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Published in: Current Neurology and Neuroscience Reports 6/2010

01-11-2010

The New Classification of Primary Progressive Aphasia into Semantic, Logopenic, or Nonfluent/Agrammatic Variants

Authors: Michael F. Bonner, Sharon Ash, Murray Grossman

Published in: Current Neurology and Neuroscience Reports | Issue 6/2010

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Abstract

Primary progressive aphasia (PPA), typically resulting from a neurodegenerative disease such as frontotemporal lobar degeneration or Alzheimer’s disease, is characterized by a progressive loss of specific language functions with relative sparing of other cognitive domains. Three variants of PPA are now recognized: semantic variant, logopenic variant, and nonfluent/agrammatic variant. We discuss recent work characterizing the neurolinguistic, neuropsychological, imaging and pathologic profiles associated with these variants. Improved reliability of diagnoses will be increasingly important as trials for etiology-specific treatments become available. We also discuss the implications of these syndromes for theories of language function.
Literature
2.
go back to reference Mesulam MM: Primary progressive aphasia--a language-based dementia. N Engl J Med 2003, 349:1535–1542.CrossRefPubMed Mesulam MM: Primary progressive aphasia--a language-based dementia. N Engl J Med 2003, 349:1535–1542.CrossRefPubMed
3.
go back to reference Gorno-Tempini ML, Dronkers NF, Rankin KP, et al.: Cognition and anatomy in three variants of primary progressive aphasia. Ann Neurol 2004, 55:335–346.CrossRefPubMed Gorno-Tempini ML, Dronkers NF, Rankin KP, et al.: Cognition and anatomy in three variants of primary progressive aphasia. Ann Neurol 2004, 55:335–346.CrossRefPubMed
4.
go back to reference • Grossman M: Primary progressive aphasia: clinicopathological correlations. Nat Rev Neurol 2010, 6:88–97. This paper summarizes the autopsy-confirmed pathology results from 145 PPA patients across seven institutions. The current findings for neuropsychological, neuroimaging, genetic, and cerebrospinal fluid biomarkers of the PPA variants are reviewed.CrossRefPubMed • Grossman M: Primary progressive aphasia: clinicopathological correlations. Nat Rev Neurol 2010, 6:88–97. This paper summarizes the autopsy-confirmed pathology results from 145 PPA patients across seven institutions. The current findings for neuropsychological, neuroimaging, genetic, and cerebrospinal fluid biomarkers of the PPA variants are reviewed.CrossRefPubMed
5.
go back to reference Mesulam M, Wieneke C, Rogalski E, et al.: Quantitative template for subtyping primary progressive aphasia. Arch Neurol 2009, 66:1545–1551.CrossRefPubMed Mesulam M, Wieneke C, Rogalski E, et al.: Quantitative template for subtyping primary progressive aphasia. Arch Neurol 2009, 66:1545–1551.CrossRefPubMed
6.
go back to reference Hu WT, McMillan C, Libon D, et al.: Multi-modal predictors for Alzheimer’s disease in non-fluent primary progressive aphasia. Neurology 2010, 75:595–602.CrossRefPubMed Hu WT, McMillan C, Libon D, et al.: Multi-modal predictors for Alzheimer’s disease in non-fluent primary progressive aphasia. Neurology 2010, 75:595–602.CrossRefPubMed
7.
go back to reference • Hodges JR, Patterson K: Semantic dementia: a unique clinicopathological syndrome. Lancet Neurol 2007, 6:1004–1014. This paper provides an overview of SD. Clinical and pathologic features are summarized. The authors discuss neuropsychological findings and considerations in choosing testing methods for these patients.CrossRefPubMed • Hodges JR, Patterson K: Semantic dementia: a unique clinicopathological syndrome. Lancet Neurol 2007, 6:1004–1014. This paper provides an overview of SD. Clinical and pathologic features are summarized. The authors discuss neuropsychological findings and considerations in choosing testing methods for these patients.CrossRefPubMed
8.
go back to reference Meteyard L, Patterson K: The relation between content and structure in language production: an analysis of speech errors in semantic dementia. Brain Lang 2009, 110:121–134.CrossRefPubMed Meteyard L, Patterson K: The relation between content and structure in language production: an analysis of speech errors in semantic dementia. Brain Lang 2009, 110:121–134.CrossRefPubMed
9.
go back to reference Kertesz A, Jesso S, Harciarek M, et al.: What is semantic dementia?: a cohort study of diagnostic features and clinical boundaries. Arch Neurol 2010, 67:483–489.CrossRefPubMed Kertesz A, Jesso S, Harciarek M, et al.: What is semantic dementia?: a cohort study of diagnostic features and clinical boundaries. Arch Neurol 2010, 67:483–489.CrossRefPubMed
10.
go back to reference Halpern C, Glosser G, Clark R, et al.: Dissociation of numbers and objects in corticobasal degeneration and semantic dementia. Neurology 2004, 62:1163–1169.PubMed Halpern C, Glosser G, Clark R, et al.: Dissociation of numbers and objects in corticobasal degeneration and semantic dementia. Neurology 2004, 62:1163–1169.PubMed
11.
go back to reference Mesulam M, Rogalski E, Wieneke C, et al.: Neurology of anomia in the semantic variant of primary progressive aphasia. Brain 2009, 132(Pt 9):2553–2565.CrossRefPubMed Mesulam M, Rogalski E, Wieneke C, et al.: Neurology of anomia in the semantic variant of primary progressive aphasia. Brain 2009, 132(Pt 9):2553–2565.CrossRefPubMed
12.
go back to reference Adlam AL, Patterson K, Rogers TT, et al.: Semantic dementia and fluent primary progressive aphasia: two sides of the same coin? Brain 2006, 129(Pt 11):3066–3080.CrossRefPubMed Adlam AL, Patterson K, Rogers TT, et al.: Semantic dementia and fluent primary progressive aphasia: two sides of the same coin? Brain 2006, 129(Pt 11):3066–3080.CrossRefPubMed
13.
go back to reference Knibb JA, Xuereb JH, Patterson K, et al.: Clinical and pathological characterization of progressive aphasia. Ann Neurol 2006, 59:156–165.CrossRefPubMed Knibb JA, Xuereb JH, Patterson K, et al.: Clinical and pathological characterization of progressive aphasia. Ann Neurol 2006, 59:156–165.CrossRefPubMed
14.
go back to reference Patterson K, Nestor PJ, Rogers TT: Where do you know what you know? The representation of semantic knowledge in the human brain. Nat Rev Neurosci 2007, 8:976–987.CrossRefPubMed Patterson K, Nestor PJ, Rogers TT: Where do you know what you know? The representation of semantic knowledge in the human brain. Nat Rev Neurosci 2007, 8:976–987.CrossRefPubMed
15.
go back to reference Weinstein J, Koenig P, Gunawardena D, et al.: Preserved musical semantic memory in semantic dementia. Arch Neurol 2010 (in press). Weinstein J, Koenig P, Gunawardena D, et al.: Preserved musical semantic memory in semantic dementia. Arch Neurol 2010 (in press).
16.
go back to reference Hailstone JC, Omar R, Warren JD: Relatively preserved knowledge of music in semantic dementia. J Neurol Neurosurg Psychiatry 2009, 80:808–809.CrossRefPubMed Hailstone JC, Omar R, Warren JD: Relatively preserved knowledge of music in semantic dementia. J Neurol Neurosurg Psychiatry 2009, 80:808–809.CrossRefPubMed
17.
go back to reference Yi HA, Moore P, Grossman M: Reversal of the concreteness effect for verbs in semantic dementia. Neuropsychology 2007, 21:9–19.CrossRefPubMed Yi HA, Moore P, Grossman M: Reversal of the concreteness effect for verbs in semantic dementia. Neuropsychology 2007, 21:9–19.CrossRefPubMed
18.
go back to reference • Bonner MF, Vesely L, Price C, et al.: Reversal of the concreteness effect in semantic dementia. Cogn Neuropsychol 2009, 26:568–579. This paper examines the proposed deficit in visual semantic features in SD. The authors find that atrophy in ventral temporal cortex correlates with a deficit in concrete word knowledge.CrossRefPubMed • Bonner MF, Vesely L, Price C, et al.: Reversal of the concreteness effect in semantic dementia. Cogn Neuropsychol 2009, 26:568–579. This paper examines the proposed deficit in visual semantic features in SD. The authors find that atrophy in ventral temporal cortex correlates with a deficit in concrete word knowledge.CrossRefPubMed
19.
20.
go back to reference Jefferies E, Patterson K, Jones RW, et al.: Comprehension of concrete and abstract words in semantic dementia. Neuropsychology 2009, 23:492–499.CrossRefPubMed Jefferies E, Patterson K, Jones RW, et al.: Comprehension of concrete and abstract words in semantic dementia. Neuropsychology 2009, 23:492–499.CrossRefPubMed
21.
go back to reference Rohrer JD, Warren JD, Modat M, et al.: Patterns of cortical thinning in the language variants of frontotemporal lobar degeneration. Neurology 2009, 72:1562–1569.CrossRefPubMed Rohrer JD, Warren JD, Modat M, et al.: Patterns of cortical thinning in the language variants of frontotemporal lobar degeneration. Neurology 2009, 72:1562–1569.CrossRefPubMed
22.
go back to reference Avants B, Anderson C, Grossman M, et al.: Spatiotemporal normalization for longitudinal analysis of gray matter atrophy in frontotemporal dementia. Med Image Comput Comput Assist Interv 2007, 10(Pt 2):303–310.PubMed Avants B, Anderson C, Grossman M, et al.: Spatiotemporal normalization for longitudinal analysis of gray matter atrophy in frontotemporal dementia. Med Image Comput Comput Assist Interv 2007, 10(Pt 2):303–310.PubMed
23.
go back to reference Rohrer JD, McNaught E, Foster J, et al.: Tracking progression in frontotemporal lobar degeneration: serial MRI in semantic dementia. Neurology 2008, 71:1445–1451.CrossRefPubMed Rohrer JD, McNaught E, Foster J, et al.: Tracking progression in frontotemporal lobar degeneration: serial MRI in semantic dementia. Neurology 2008, 71:1445–1451.CrossRefPubMed
24.
go back to reference Duda JT, Avants BB, Asmuth JC, et al.: A fiber tractography based examination of neurodegeneration on language-network neuroanatomy. Med Image Comput Comput Assist Interv 2008, 12:191–198. Duda JT, Avants BB, Asmuth JC, et al.: A fiber tractography based examination of neurodegeneration on language-network neuroanatomy. Med Image Comput Comput Assist Interv 2008, 12:191–198.
25.
go back to reference Agosta F, Henry RG, Migliaccio R, et al.: Language networks in semantic dementia. Brain 2010, 133:286–299.CrossRefPubMed Agosta F, Henry RG, Migliaccio R, et al.: Language networks in semantic dementia. Brain 2010, 133:286–299.CrossRefPubMed
26.
go back to reference Josephs KA, Whitwell JL, Duffy JR, et al.: Progressive aphasia secondary to Alzheimer disease vs FTLD pathology. Neurology 2008, 70:25–34.CrossRefPubMed Josephs KA, Whitwell JL, Duffy JR, et al.: Progressive aphasia secondary to Alzheimer disease vs FTLD pathology. Neurology 2008, 70:25–34.CrossRefPubMed
27.
go back to reference Grossman M, Libon DJ, Forman MS, et al.: Distinct antemortem profiles in patients with pathologically defined frontotemporal dementia. Arch Neurol 2007, 64:1601–1609.CrossRefPubMed Grossman M, Libon DJ, Forman MS, et al.: Distinct antemortem profiles in patients with pathologically defined frontotemporal dementia. Arch Neurol 2007, 64:1601–1609.CrossRefPubMed
28.
go back to reference •• Gorno-Tempini ML, Brambati SM, Ginex V, et al.: The logopenic/phonological variant of primary progressive aphasia. Neurology 2008, 71:1227–1234. This paper characterizes the language impairments and neuroimaging data in six lvPPA patients. This is one of only a handful of studies characterizing this patient group. The results are discussed in light of the proposed phonological-loop deficit for lvPPA.CrossRefPubMed •• Gorno-Tempini ML, Brambati SM, Ginex V, et al.: The logopenic/phonological variant of primary progressive aphasia. Neurology 2008, 71:1227–1234. This paper characterizes the language impairments and neuroimaging data in six lvPPA patients. This is one of only a handful of studies characterizing this patient group. The results are discussed in light of the proposed phonological-loop deficit for lvPPA.CrossRefPubMed
29.
go back to reference Kertesz A, Davidson W, McCabe P: Primary progressive aphasia: diagnosis, varieties, evolution. J Int Neuropsychol Soc 2003, 9:710–719.CrossRefPubMed Kertesz A, Davidson W, McCabe P: Primary progressive aphasia: diagnosis, varieties, evolution. J Int Neuropsychol Soc 2003, 9:710–719.CrossRefPubMed
30.
go back to reference Sonty SP, Mesulam M, Thompson CK, et al.: Primary progressive aphasia: PPA and the language network. Ann Neurol 2003, 53:35–49.CrossRefPubMed Sonty SP, Mesulam M, Thompson CK, et al.: Primary progressive aphasia: PPA and the language network. Ann Neurol 2003, 53:35–49.CrossRefPubMed
31.
go back to reference Galton CJ, Patterson K, Xuereb JH, et al.: Atypical and typical presentations of Alzheimer’s disease: a clinical, neuropsychological, neuroimaging and pathological study of 13 cases. Brain 2000, 123:484–498.CrossRefPubMed Galton CJ, Patterson K, Xuereb JH, et al.: Atypical and typical presentations of Alzheimer’s disease: a clinical, neuropsychological, neuroimaging and pathological study of 13 cases. Brain 2000, 123:484–498.CrossRefPubMed
32.
go back to reference Mendez MF, Clark DG, Shapira JS, et al.: Speech and language in progressive nonfluent aphasia compared with early Alzheimer’s disease. Neurology 2003, 61:1108–1113.PubMed Mendez MF, Clark DG, Shapira JS, et al.: Speech and language in progressive nonfluent aphasia compared with early Alzheimer’s disease. Neurology 2003, 61:1108–1113.PubMed
33.
go back to reference Rohrer JD, Ridgway GR, Crutch SJ, et al.: Progressive logopenic/phonological aphasia: erosion of the language network. Neuroimage 2010, 49:984–993.CrossRefPubMed Rohrer JD, Ridgway GR, Crutch SJ, et al.: Progressive logopenic/phonological aphasia: erosion of the language network. Neuroimage 2010, 49:984–993.CrossRefPubMed
34.
go back to reference Baldo JV, Dronkers NF: The role of inferior parietal and inferior frontal cortex in working memory. Neuropsychology 2006, 20:529–538.CrossRefPubMed Baldo JV, Dronkers NF: The role of inferior parietal and inferior frontal cortex in working memory. Neuropsychology 2006, 20:529–538.CrossRefPubMed
35.
go back to reference Grossman M, Mega M, Cummings J, et al.: The aphasias and related disturbances. In Clinical Neurology. Edited by Baker AB, Joynt RJ. Philadelphia: Lippincott Williams and Wilkins; 2004:1–83. Grossman M, Mega M, Cummings J, et al.: The aphasias and related disturbances. In Clinical Neurology. Edited by Baker AB, Joynt RJ. Philadelphia: Lippincott Williams and Wilkins; 2004:1–83.
37.
go back to reference Knibb JA, Woollams AM, Hodges JR, et al.: Making sense of progressive non-fluent aphasia: an analysis of conversational speech. Brain 2009, 132(Pt 10):2734–2746.CrossRefPubMed Knibb JA, Woollams AM, Hodges JR, et al.: Making sense of progressive non-fluent aphasia: an analysis of conversational speech. Brain 2009, 132(Pt 10):2734–2746.CrossRefPubMed
38.
go back to reference •• Ash S, McMillan C, Gunawardena D, et al.: Speech errors in progressive non-fluent aphasia. Brain Lang 2010, 113:13–20. This paper provides a detailed, quantitative study of speech sound errors in the connected speech of navPPA patients, with linguistic, neuropsychological, and neural correlates. It is argued that speech errors in navPPA mainly arise within the language system and are not caused by a motor impairment.CrossRefPubMed •• Ash S, McMillan C, Gunawardena D, et al.: Speech errors in progressive non-fluent aphasia. Brain Lang 2010, 113:13–20. This paper provides a detailed, quantitative study of speech sound errors in the connected speech of navPPA patients, with linguistic, neuropsychological, and neural correlates. It is argued that speech errors in navPPA mainly arise within the language system and are not caused by a motor impairment.CrossRefPubMed
39.
go back to reference Ash S, Moore P, Vesely L, et al.: Non-fluent speech in frontotemporal lobar degeneration. J Neurolinguistics 2009, 22:370–383.CrossRef Ash S, Moore P, Vesely L, et al.: Non-fluent speech in frontotemporal lobar degeneration. J Neurolinguistics 2009, 22:370–383.CrossRef
40.
go back to reference Josephs KA, Duffy JR, Strand EA, et al.: Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Brain 2006, 129(Pt 6):1385–1398.CrossRefPubMed Josephs KA, Duffy JR, Strand EA, et al.: Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Brain 2006, 129(Pt 6):1385–1398.CrossRefPubMed
41.
go back to reference Ogar JM, Dronkers NF, Brambati SM, et al.: Progressive nonfluent aphasia and its characteristic motor speech deficits. Alzheimer Dis Assoc Disord 2007, 21:S23–S30.CrossRefPubMed Ogar JM, Dronkers NF, Brambati SM, et al.: Progressive nonfluent aphasia and its characteristic motor speech deficits. Alzheimer Dis Assoc Disord 2007, 21:S23–S30.CrossRefPubMed
42.
go back to reference Peelle JE, Troiani V, Gee J, et al.: Sentence comprehension and voxel-based morphometry in progressive nonfluent aphasia, semantic dementia, and nonaphasic frontotemporal dementia. J Neurolinguistics 2008, 21:418–432.CrossRefPubMed Peelle JE, Troiani V, Gee J, et al.: Sentence comprehension and voxel-based morphometry in progressive nonfluent aphasia, semantic dementia, and nonaphasic frontotemporal dementia. J Neurolinguistics 2008, 21:418–432.CrossRefPubMed
43.
go back to reference Peelle JE, Cooke A, Moore P, et al.: Syntactic and thematic components of sentence processing in progressive nonfluent aphasia and nonaphasic frontotemporal dementia. J Neurolinguistics 2007, 20:482–494.CrossRefPubMed Peelle JE, Cooke A, Moore P, et al.: Syntactic and thematic components of sentence processing in progressive nonfluent aphasia and nonaphasic frontotemporal dementia. J Neurolinguistics 2007, 20:482–494.CrossRefPubMed
44.
go back to reference Weintraub S, Rubin NP, Mesulam MM: Primary progressive aphasia: longitudinal course, neuropsychological profile, and language features. Arch Neurol 1990, 47:1329–1335.PubMed Weintraub S, Rubin NP, Mesulam MM: Primary progressive aphasia: longitudinal course, neuropsychological profile, and language features. Arch Neurol 1990, 47:1329–1335.PubMed
45.
go back to reference Libon DJ, Xie SX, Moore P, et al.: Patterns of neuropsychological impairment in frontotemporal dementia. Neurology 2007, 68:369–375.CrossRefPubMed Libon DJ, Xie SX, Moore P, et al.: Patterns of neuropsychological impairment in frontotemporal dementia. Neurology 2007, 68:369–375.CrossRefPubMed
46.
go back to reference Patterson K, Graham NL, Ralph MA, et al.: Progressive non-fluent aphasia is not a progressive form of non-fluent (post-stroke) aphasia. Aphasiology 2006, 20:1018–1034.CrossRef Patterson K, Graham NL, Ralph MA, et al.: Progressive non-fluent aphasia is not a progressive form of non-fluent (post-stroke) aphasia. Aphasiology 2006, 20:1018–1034.CrossRef
47.
go back to reference Gunawardena D, Ash S, McMillan CT, et al.: Why are progressive non-fluent aphasics non-fluent? Neurology 2010, 75:588–594.CrossRefPubMed Gunawardena D, Ash S, McMillan CT, et al.: Why are progressive non-fluent aphasics non-fluent? Neurology 2010, 75:588–594.CrossRefPubMed
48.
49.
go back to reference Hillis AE, Work M, Barker PB, et al.: Re-examining the brain regions crucial for orchestrating speech articulation. Brain 2004, 127(Pt 7):1479–1487.CrossRefPubMed Hillis AE, Work M, Barker PB, et al.: Re-examining the brain regions crucial for orchestrating speech articulation. Brain 2004, 127(Pt 7):1479–1487.CrossRefPubMed
50.
go back to reference Nestor PJ: Nuclear imaging can predict pathologic diagnosis in progressive nonfluent aphasia. Neurology 2007, 68:238–239.CrossRefPubMed Nestor PJ: Nuclear imaging can predict pathologic diagnosis in progressive nonfluent aphasia. Neurology 2007, 68:238–239.CrossRefPubMed
Metadata
Title
The New Classification of Primary Progressive Aphasia into Semantic, Logopenic, or Nonfluent/Agrammatic Variants
Authors
Michael F. Bonner
Sharon Ash
Murray Grossman
Publication date
01-11-2010
Publisher
Current Science Inc.
Published in
Current Neurology and Neuroscience Reports / Issue 6/2010
Print ISSN: 1528-4042
Electronic ISSN: 1534-6293
DOI
https://doi.org/10.1007/s11910-010-0140-4

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