Primary progressive aphasia: From syndrome to disease;Afasia progresiva primaria: Del síndrome a la enfermedad

Neurologia

Volumn 28, Issue 6, 2013, Pages 366-374

  Matias Guiu J A ^a   Garcia Ramos R ^a  

^a HOSPITAL CLÍNICO SAN CARLOS   (Spain)

Author keywords

Alzheimer disease; Frontotemporal dementia; Frontotemporal lobar degeneration; Logopenic; Primary progressive aphasia; Semantic dementia; Tauopathies

Indexed keywords

BIOLOGICAL MARKER; CHOLINESTERASE INHIBITOR;

CLINICAL FEATURE; DISEASE COURSE; HISTOPATHOLOGY; NEUROIMAGING; ONSET AGE; PREDICTIVE VALUE; PRIMARY PROGRESSIVE APHASIA; SHORT SURVEY; TREATMENT PLANNING;

AFASIA PROGRESIVA PRIMARIA; ALZHEIMER DISEASE; DEGENERACIÓN FRONTOTEMPORAL LOBAR; DEMENCIA FRONTOTEMPORAL; DEMENCIA SEMÁNTICA; ENFERMEDAD DE ALZHEIMER; FRONTOTEMPORAL DEMENTIA; FRONTOTEMPORAL LOBAR DEGENERATION; LOGOPENIC; LOGOPÉNICA; PRIMARY PROGRESSIVE APHASIA; SEMANTIC DEMENTIA; TAUOPATHIES; TAUPATÍAS;

APHASIA, PRIMARY PROGRESSIVE; HUMANS;

EID: 84881222074     PISSN: 02134853     EISSN: 15781968     Source Type: Journal    
DOI: 10.1016/j.nrl.2012.04.003     Document Type: Short Survey

References (101)

1. Mesulam M.M. Primary progressive aphasia. Ann Neurol 2001, 49:425-432.
2. Westbury C., Bub D. Primary progressive aphasia: a review of 112 cases. Brain Lang 1997, 60:381-406.
3. Gorno-Tempini M.L., Dronkers N.F., Rankin K.P., Ogar J.M., La Phengrasamy B.A., Rosen H.J., et al. Cognition and anatomy in three variants of primary progressive aphasia. Ann Neurol 2004, 55:335-346.
4. Stevens M., Van Duijn C.M., Kamphorst W., De Knijff P., Heutink P., Van Gool W.A., et al. Familial aggregation in frontotemporal dementia. Neurology 1998, 50:1541-1545.
5. Ratnavalli E., Brayne C., Dawson K., Hodges J.R. The prevalence of frontotemporal dementia. Neurology 2002, 58:1615-1621.
6. Ikejima C., Yasuno F., Mizukami K., Sasaki M., Tanimukai S., Asada T. Prevalence and causes of early-onset dementia in Japan: a population-based study. Stroke 2009, 40:2709-2714.
7. Grossman M. Primary progressive aphasia: clinicopathological correlations. Nat Rev Neurol 2010, 6:88-97.
8. Del Barrio J.L., De Pedro-Cuesta J., Boix R., Acosta J., Bergareche A., Bermejo-Pareja F., et al. Dementia, stroke and Parkinson's disease in Spanish populations: a review of door-to-door prevalence surveys. Neuroepidemiology 2005, 24:179-188.
9. De Pedro-Cuesta J., Virués-Ortega J., Vega S., Seijo-Martínez M., Saz P., Rodríguez F., et al. Prevalence of dementia and major dementia subtypes in Spanish populations: a reanalysis of dementia prevalence surveys, 1990-2008. BMC Neurol 2009, 9:55.
10. Gascón-Bayarri J., Reñé R., Del Barrio J.L., De Pedro-Cuesta J., Ramón J.M., Manubens J.M., et al. Prevalence of dementia subtypes in El Prat de Llobregat, Catalonia, Spain: the PRATICON study. Neuroepidemiology 2007, 28:223-234.
11. Fernández M., Castro-Flores J., Pérez-de las Heras S., Mandaluniz-Lekumberri A., Gordejuela M., Zarranz J. Prevalencia de la demencia en mayores de 65 años en una comarca del País Vasco. Rev Neurol 2008, 46:89-96.
12. Rogalski E., Johnson N., Weintraub S., Mesulam M. Increased frequency of learning disability in patients with primary progressive aphasia and their first-degree relatives. Arch Neurol 2008, 65:244-248.
13. Weintraub S., Fahey C., Johnson N., Mesulam M., Gitelman D.R., Weitner B.B., et al. Vasectomy in men with primary progressive aphasia. Cogn Behav Neurol 2006, 19:190-193.
14. Mesulam M.M. Primary progressive aphasia. A 25-year retrospective. Alzheimer Dis Assoc Disord 2007, 21:S8-S11.
15. Rogalski E.J., Mesulam M.M. Clinical trajectories and biological features of primary progressive aphasia. Curr Alzheimer Res 2009, 6:334-336.
16. Snowden J.S., Pickering-Brown S.M., Mackenzie I.R., Richardson A.M.T., Varma A., Neary D., et al. Progranulin gene mutations associated with frontotemporal dementia and progressive non-fluent aphasia. Brain 2006, 129:3091-3102.
17. Mesulam M., Johnson N., Krefft T.A., Gass J.M., Cannon A.D., Adamson J.L., et al. Progranulin mutations in primary progressive aphasia. Arch Neurol 2007, 64:43-47.
18. Pickering-Brown S.M., Rollinson S., DuPlessis D., Morrison K.E., Varma A., Richardson A.M.T., et al. Frequency and clinical characteristics of progranulin mutation carriers in the Manchester frontotemporal lobar degeneration cohort: comparison with patients with MAPT and no known mutations. Brain 2008, 131:721-731.
19. Rohrer J.D., Crutch S.J., Wattington E.K., Warren J.D. Progranulin-associated primary progressive aphasia: a distinct phenothype. Neuropsychologia 2010, 48:288-297.
20. Bessi V., Bagnoli S., Nacmias B., Tedde A., Sorbi S., Bracco L. Semantic dementia associated with mutation V363I in the tau gene. J Neurol Sci 2010, 296:112-114.
21. Mesulam M.M., Wicklund A., Johnson N., Rogalski E., Léger G.C., Rademaker A., et al. Alzheimer and Frontotemporal pathology in subsets of primary progressive aphasia. Ann Neurol 2008, 63:709-719.
22. Rogalski E.J., Rademaker A., Harrison T.M., Helenowski I., Johnson N., Bigio E., et al. ApoE E4 is a susceptibility factor in amnestic but not aphasic dementias. Alzheimer Dis Assoc Disord 2011, 25:159-163.
23. Gorno-Tempini M.L., Brambati S.M., Ginex V., Ogar J., Dronkers N.F., Marcone A., et al. The logopenic/phonological variant of primary progressive aphasia. Neurology 2008, 71:1227-1234.
24. Sobrido M.J., Abu-Khalil A., Weintraub S., Johnson N., Quinn B., Cummings J.L., et al. Possible association of the tau H1/H1 genotype with primary progressive aphasia. Neurology 2003, 60:862-864.
25. Verpillat P., Camuzat A., Hannequin D., Thomas-Anterion C., Puel M., Belliard S., et al. Association between the extended tau haplotype and frontotemporal dementia. Arch Neurol 2002, 59:935-939.
26. Pittman A.M., Myers A.J., Abou-Sleiman P., Fung H.C., Kaleem M., Marlowe L., et al. Linkage disequilibrium fine mapping and haplotype association analysis of the tau gene in progressive supranuclear palsy and corticobasal degeneration. J Med Genet 2005, 42:837-846.
27. Lladó A., Sánchez-Valle R., Rey M.J., Ezquerra M., Tolosa E., Ferrer I., et al. Clinicopathological and genetic correlates of frontotemporal lobar degeneration and corticobasal degeneration. J Neurol 2008, 255:488-494.
28. Li X., Rowland L.P., Mitsumoto H., Przedborski S., Bird T.D., Schellenberg G.D., et al. Prion protein codon 129 genotype prevalence is altered in primary progressive aphasia. Ann Neurol 2005, 58:858-864.
29. Bonner M.F., Ash S., Grossman M. The new classification of primary progressive aphasia into semantic, logopenic, or nonfluent/agrammatic variants. Curr Neurol Neurosci Rep 2010, 10:484-490.
30. Gorno-Tempini M.L., Hillis A.E., Weintraub S., Kertesz A., Mendez M., Cappa S.F., et al. Classification of primary progressive aphasia and its variants. Neurology 2011, 76:1006-1014.
31. Davies R.R., Hodges J.R., Kril J.J., Patterson K., Halliday G.M., Xuereb J.H. The pathological basis of semantic dementia. Brain 2005, 128:1984-1995.
32. Hodges J.R., Patterson K. Semantic dementia: a unique clinicopathological syndrome. Lancet Neurol 2007, 6:1004-1014.
33. Mesulam M.M. Primary progressive aphasia -a language-based dementia. N Eng J Med 2003, 349:1535-1542.
34. Mesulam M.M., Grossman M., Hillis A., Kertesz A., Weintraub S. The core and halo of primary progressive aphasia and semantic dementia. Ann Neurol 2003, 54:S11-S14.
35. Adlam A.L.R., Patterson K., Rogers T.T., Nestor P.J., Salmond C.H., Acosta-Cabronero J., et al. Semantic dementia and fluent primary progressive aphasia: two sides of the same coin. Brain 2006, 129:3066-3080.
36. Rohrer J.D., Knight W.D., Warren J.E., Fox N.C., Rossor M.N., Warren J.D. Word-finding difficulty: a clinical analysis of the progressive aphasias. Brain 2008, 131:8-38.
37. Ogar J.M., Dronkers N.F., Brambati S.M., Miller B.L., Gorno-Tempini M.L. Progressive nonfluent aphasia and its characteristic motor speech deficits. Alzheimer Dis Assoc Disord 2007, 21:S23-S30.
38. Rosen H.J., Allison S.C., Ogar J.M., Amici S., Rose K., Dronkers N., et al. Behavioral features in semantic dementia vs other forms of progressive aphasias. Neurology 2006, 67:1752-1756.
39. Kremen S.A., Mendez M.F., Tsai P.H., Teng E. Extrapyramidal signs in the primary progressive aphasia. Am J Alzheimers Dis Other Demen 2011, 26:72-77.
40. Le Rhun E., Richard F., Pasquier F. Natural history of primary progressive aphasia. Neurology 2005, 65:887-891.
41. Hodges J.R., Mitchell J., Dawson K., Spillantini M.G., Xuereb J.H., McMonagle P., et al. Semantic dementia: demography, familial factors and survival in a consecutive series of 100 cases. Brain 2010, 133:300-306.
42. Kertesz A., McMonagle P., Blair M., Davidson W., Munoz D.G. The evolution and pathology of frontotemporal dementia. Brain 2005, 128:1996-2005.
43. Osher J.E., Wicklund A.H., Rademaker A., Johnson N., Weintraub S. The mini-mental state examination in behavioral variant frontotemporal dementia and primary progressive aphasia. Am J Alzheimers Dis Other Demen 2007, 22:468-473.
44. Leyton C.E., Hornberger M., Mioshi E., Hodges J.R. Application of Addensbrooke's cognitive examination to diagnosis and monitoring of progressive primary aphasia. Dement Geriatr Cogn Disord 2010, 29:504-509.
45. Dickerson B.C. Quantitating severity and progression in primary progressive aphasia. J Mol Neurosci 2011, 45:618-628.
46. Sapolski D., Bakkour A., Negreira A., Nalipinski P., Weintraub S., Mesulam M.M., et al. Cortical neuroanatomic correlated of symptom severity in primary progressive aphasia. Neurology 2010, 75:358-366.
47. Knibb J.A., Xuereb J.H., Patterson K., Hodges J.R. Clinical and pathological characterization of progressive aphasia. Ann Neurol 2006, 59:156-165.
48. Knopman D.S., Boeve B.F., Parisi J.E., Dickson D.W., Smith G.E., Ivnik R.J., et al. Antemortem diagnosis of frontotemporal lobar degeneration. Ann Neurol 2005, 57:480-488.
49. Josephs K.A., Duffy J.R., Strand E.A., Whitwell J.L., Layton K.F., Parisi J.E., et al. Clinocopathological and imaging correlates of progressive aphasia and apraxia of speech. Brain 2006, 129:1385-1398.
50. Snowden J., Neary D., Mann D. Frontotemporal degeneration: clinical and pathological relationships. Acta Neuropathol 2007, 114:31-38.
51. Alladi S., Xuereb J., Bak T., Nestor P., Knibb J., Patterson K., et al. Focal cortical presentations of Alzheimer's disease. Brain 2007, 130:2636-2645.
52. Deramecourt V., Lebert F., Debachy B., Mackowiak-Cordoliani M.A., Bombois S., Kerdraon O., et al. Neurology 2010, 74:42-49.
53. Josephs K.A., Duffy J.R., Strand E.A., Machulda M.M., Senjem M.L., Master A.V., et al. Characterizing a neurodegenerative syndrome: primary progressive apraxia of speech. Brain 2012, 135:1522-1536.
54. Hodges J.R., Davies R.R., Xuereb J.H., Casey B., Broe M., Bak T.H., et al. Clinicopathological correlates in frontotemporal dementia. Ann Neurol 2004, 56:399-406.
55. Forman M.S., Farmer J., Johnson J.K., Clark C.M., Arnold S.E., Coslett H.B., et al. Frontotemporal dementia: clinicopathological correlations. Ann Neurol 2006, 59:952-962.
56. Hu W.T., McMillan C., Libon D., Leight S., Forman M., Lee V.M.Y., et al. Multimodal predictors for Alzheimer disease in nonfluent primary progressive aphasia. Neurology 2010, 75:595-602.
57. Rohrer J.D., Rossor M.N., Warren J.D. Alzheimer's pathology in primary progressive aphasia. Neurobiol Aging 2012, 33:744-752.
58. Munoz D.G., Woulfe J., Kertesz A. Argyrophilic thorny astrocyte clusters in association with Alzheimer's disease pathology in possible primary progressive aphasia. Acta Neuropathol 2007, 114:347-357.
59. Bigio E.H., Mishra M., Hatanpaa K.J., White C.L., Johnson N., Rademaker A., et al. TDP-43 pathology in primary progressive aphasia and frontotemporal dementia with pathologic Alzheimer disease. Acta Neuropathol 2010, 120:43-54.
60. Galton C.J., Paterson K., Xuereb J.H., Hodges J.R. Atypical and typical presentations of Alzheimer's disease: a clinical, neuropsychological, neuroimaging and pathological study of 13 cases. Brain 2000, 123:484-498.
61. Rohrer J.D., Warren J.D., Modat M., Ridgway G.R., Douiri A., Rosson M.N., et al. Patterns of cortical thinning in the language variants of frontotemporal degeneration. Neurology 2009, 72:1562-1569.
62. Josephs K.A., Whitwell J.L., Duffy J.R., Vanvoorst W.A., Strand E.A., Hu W.T., et al. Progressive aphasia secondary to Alzheimer disease vs FTLD pathology. Neurology 2008, 70:25-34.
63. Gorno-Tempini M.L., Ogar J.M., Brambati S.M., Wang P., Jeong J.H., Rankin K.P., et al. Anatomical correlates of early mutism in progressive nonfluent aphasia. Neurology 2006, 67:1849-1851.
64. Whitwell J.L., Avula R., Senjem M.L., Kantarci K., Weigan S.D., Samikoglu A., et al. Gray and white matter diffusion in the syndromic variants of frontotemporal dementia. Neurology 2010, 74:1279-1287.
65. Oliveira F.P., Costa J.C., Marroni S.P., Silva A.M., Barreiro S.H., Maeda F.K., et al. Primary progressive aphasia patients evaluated using diffusion tensor imaging and voxel based volumetry-preliminary results. Arq Neuropsiquiatr 2011, 69:446-451.
66. Galantucci S., Tartaglia M.C., Wilson S.M., Henry M.L., Filippi M., Agosta F., et al. White matter damage in primary progressive aphasias: a diffusion tensor tractography study. Brain 2011, 134:3011-3029.
67. Schwindt GC, Graham NL, Rochon E, Tang-Wai DF, Lobaugh NJ, Chow TW, et al. Whole-brain white matter disruption in semantic and nonfluent variants of primary progressive aphasia. Hum Brain Mapp. 2011, doi:10.1002/hbm.21484.
68. Nestor P.J., Balan K., Cheow H.K., Fryer T.D., Knibb J.A., Xuereb J.H., et al. Nuclear imaging can predict pathologic diagnosis in progressive nonfluent aphasia. Neurology 2007, 68:238-239.
69. Nestor P.J., Graham N.L., Fryer T.D., Williams G.B., Patterson K., Hodges J.R. Progressive non-fluent aphasia is associated with hypometabolism centred on the left anterior insula. Brain 2003, 126:2406-2418.
70. Clark D.G., Charuvastra A., Miller B.L., Shapira J.S., Mendez M.F. Fluent versus nonfluent primary progressive aphasia: a comparison of clinical and functional neuroimaging features. Brain Lang 2005, 94:54-60.
71. Rabinovici G.D., Jagust W.J., Furst A.J., Ogar J.M., Racine C.A., Mormino E.C., et al. Abeta amyloid and glucose metabolism in three variants of primary progressive aphasia. Ann Neurol 2008, 64:388-401.
72. Josephs K.A., Duffy J.R., Fossett T.R., Strand E.A., Claassen D.O., Whitwell J.L., et al. Fluorodeoxyglucose F18 positron emission tomography in progressive apraxia of speech and primary progressive aphasia variants. Arch Neurol 2010, 67:596-605.
73. Panegyres P.K., McCarthy M., Campbell A., Lenzo N., Fallon M., Thompson J. Correlative studies of structural and functional imaging in primary progressive aphasia. Am J Alzheimers Dis Other Demen 2008, 23:184-191.
74. Sunderlan T., Linker G., Mirza N., Putman K.T., Friedman D.L., Kimmel L.H., et al. Decreased B-amyloid 1-42 and increased tau levels in cerebrospinal fluid of patients with alzheimer disease. JAMA 2003, 289:2097-2103.
75. Tapiola T., Alafuzoff I., Herukka S.K., Parkkinen L., Hartikainen P., Soininen H., et al. Cerebrospinal fluid B-amyloid 42 and tau proteins as biomarkers of Alzheimer-type pathologic changes in the brain. Arch Neurol 2009, 66:382-389.
76. Shaw L.M., Korecka M., Clark C.M., Lee V.M.Y., Troyanowski J.Q. Biomarkers of neurodegeneration for diagnosis and monitoring therapeutics. Nat Rev Drug Discov 2007, 4:295-303.
77. De Souza L.C., Lamari F., Belliard S., Jardel C., Houillier C., De Paz R., et al. Cerebrospinal fluid biomarkers in the differential diagnosis of Alzheimer's disease from other cortical dementias. J Neurol Neurosurg Psychiatry 2011, 82:240-246.
78. Grossman M., Farmer J., Leight S., Work M., Moore P., Van Deerlin V., et al. Cerebrospinal fluid profile in frontotemporal dementia and Alzheimer's disease. Ann Neurol 2005, 57:721-729.
79. Bian H., Van Swieten J.C., Leight S., Massimo L., Wood E., Forman M., et al. CSF biomarkers in frontotemporal lobar degeneration with known pathology. Neurology 2008, 70:1827-1835.
80. Kapaki E., Paraskevas G.P., Papageorgiou S.G., Bonakis A., Kalfakis N., Zalonis I., et al. Diagnostic value of CSF biomarker profile in frontotemporal lobar degeneration. Alzheimer Dis Assoc Disord 2008, 22:47-53.
81. Andersen C., Fabre S.F., Östberg P., Lannfelt L., Wahlund L.O. Tau protein in cerebrospinal fluid from semantic dementia patients. Neurosci Lett 2000, 294:155-158.
82. Maeck L., Meller J., Otto M., Stiens G., Wiltfang J., Stoppe G. Abeta peptide 1-42, tau protein and S-100B protein level in cerebrospinal fluid of three patients with primary progressive aphasia. Neurosci Lett 2002, 333:33-36.
83. Bibl M., Mollenhauer B., Lewczuk P., Esselmann H., Wolf S., Otto M., et al. Cerebrospinal fluid tau, p-tau 181 and amyloid-B38/40/42 in frontotemporal dementias and primary progressive aphasias. Dement Geriatr Cogn Disord 2011, 31:37-44.
84. Hu W.T., Chen-Plotkin A., Grossman M., Arnold S.E., Clark C.M., Shaw L.M., et al. Novel CSF biomarkers for frontotemporal lobar degenerations. Neurology 2010, 75:2079-2086.
85. Weintraub S., Morhardt D.J. Treatment, education and resources for non Alzheimer dementia: one size does not fit all. Alzheimer's Care Q 2005, 6:201-214.
86. Reed D.A., Johnson N.A., Thompson C., Weintraub S., Mesulam M. A clinical trial of bromocriptine for treatment of primary progressive aphasia. Ann Neurol 2004, 56:750.
87. Kertesz A., Morlog D., Light M., Blair M., Davidson W., Jesso S., et al. Galantamine in frontotemporal dementia and primary progressive aphasia. Dement Geriatr Cogn Disord 2008, 25:178-185.
88. Moretti R., Torre P., Antonello R.M., Cattaruzza T., Cazzato G., Brava A. Rivastigmine in frontotemporal dementia: an open-label study. Drugs Aging 2004, 21:931-937.
89. Moretti R., Torre P., Antonello R.M., Cazzato G., Brava A. Effects of selegiline on fronto-temporal dementia: a neuropsychological evaluation. Int J Geriatr Psychiatry 2002, 17:391-392.
90. Boxer A.L., Lipton A.M., Womack K., Merrilees J., Neuhaus J., Pavlic D., et al. An open-label study of memantine treatment in 3 subtypes of frontotemporal lobar degeneration. Alzheimer Dis Assoc Disord 2009, 23:211-217.
91. Johnson N.A., Rademaker A., Weintraub S., Gitelman D., Wienecke C., Mesulam M. Pilot trial of memantine in primary progressive aphasia. Alzheimer Dis Assoc Disord 2010, 24:308.
92. Decker D.A., Heilman K.M. Steroid treatment of primary progressive aphasia. Arch Neurol 2008, 65:1533-1535.
93. Tobinick E. Perispinal etanercept produces rapid improvement in primary progressive aphasia: identification of a novel, rapidly reversible TNF-mediated pathophysiologic mechanism. Medscape J Med 2008, 10:135.
94. Banks S.J., Weintraub S. Neuropsychiatric symptoms in behavioral variant frontotemporal dementia and primary progressive aphasia. J Geriatr Psychiatry Neurol 2008, 21:133-141.
95. Louis M., Espesser R., Rey V., Daffaure V., Di Cristo A., Habib M. Intensive training of phonological skills in progressive aphasia: a model of brain plasticity in neurodegenerative disease. Brain Cogn 2001, 46:197-201.
96. Jokel R., Rochon E., Leonard C. Treating anomia in semantic dementia: improvement, maintenance, or both. Neuropsychol Rehabil 2006, 16:241-256.
97. Marcotte K., Ansaldo A.I. The neural correlates of semantic feature analysis in chronic aphasia: discordant patterns according to the etiology. Semin Speech Lang 2010, 31:52-63.
98. Dressel K. Model-oriented naming therapy in semantic dementia: a single-case fMRI study. Aphasiology 2011, 24:1-23.
99. Beeson P.M., King R.M., Bonakdarpour B., Henry M.L., Cho H., Rapcsak S.Z. Possitive effects of language treatment for the logopenic variant of primary progressive aphasia. J Mol Neurosci 2011, 45:724-736.
100. Grossman M., Libon D.J., Forman M.S., Massimo L., Wood E., Moore P., et al. Distinct antemortem profiles in patients with pathologically defined frontotemporal dementia. Arch Neurol 2007, 64:1601-1609.
101. Grossman M., Xie S.X., Libon D.J., Wang X., Massimo L., Moore P., et al. Longitudinal decline in autopsy-defined frontotemporal lobar degeneration. Neurology 2008, 70:2036-2045.