Profile of linguistic and cognitive deficits in persons with a semantic variant of a primary progressive aphasia
Keywords:
primary progressive aphasia, semantic dementia, semantic variant of primary progressive aphasia, language disorders
Abstract
Primary progressive aphasia (PPA) includes a group of neurodegenerative disorders that are characterized by progressive deterioration of language functions, while other cognitive functions, at least at the onset of the disease, are relatively spared. There are three basic subtypes of PPA: the nonfluent progressive aphasia (nvPPA), the semantic variant of a primary progressive aphasia (svPPA), and the logopenic progressive aphasia (lvPPA). The semantic variant of a PPA can also be found in the literature under the term of semantic dementia. It is clinically manifested by progressive deterioration of semantic knowledge, fluent aphasia, impaired naming and comprehension, prosopagnosia and surface dyslexia and dysgraphia (in languages with irregular orthography). As the disease progresses, other cognitive changes can be observed. The main cause of the disorder is progressive bilateral atrophy of the anterior temporal lobes, which is more manifested in the left hemisphere. The literature is modest in terms of the use of specific treatment methods in the rehabilitation of these patients. Since speech and language disorders are the most conspicuous symptom, at least at the beginning of the disease, the role of speech therapists in the assessment and restitution of speech-language and communication skills is also indisputable.
References
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34 - Rosen H, Allison S, Ogar J, Amici S, Rose K, Dronkers N et al. Behavioral features in semantic dementia vs other forms of progressive aphasias. Neurology. 2006;67(10):1752-1756.
35 - Joubert S, Felician O, Barbeau E, Ranjeva J, Christophe M, Didic M et al. The right temporal lobe variant of frontotemporal dementia. Journal of Neurology. 2006;253(11):1447-1458.
36 - Fittipaldi S, Ibanez A, Baez S, Manes F, Sedeno L, Garcia A. More than words: Social cognition across variants of primary progressive aphasia. Neuroscience & Biobehavioral Reviews. 2019;100:263-284.
37 - Bejanin A, Chételat G, Laisney M, Pélerin A, Landeau B, Merck C et al. Distinct neural substrates of affective and cognitive theory of mind impairment in semantic dementia. Social Neuroscience. 2016;12(3):287-302.
38 - Patterson K, Nestor P, Rogers T. Where do you know what you know? The representation of semantic knowledge in the human brain. Nature Reviews Neuroscience. 2007;8(12):976-987.
39 - Visser M, Jefferies E, Lambon Ralph MA. Semantic processing in the anterior temporal lobes: a meta-analysis of the functional neuroimaging literature. Journal of cognitive neuroscience. 2010 Jun;22(6):1083-94.
40 - McClelland J, Rogers T. The parallel distributed processing approach to semantic cognition. Nature Reviews Neuroscience. 2003;4(4):310-322.
41 - Plaut D, McClelland J, Seidenberg M, Patterson K. Understanding normal and impaired word reading: Computational principles in quasi-regular domains. Psychological Review. 1996;103(1):56-115.
42 - Teichmann M, Sanches C, Moreau J, Ferrieux S, Nogues M, Dubois B et al. Does surface dyslexia/dysgraphia relate to semantic deficits in the semantic variant of primary progressive aphasia?. Neuropsychologia. 2019;135:107241.
43 - Grasso S, Henry M. Assessment of Individuals with Primary Progressive Aphasia. Seminars in Speech and Language. 2018;39(03):231-241.
44 - Goodglass H, Kaplan E, Barresi B. BDAE-3: Boston Diagnostic Aphasia Examination–Third Edition. Philadelphia, PA: Lippincott Williams & Wilkins; 2001.
45 - Kertesz A. WAB-R: Western aphasia battery-revised. PsychCorp; 2007.
46 - Sapolsky D, Domoto-Reilly K, Dickerson B. Use of the Progressive Aphasia Severity Scale (PASS) in monitoring speech and language status in PPA. Aphasiology. 2014;28(8-9):993-1003.
47 - Leyton C, Villemagne V, Savage S, Pike K, Ballard K, Piguet O et al. Subtypes of progressive aphasia: application of the international consensus criteria and validation using β-amyloid imaging. Brain. 2011;134(10):3030-3043.
48 - Morris J. The Clinical Dementia Rating (CDR): Current version and scoring rules. Neurology. 1993;43(11):2412-2412.
49 - Graham KS, Patterson K, Pratt KH, Hodges JR. Can repeated exposure to" forgotten" vocabulary help alleviate word-finding difficulties in semantic dementia? An illustrative case study. Neuropsychological Rehabilitation. 2001 Jul 1;11(3-4):429-54.
50 - Henry M, Beeson P, Rapcsak S. Treatment for Anomia in Semantic Dementia. Seminars in Speech and Language. 2008;29(1):60-70.
51 - Tippett D, Hillis A, Tsapkini K. Treatment of Primary Progressive Aphasia. Current Treatment Options in Neurology. 2015;17(8).
52 - Bier N, Macoir J, Gagnon L, Van der Linden M, Louveaux S, Desrosiers J. Known, lost, and recovered: Efficacy of formal‐semantic therapy and spaced retrieval method in a case of semantic dementia. Aphasiology. 2009;23(2):210-235.
53 - Jokel R, Graham N, Rochon E, Leonard C. Word retrieval therapies in primary progressive aphasia. Aphasiology. 2014;28(8-9):1038-1068.
54 - Milano N, Williamson J, Heilman K. Improved verbal learning in the semantic variant of primary progressive aphasia when using semantic cues. Neurocase. 2014;21(3):345-350.
2 - Grossman M. Primary progressive aphasia: clinicopathological correlations. Nature Reviews Neurology. 2010;6(2):88-97.
3 - Vuković M. Neurodegenerativni poremećaji govora i jezika. Beograd: Univerzitet u Beogradu, Fakultet za specijalnu edukaciju i rehabilitaciju; 2019.
4 - Pavlović D, Pavlović A. Više kortikalne funkcije: neuropsihologija i neurologija ponašanja. Beograd: Orion Art; 2016
5 - Vuković M. Primarna progresivna afazija. Inovativni pristupi u logopediji. Beograd: Udruženje logopeda Srbije; 2017. p. 29-36.
6 - Mesulam M. Primary progressive aphasia. Annals of Neurology. 2001;49(4):425-432.
7 - Marshall C, Hardy C, Volkmer A, Russell L, Bond R, Fletcher P et al. Primary progressive aphasia: a clinical approach. Journal of Neurology. 2018;265(6):1474-1490.
8 - Hodges J, Mitchell J, Dawson K, Spillantini M, Xuereb J, McMonagle P et al. Semantic dementia: demography, familial factors and survival in a consecutive series of 100 cases. Brain. 2009;133(1):300-306.
9 - Harciarek M, Sitek E, Kertesz A. The patterns of progression in primary progressive aphasia—Implications for assessment and management. Aphasiology. 2014;28(8-9):964-980.
10 - Johnson J, Diehl J, Mendez M, Neuhaus J, Shapira J, Forman M et al. Frontotemporal Lobar Degeneration. Archives of Neurology. 2005;62(6).
11 - Montembeault M, Brambati S, Gorno-Tempini M, Migliaccio R. Clinical, Anatomical, and Pathological Features in the Three Variants of Primary Progressive Aphasia: A Review. Frontiers in Neurology. 2018;9.
12 - Rohrer JD, Geser F, Zhou J, Gennatas ED, Sidhu M, Trojanowski JQ, Dearmond SJ, Miller BL, Seeley WW. TDP-43 subtypes are associated with distinct atrophy patterns in frontotemporal dementia. Neurology. 2010 Dec 14;75(24):2204-11
13 - Gorno-Tempini M, Hillis A, Weintraub S, Kertesz A, Mendez M, Cappa S et al. Classification of primary progressive aphasia and its variants. Neurology. 2011;76(11):1006-1014.
14 - Collins J, Montal V, Hochberg D, Quimby M, Mandelli M, Makris N et al. Focal temporal pole atrophy and network degeneration in semantic variant primary progressive aphasia. Brain. 2016;140(2):457-471.
15 - Gorno-Tempini M, Dronkers N, Rankin K, Ogar J, Phengrasamy L, Rosen H et al. Cognition and anatomy in three variants of primary progressive aphasia. Annals of Neurology. 2004;55(3):335-346.
16 - Woollacott I, Rohrer J. The clinical spectrum of sporadic and familial forms of frontotemporal dementia. Journal of Neurochemistry. 2016;138:6-31.
17 - Bouchard L, Wilson M, Laforce R, Duchesne S. White Matter Damage in the Semantic Variant of Primary Progressive Aphasia. Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques. 2019;46(04):373-382.
18 - Pavlović D. Frontotemporalne demencije. Beograd: Orion Art; 2019.
19 - Kamminga J, Kumfor F, Burrell J, Piguet O, Hodges J, Irish M. Differentiating between right-lateralised semantic dementia and behavioural-variant frontotemporal dementia: an examination of clinical characteristics and emotion processing. Journal of Neurology, Neurosurgery & Psychiatry. 2014;86(10):1082-1088.
20 - Chapleau M, Aldebert J, Montembeault M, Brambati S. Atrophy in Alzheimer’s Disease and Semantic Dementia: An ALE Meta-Analysis of Voxel-Based Morphometry Studies. Journal of Alzheimer's Disease. 2016;54(3):941-955.
21 - Chapleau M, Montembeault M, Boukadi M, Bedetti C, Laforce R, Wilson M et al. The role of the hippocampus in the semantic variant of primary progressive aphasia: A resting‐state fcMRI study. Hippocampus. 2019;29(11):1127-1132.
22 - Vuković M. Afaziologija. 4. izdanje. Beograd: Udruženje logopeda Srbije; 2016.
23 - Volkmer A, Rogalski E, Henry M, Taylor-Rubin C, Ruggero L, Khayum R et al. Speech and language therapy approaches to managing primary progressive aphasia. Practical Neurology. 2019;:practneurol-2018-001921.
24 - Pavlović D. Demencije – dijagnoza, terapija i nega. Beograd: Orion Art; 2012.
25 - Mesulam M, Rogalski E, Wieneke C, Hurley R, Geula C, Bigio E et al. Primary progressive aphasia and the evolving neurology of the language network. Nature Reviews Neurology. 2014;10(10):554-569.
26 - Leyton C, Savage S, Irish M, Schubert S, Piguet O, Ballard K et al. Verbal Repetition in Primary Progressive Aphasia and Alzheimer's Disease. Journal of Alzheimer's Disease. 2014;41(2):575-585.
27 - Lima BS, Levine B, Graham NL, Leonard C, Tang-Wai D, Black S, Rochon E. Impaired coherence for semantic but not episodic autobiographical memory in semantic variant primary progressive aphasia. Cortex. 2020 Feb 1;123:72-85.
28 - Jarrold W, Peintener B, Wilkins D, Vergryi D, Richey C, Gorno-Tempini M. Aided diagnosis of dementia type through computer-based analysis of spontaneous speech. Baltimore, Maryland: Association for Computational Linguistics; 2014. p. 27-37.
29 - Ellajosyula R, Narayanan J, Patterson K. Striking loss of second language in bilingual patients with semantic dementia. Journal of Neurology. 2019;:1-10.
30 - Iaccarino L, Crespi C, Della Rosa P, Catricalà E, Guidi L, Marcone A et al. The Semantic Variant of Primary Progressive Aphasia: Clinical and Neuroimaging Evidence in Single Subjects. PLOS ONE. 2015;10(3):e0120197.
31 - Gordon E, Rohrer J, Kim L, Omar R, Rossor M, Fox N et al. Measuring disease progression in frontotemporal lobar degeneration: A clinical and MRI study. Neurology. 2010;74(8):666-673.
32 - Fletcher P, Warren J. Semantic Dementia: a specific network-opathy. Journal of Molecular Neuroscience. 2011;45(3):629-636.
33 - Snowden J, Adams J, Harris J, Thompson J, Rollinson S, Richardson A et al. Distinct clinical and pathological phenotypes in frontotemporal dementia associated with MAPT, PGRN and C9orf72 mutations. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. 2015;16(7-8):497-505.
34 - Rosen H, Allison S, Ogar J, Amici S, Rose K, Dronkers N et al. Behavioral features in semantic dementia vs other forms of progressive aphasias. Neurology. 2006;67(10):1752-1756.
35 - Joubert S, Felician O, Barbeau E, Ranjeva J, Christophe M, Didic M et al. The right temporal lobe variant of frontotemporal dementia. Journal of Neurology. 2006;253(11):1447-1458.
36 - Fittipaldi S, Ibanez A, Baez S, Manes F, Sedeno L, Garcia A. More than words: Social cognition across variants of primary progressive aphasia. Neuroscience & Biobehavioral Reviews. 2019;100:263-284.
37 - Bejanin A, Chételat G, Laisney M, Pélerin A, Landeau B, Merck C et al. Distinct neural substrates of affective and cognitive theory of mind impairment in semantic dementia. Social Neuroscience. 2016;12(3):287-302.
38 - Patterson K, Nestor P, Rogers T. Where do you know what you know? The representation of semantic knowledge in the human brain. Nature Reviews Neuroscience. 2007;8(12):976-987.
39 - Visser M, Jefferies E, Lambon Ralph MA. Semantic processing in the anterior temporal lobes: a meta-analysis of the functional neuroimaging literature. Journal of cognitive neuroscience. 2010 Jun;22(6):1083-94.
40 - McClelland J, Rogers T. The parallel distributed processing approach to semantic cognition. Nature Reviews Neuroscience. 2003;4(4):310-322.
41 - Plaut D, McClelland J, Seidenberg M, Patterson K. Understanding normal and impaired word reading: Computational principles in quasi-regular domains. Psychological Review. 1996;103(1):56-115.
42 - Teichmann M, Sanches C, Moreau J, Ferrieux S, Nogues M, Dubois B et al. Does surface dyslexia/dysgraphia relate to semantic deficits in the semantic variant of primary progressive aphasia?. Neuropsychologia. 2019;135:107241.
43 - Grasso S, Henry M. Assessment of Individuals with Primary Progressive Aphasia. Seminars in Speech and Language. 2018;39(03):231-241.
44 - Goodglass H, Kaplan E, Barresi B. BDAE-3: Boston Diagnostic Aphasia Examination–Third Edition. Philadelphia, PA: Lippincott Williams & Wilkins; 2001.
45 - Kertesz A. WAB-R: Western aphasia battery-revised. PsychCorp; 2007.
46 - Sapolsky D, Domoto-Reilly K, Dickerson B. Use of the Progressive Aphasia Severity Scale (PASS) in monitoring speech and language status in PPA. Aphasiology. 2014;28(8-9):993-1003.
47 - Leyton C, Villemagne V, Savage S, Pike K, Ballard K, Piguet O et al. Subtypes of progressive aphasia: application of the international consensus criteria and validation using β-amyloid imaging. Brain. 2011;134(10):3030-3043.
48 - Morris J. The Clinical Dementia Rating (CDR): Current version and scoring rules. Neurology. 1993;43(11):2412-2412.
49 - Graham KS, Patterson K, Pratt KH, Hodges JR. Can repeated exposure to" forgotten" vocabulary help alleviate word-finding difficulties in semantic dementia? An illustrative case study. Neuropsychological Rehabilitation. 2001 Jul 1;11(3-4):429-54.
50 - Henry M, Beeson P, Rapcsak S. Treatment for Anomia in Semantic Dementia. Seminars in Speech and Language. 2008;29(1):60-70.
51 - Tippett D, Hillis A, Tsapkini K. Treatment of Primary Progressive Aphasia. Current Treatment Options in Neurology. 2015;17(8).
52 - Bier N, Macoir J, Gagnon L, Van der Linden M, Louveaux S, Desrosiers J. Known, lost, and recovered: Efficacy of formal‐semantic therapy and spaced retrieval method in a case of semantic dementia. Aphasiology. 2009;23(2):210-235.
53 - Jokel R, Graham N, Rochon E, Leonard C. Word retrieval therapies in primary progressive aphasia. Aphasiology. 2014;28(8-9):1038-1068.
54 - Milano N, Williamson J, Heilman K. Improved verbal learning in the semantic variant of primary progressive aphasia when using semantic cues. Neurocase. 2014;21(3):345-350.