rene L. Utianski*
Affiliation:
Department of Neurology, Mayo Clinic, Rochester, MN, USA
*
*Correspondence and reprint requests to: Rene L. Utianski, PhD, Mayo Clinic, 200 1st Street SW, Rochester, MN, 55904, USA. E-mail: utianski.rene@mayo.edu

Abstract

Objective:

To characterize and compare the neuropsychological profiles of patients with primary progressive apraxia of speech (PPAOS) and apraxia of speech with progressive agrammatic aphasia (AOS-PAA).

Method:

Thirty-nine patients with PPAOS and 49 patients with AOS-PAA underwent formal neurological, speech, language, and neuropsychological evaluations. Cognitive domains assessed included immediate and delayed episodic memory (Wechsler Memory Scale-Third edition; Logical Memory; Visual Reproduction; Rey Auditory Verbal Learning Test), processing speed (Trail Making Test A), executive functioning (Trail Making Test B; Delis-Kaplan Executive Functioning Scale – Sorting), and visuospatial ability (Rey-Osterrieth Complex Figure copy).

Results:

The PPAOS patients were cognitively average or higher in the domains of immediate and delayed episodic memory, processing speed, executive functioning, and visuospatial ability. Patients with AOS-PAA performed more poorly on tests of immediate and delayed episodic memory and executive functioning compared to those with PPAOS. For every 1 unit increase in aphasia severity (e.g. mild to moderate), performance declined by 1/3 to 1/2 a standard deviation depending on cognitive domain. The degree of decline was stronger within the more verbally mediated domains, but was also notable in less verbally mediated domains.

Conclusion:

The study provides neuropsychological evidence further supporting the distinction of PPAOS from primary progressive aphasia and should be used to inform future diagnostic criteria. More immediately, it informs prognostication and treatment planning.

Type
Regular Research
Copyright
Copyright © INS. Published by Cambridge University Press, 2021

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Armstrong, M.J., Litvan, I., Lang, A.E., Bak, T.H., Bhatia, K.P., Borroni, B., … Hallett, M. (2013). Criteria for the diagnosis of corticobasal degeneration. Neurology, 80(5), 496503.CrossRefGoogle Scholar
Bettcher, B.M. & Sturm, V.E. (2014). Neuropsychological assessment of primary progressive aphasia (PPA). Perspectives on Neurophysiology and Neurogenic Speech and Language Disorders, 24(4), 128136.CrossRefGoogle Scholar
Botha, H., Duffy, J.R., Whitwell, J.L., Strand, E.A., Machulda, M.M., Schwarz, C.G., … Josephs, K.A. (2015). Classification and clinicoradiologic features of primary progressive aphasia (PPA) and apraxia of speech. Cortex, 69, 220236. doi: 10.1016/j.cortex.2015.05.013 CrossRefGoogle ScholarPubMed
Botha, H. & Josephs, K.A. (2019). Primary progressive aphasias and apraxia of speech. Continuum: Lifelong Learning in Neurology, 25(1), 101.Google ScholarPubMed
Botha, H., Utianski, R.L., Whitwell, J.L., Duffy, J.R., Clark, H.M., Strand, E.A., … Jones, D.T. (2018). Disrupted functional connectivity in primary progressive apraxia of speech. Neuroimage Clinical, 18, 617629. doi: 10.1016/j.nicl.2018.02.036 CrossRefGoogle ScholarPubMed
Butts, A.M., Machulda, M.M., Duffy, J.R., Strand, E.A., Whitwell, J.L., & Josephs, K.A. (2015). Neuropsychological profiles differ among the three variants of primary progressive aphasia. Journal of the International Neuropsychological Society, 21(6), 429435. doi: 10.1017/S1355617715000399 CrossRefGoogle ScholarPubMed
Darley, F. (1967). Lacunae and research approaches to them. Brain Mechanisms Underlying (pp. 236240). New York, NY: Grune & Stratton.Google Scholar
Darley, F. (1969). Aphasia: Input and output disturbances in speech and language processing. Paper presented to the annual convention of the American Speech and Hearing Association, Chicago [unpublished].Google Scholar
Delis, D.C., Kaplan, E., & Kramer, J.H. (2001). The Delis Kaplan Executive Function System: Examiner’s Manual. San Antonio, TX: The Psychological Corporation.Google Scholar
Deramecourt, V., Lebert, F., Debachy, B., Mackowiak-Cordoliani, M., Bombois, S., Kerdraon, O., … Pasquier, F. (2010). Prediction of pathology in primary progressive language and speech disorders. Neurology, 74(1), 4249.CrossRefGoogle ScholarPubMed
Duffy, J.R. (2006). Apraxia of speech in degenerative neurologic disease. Aphasiology, 20(6), 511527. doi: 10.1080/02687030600597358 CrossRefGoogle Scholar
Duffy, J.R., Hanley, H., Utianski, R., Clark, H., Strand, E., Josephs, K.A., & Whitwell, J.L. (2017). Temporal acoustic measures distinguish primary progressive apraxia of speech from primary progressive aphasia. Brain and Language, 168, 8494. doi: 10.1016/j.bandl.2017.01.012 CrossRefGoogle ScholarPubMed
Duffy, J.R. & Josephs, K.A. (2012). The diagnosis and understanding of apraxia of speech: Why including neurodegenerative etiologies may be important. Journal of Speech, Language, and Hearing Research, 55(5), S1518S1522.CrossRefGoogle ScholarPubMed
Duffy, J.R., Strand, E., & Josephs, K. (2014). Motor speech disorders associated with primary progressive aphasia. Aphasiology, 28(8–9), 10041017.CrossRefGoogle ScholarPubMed
Duffy, J.R., Strand, E.A., Clark, H., Machulda, M., Whitwell, J.L., & Josephs, K.A. (2015). Primary progressive apraxia of speech: clinical features and acoustic and neurologic correlates. American Journal of Speech and Language Pathology, 24(2), 88100. doi: 10.1044/2015_AJSLP-14-0174 CrossRefGoogle ScholarPubMed
Eikelboom, W.S., Janssen, N., Jiskoot, L.C., van den Berg, E., Roelofs, A., & Kessels, R.P. (2018). Episodic and working memory function in Primary Progressive Aphasia: A meta-analysis. Neuroscience & Biobehavioral Reviews, 92, 243254.CrossRefGoogle ScholarPubMed
Gelman, A., & Hill, J. (2006). Data Analysis Using Regression and Multilevel/hierarchical Models. New York, NY: Cambridge University Press.CrossRefGoogle Scholar
Goetz, C.G., Fahn, S., Martinez-Martin, P., Poewe, W., Sampaio, C., Stebbins, G.T., … Dubois, B. (2007). Movement Disorder Society-sponsored revision of the Unified Parkinson’s Disease Rating Scale (MDS-UPDRS): process, format, and clinimetric testing plan. Movement Disorders, 22(1), 4147.CrossRefGoogle ScholarPubMed
Gorno-Tempini, M.L., Hillis, A.E., Weintraub, S., Kertesz, A., Mendez, M., Cappa, S.F., … Grossman, M. (2011). Classification of primary progressive aphasia and its variants. Neurology, 76(11), 10061014. doi: 10.1212/WNL.0b013e31821103e6 CrossRefGoogle ScholarPubMed
Harris, J.M., Saxon, J.A., Jones, M., Snowden, J.S., & Thompson, J.C. (2019). Neuropsychological differentiation of progressive aphasic disorders. Journal of Neuropsychology, 13(2), 214239.CrossRefGoogle ScholarPubMed
Hoglinger, G.U., Respondek, G., Stamelou, M., Kurz, C., Josephs, K.A., Lang, A.E., … Movement Disorder Society-endorsed PSP Study Group (2017). Clinical diagnosis of progressive supranuclear palsy: The movement disorder society criteria. Movement Disorders, 32(6), 853864. doi: 10.1002/mds.26987 CrossRefGoogle ScholarPubMed
Ivnik, R.J., Malec, J.F., Smith, G.E., Tangalos, E.G., & Petersen, R.C. (1996). Neuropsychological tests’ norms above age 55: COWAT, BNT, MAE token, WRAT-R reading, AMNART, STROOP, TMT, and JLO. The Clinical Neuropsychologist, 10(3), 262278.CrossRefGoogle Scholar
Ivnik, R.J., Malec, J.F., Smith, G.E., Tangalos, E.G., Petersen, R.C., Kokmen, E., & Kurland, L.T. (1992). Mayo’s Older Americans Normative Studies: updated AVLT norms for ages 56 to 97. The Clinical Neuropsychologist, 6(S1), 83104.CrossRefGoogle Scholar
Josephs, K.A., Boeve, B.F., Duffy, J.R., Smith, G.E., Knopman, D.S., Parisi, J.E., … Dickson, D.W. (2005). Atypical progressive supranuclear palsy underlying progressive apraxia of speech and nonfluent aphasia. Neurocase, 11(4), 283296. doi: 10.1080/13554790590963004 CrossRefGoogle ScholarPubMed
Josephs, K.A., Duffy, J.R., Strand, E.A., Machulda, M., Senjem, M.L., Lowe, V., … Whitwell, J.L. (2013). Syndromes dominated by apraxia ofspeech show distinct characteristics from agrammatic PPA. Neurology, 81, 337345. doi: 10.1212/WNL.0b013e31829c5ed5 CrossRefGoogle ScholarPubMed
Josephs, K.A., Duffy, J.R., Strand, E.A., Machulda, M.M., Senjem, M.L., Gunter, J.L., … Whitwell, J.L. (2014). The evolution of primary progressive apraxia of speech. Brain, 137(Pt 10), 27832795. doi: 10.1093/brain/awu223 CrossRefGoogle ScholarPubMed
Josephs, K.A., Duffy, J.R., Strand, E.A., Machulda, M.M., Senjem, M.L., Master, A.V., … Whitwell, J.L. (2012). Characterizing a neurodegenerative syndrome: primary progressive apraxia of speech. Brain, 135(Pt 5), 15221536. doi: 10.1093/brain/aws032 CrossRefGoogle ScholarPubMed
Josephs, K.A., Duffy, J.R., Strand, E.A., Whitwell, J.L., Layton, K.F., Parisi, J.E., … Petersen, R.C. (2006). Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Brain, 129(Pt 6), 13851398. doi: 10.1093/brain/awl078 CrossRefGoogle ScholarPubMed
Kertesz, A. (2007). Western Aphasia Battery (Revised). San Antonio, TX: PsychCorp.Google Scholar
Lansing, A.E., Ivnik, R.J., Cullum, C.M., & Randolph, C. (1999). An empirically derived short form of the Boston Naming Test. Archives of Clinical Neuropsychology, 14, 481487.CrossRefGoogle ScholarPubMed
Machulda, M.M., Ivnik, R., Smith, G., Ferman, T.J., Boeve, B.F., Knopman, D., … Tangalos, E.G. (2007). Mayo’s older Americans normative studies: Visual form discrimination and copy trial of the Rey–Osterrieth complex figure. Journal of Clinical and Experimental Neuropsychology, 29(4), 377384.CrossRefGoogle ScholarPubMed
Mesulam, M.M. (2001). Primary progressive aphasia. Annals of Neurology, 49(4), 425432.CrossRefGoogle ScholarPubMed
Mesulam, M.M. (2003). Primary progressive aphasia—a language-based dementia. New England Journal of Medicine, 349(16), 15351542.CrossRefGoogle Scholar
Nasreddine, Z.S., Phillips, N.A., Bédirian, V., Charbonneau, S., Whitehead, V., Collin, I., … Chertkow, H. (2005). The Montreal Cognitive Assessment, MoCA: a brief screening tool for mild cognitive impairment. Journal of the American Geriatrics Society, 53(4), 695699.CrossRefGoogle ScholarPubMed
Ogar, J.M., Dronkers, N.F., Brambati, S.M., Miller, B.L., & Gorno-Tempini, M.L. (2007). Progressive nonfluent aphasia and its characteristic motor speech deficits. Alzheimer Disease & Associated Disorders, 21(4), S23S30.CrossRefGoogle ScholarPubMed
Osterrieth, P. (1944). Test of copying a complex figure: Contribution to the study of perception and memory. Archives de Psychologie, 30, 286356.Google Scholar
Rey, A. (1964). The Clinical Psychological Examination. Paris, France: Presses Universitaires de France.Google Scholar
Spreen, O., & Strauss, E. (1998). Compendium of neuropsychological tests: Administration, norms and commentary (2nd ed.). New York, NY: Oxford University Press.Google Scholar
Strand, E.A., Duffy, J.R., Clark, H.M., & Josephs, K. (2014). The Apraxia of Speech Rating Scale: a tool for diagnosis and description of apraxia of speech. Journal of Communication Disorders, 51, 4350. doi: 10.1016/j.jcomdis.2014.06.008 CrossRefGoogle ScholarPubMed
Tetzloff, K.A., Duffy, J.R., Clark, H.M., Utianski, R.L., Strand, E.A., Machulda, M.M., … Senjem, M.L. (2019). Progressive agrammatic aphasia without apraxia of speech as a distinct syndrome. Brain, 142(8), 24662482.CrossRefGoogle ScholarPubMed
Tetzloff, K.A., Duffy, J.R., Strand, E.A., Machulda, M.M., Boland, S.M., Utianski, R.L., … Josephs, K.A. (2018). Clinical and imaging progression over 10 years in a patient with primary progressive apraxia of speech and autopsy-confirmed corticobasal degeneration. Neurocase, 24(2), 111120.CrossRefGoogle Scholar
Tetzloff, K.A., Utianski, R.L., Duffy, J.R., Clark, H.M., Strand, E.A., Josephs, K.A., & Whitwell, J.L. (2018). Quantitative Analysis of Agrammatism in Agrammatic Primary Progressive Aphasia and Dominant Apraxia of Speech. Journal of Speech, Language, and Hearing Research, 61(9), 23372346. doi:doi: 10.1044/2018_JSLHR-L-17-0474 CrossRefGoogle ScholarPubMed
Utianski, R.L., Duffy, J.R., Clark, H.M., Strand, E.A., Boland, S.M., Machulda, M.M., … Josephs, K.A. (2018). Clinical Progression in Four Cases of Primary Progressive Apraxia of Speech. American Journal of Speech-Language Pathology, 27(4), 13031318. doi: 10.1044/2018_ajslp-17-0227 CrossRefGoogle ScholarPubMed
Utianski, R.L., Duffy, J.R., Clark, H.M., Strand, E.A., Botha, H., Schwarz, C.G., … Josephs, K.A. (2018). Prosodic and phonetic subtypes of primary progressive apraxia of speech. Brain & Language, 184, 5465. doi: 10.1016/j.bandl.2018.06.004 CrossRefGoogle ScholarPubMed
Utianski, R.L., Whitwell, J.L., Schwarz, C.G., Duffy, J.R., Botha, H., Clark, H.M., … Petersen, R.C. (2018). Tau uptake in agrammatic primary progressive aphasia with and without apraxia of speech. European Journal of Neurology, 25(11), 13521357.CrossRefGoogle ScholarPubMed
Utianski, R.L., Whitwell, J.L., Schwarz, C.G., Senjem, M.L., Tosakulwong, N., Duffy, J.R., … Josephs, K.A. (2018). Tau-PET imaging with [18F]AV-1451 in primary progressive apraxia of speech. Cortex, 99, 358374. doi: 10.1016/j.cortex.2017.12.021 CrossRefGoogle Scholar
Vöglein, J., Kostova, I., Arzberger, T., Röber, S., Schmitz, P., Simons, M., … & Levin, J. (2021). First symptom guides diagnosis and prognosis in neurodegenerative diseases – A retrospective study of autopsy proven cases. European Journal of Neurology. doi: 10.1111/ene.14800 CrossRefGoogle Scholar
Warrington, E.K., & James, M. (1991). The Visual Object and Space Perception Battery. Bury St Edmunds, England: Thames Valley Test Company.Google Scholar
Watson, C.L., Possin, K., Allen, I.E., Hubbard, H.I., Meyer, M., Welch, A.E., … Miller, Z. (2018). Visuospatial functioning in the primary progressive aphasias. Journal of the International Neuropsychological Society, 24(3), 259268.CrossRefGoogle ScholarPubMed
Wechsler, D. (1997). Wechsler Memory Scale (3rd ed.). San Antonio, TX: Psychological Corporation.Google Scholar
Whitwell, J.L., Duffy, J.R., Strand, E.A., Xia, R., Mandrekar, J., Machulda, M.M., … Josephs, K.A. (2013). Distinct regional anatomic and functional correlates of neurodegenerative apraxia of speech and aphasia: an MRI and FDG-PET study. Brain Lang, 125(3), 245252. doi: 10.1016/j.bandl.2013.02.005 CrossRefGoogle ScholarPubMed
Whitwell, J.L., Martin, P., Duffy, J.R., Clark, H.M., Utianski, R.L., Botha, H., … Josephs, K.A. (2020). Survival analysis in primary progressive apraxia of speech and agrammatic aphasia. Neurology: Clinical Practice. doi: 10.1212/CPJ.0000000000000919 CrossRefGoogle Scholar
Whitwell, J.L., Weigand, S.D., Duffy, J.R., Clark, H.M., Strand, E.A., Machulda, M.M., … Josephs, K.A. (2017). Predicting clinical decline in progressive agrammatic aphasia and apraxia of speech. Neurology, 89(22), 22712279. doi: 10.1212/wnl.0000000000004685 CrossRefGoogle ScholarPubMed

Send article to Kindle

To send this article to your Kindle, first ensure no-reply@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about sending to your Kindle. Find out more about sending to your Kindle.

Note you can select to send to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be sent to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Neuropsychological Profiles of Patients with Progressive Apraxia of Speech and Aphasia
Available formats
×

Send article to Dropbox

To send this article to your Dropbox account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your <service> account. Find out more about sending content to Dropbox.

Neuropsychological Profiles of Patients with Progressive Apraxia of Speech and Aphasia
Available formats
×

Send article to Google Drive

To send this article to your Google Drive account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your <service> account. Find out more about sending content to Google Drive.

Neuropsychological Profiles of Patients with Progressive Apraxia of Speech and Aphasia
Available formats
×
×

Reply to: Submit a response

Please enter your response.

Your details

Please enter a valid email address.

Conflicting interests

Do you have any conflicting interests? *