Progranulin-associated primary progressive aphasia: A distinct phenotype?

Neuropsychologia

Volumn 48, Issue 1, 2010, Pages 288-297

  Rohrer, Jonathan D ^a   Crutch, Sebastian J ^a   Warrington, Elizabeth K ^a   Warren, Jason D ^a  

^a UNIVERSITY COLLEGE LONDON   (United Kingdom)

Author keywords

Aphasia; Dementia; Language; Primary progressive aphasia; Progranulin

Indexed keywords

PROGRANULIN;

ADULT; ANOMIA; ARTICLE; BRAIN ATROPHY; CASE REPORT; COMPREHENSION; FRONTAL LOBE; GENE MUTATION; HUMAN; LANGUAGE PROCESSING; LINGUISTICS; MALE; MEMORY DISORDER; NEUROPSYCHOLOGICAL TEST; PARIETAL LOBE; PRIMARY PROGRESSIVE APHASIA; PROGRESSIVE NONFLUENT APHASIA; SEMANTICS; SHORT TERM MEMORY; SPEECH AND LANGUAGE ASSESSMENT; TEMPORAL LOBE; VERBAL MEMORY;

APHASIA, PRIMARY PROGRESSIVE; COGNITION DISORDERS; COMPREHENSION; HUMANS; INTERCELLULAR SIGNALING PEPTIDES AND PROTEINS; LINGUISTICS; MAGNETIC RESONANCE IMAGING; MALE; MEMORY, SHORT-TERM; MIDDLE AGED; NEUROPSYCHOLOGICAL TESTS; PHENOTYPE; READING; SPEECH DISORDERS; VERBAL BEHAVIOR; VOCABULARY;

EID: 71549116105     PISSN: 00283932     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.neuropsychologia.2009.09.017     Document Type: Article

References (84)

1. Alladi S., Xuereb J., Bak T., Nestor P., Knibb J., Patterson K., and Hodges J.R. Focal cortical presentations of Alzheimer's disease. Brain 130 Pt 10 (2007) 2636-2645
2. Baayen R.H., Piepenbrock R., and van Rijn H. The CELEX lexical data base on CD-ROM (1993), Linguistic Data Consortium, Philadelphia, PA
3. Bak T.H., Yancopoulou D., Nestor P.J., Xuereb J.H., Spillantini M.G., Pulvermüller F., and Hodges J.R. Clinical, imaging and pathological correlates of a hereditary deficit in verb and action processing. Brain 129 Pt 2 (2006) 321-332
4. Baker M., Mackenzie I.R., Pickering-Brown S.M., Gass J., Rademakers R., Lindholm C., Snowden J., Adamson J., Sadovnick A.D., Rollinson S., Cannon A., Dwosh E., Neary D., Melquist S., Richardson A., Dickson D., Berger Z., Eriksen J., Robinson T., Zehr C., Dickey C.A., Crook R., McGowan E., Mann D., Boeve B., Feldman H., and Hutton M. Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17. Nature 442 7105 (2006) 916-919
5. Baxter D.M., and Warrington E.K. Measuring dysgraphia: A graded-difficulty spelling test. Behavioural Neurology 7 3-4 (1994) 107-116
6. Beck J., Rohrer J.D., Campbell T., Isaacs A., Morrison K.E., Goodall E.F., Warrington E.K., Stevens J., Revesz T., Holton J., Al-Sarraj S., King A., Scahill R., Warren J.D., Fox N.C., Rossor M.N., Collinge J., and Mead S. A distinct clinical, neuropsychological and radiological phenotype is associated with progranulin gene mutations in a large UK series. Brain 131 Pt 3 (2008) 706-720
7. Bishop D.V. Test for reception of grammar (1989), MRC Applied Psychology Unit, Cambridge
8. Bozeat S., Lambon Ralph M.A., Patterson K., Garrard P., and Hodges J.R. Non-verbal semantic impairment in semantic dementia. Neuropsychologia 38 9 (2000) 1207-1215
9. Brambati S.M., Ogar J., Neuhaus J., Miller B.L., and Gorno-Tempini M.L. Reading disorders in primary progressive aphasia: A behavioral and neuroimaging study. Neuropsychologia 47 8-9 (2009) 1893-1900
10. Buchsbaum B.R., and D'Esposito M. The search for the phonological store: From loop to convolution. Journal of Cognitive Neuroscience 20 (2008) 762-778
11. Caplan D., and Waters G.S. Verbal working memory and sentence comprehension. Behavioral and Brain Sciences 22 (1999) 77-94
12. Coltheart M. Reading, phonological reading and deep dyslexia. In: Coltheart M., Patterson K.E., and Marshall J.C. (Eds). Deep dyslexia (1980), Routledge, London
13. Corbett F., Jefferies E., Ehsan S., and Lambon Ralph M.A. Different impairments of semantic cognition in semantic dementia and semantic aphasia: Evidence from the non-verbal domain. Brain (2009) June 8 [Epub ahead of print]
14. Corsi P.M. Human memory and the medial temporal region of the brain. Dissertation Abstracts International 34 02 (1972) 891B
15. Costello A.L., and Warrington E.K. Dynamic aphasia: The selective impairment of verbal planning. Cortex 25 (1989) 103-114
16. Crisp J., and Lambon Ralph M.A. Unlocking the nature of the phonological-deep dyslexia continuum: The keys to reading aloud are in phonology and semantics. Journal of Cognitive Neuroscience 18 3 (2006) 348-362
17. Crutch S.J., Randlesome K., and Warrington E.K. The variability of country map knowledge in normal and aphasic subjects: Evidence from two new category-specific screening tests. Journal of Neuropsychology 1 2 (2007) 171-187
18. Cruts M., Gijselinck I., van der Zee J., Engelborghs S., Wils H., Pirici D., Rademakers R., Vandenberghe R., Dermaut B., Martin J.J., van Duijn C., Peeters K., Sciot R., Santens P., De Pooter T., Mattheijssens M., Van den Broeck M., Cuijt I., Vennekens K., De Deyn P.P., Kumar-Singh S., and Van Broeckhoven C. Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21. Nature 442 7105 (2006) 920-924
19. Cummings J.L., Mega M., Gray K., Rosenberg-Thompson S., Carusi D.A., and Gornbein J. The Neuropsychiatric Inventory: Comprehensive assessment of psychopathology in dementia. Neurology 44 12 (1994) 2308-2314
20. Dabul B. Apraxia Battery for Adults. 2nd ed. (2000), Pro-Pro10, Austin, Tx
21. Damasio A., and Tranel D. Nouns and verbs are retrieved with differentially distributed neural systems. Proceedings of the National Academy of Sciences USA 90 (1993) 4957-4960
22. Davies R.R., Hodges J.R., Kril J.J., Patterson K., Halliday G.M., and Xuereb J.H. The pathological basis of semantic dementia. Brain 128 (2005) 1984-1995
23. Dubois B., Slachevsky A., Litvan I., and Pillon B. The FAB: A Frontal Assessment Battery at bedside. Neurology 12 55(11) (2000) 1621-1626
24. Dunn L.M., Dunn L.M., Whetton C., and Pintilie D. British Picture Vocabulary Scale (1982), NFER-NFER10
25. Folstein M., Folstein S., and McHugh P. The "Mini Mental State": A practical method for grading the cognitive state of patients for the clinician. Journal of Psychiatric Research 12 (1975) 189-198
26. Galton C.J., Patterson K., Xuereb J.H., and Hodges J.R. Atypical and typical presentations of Alzheimer's disease: A clinical, neuropsychological, neuroimaging and pathological study of 13 cases. Brain 123 (2000) 484-498
27. Goodglass H., and Kaplan E. The assessment of aphasia and related disorders (1983), Lea and Fediger, Philadelphia
28. Gorno-Tempini M.L., Brambati S.M., Ginex V., Ogar J., Dronkers N.F., Marcone A., Perani D., Garibotto V., Cappa S.F., and Miller B.L. The logopenic/phonological variant of primary progressive aphasia. Neurology 71 16 (2008) 1227-1234
29. Gorno-Tempini M.L., Dronkers N.F., Rankin K.P., Ogar J.M., Phengrasamy L., Rosen H.J., Johnson J.K., Weiner M.W., and Miller B.L. Cognition, and anatomy in three variants of primary progressive aphasia. Annals of Neurology 55 3 (2004) 335-346
30. Grossman M., Koenig P., DeVita C., Glosser G., Alsop D., Detre J., and Gee J. Neural representation of verb meaning: An fMRI study. Human Brain Mapping 15 2 (2002) 124-134
31. Hickok G., and Poeppel D. Dorsal and ventral streams: A framework for understanding aspects of the functional anatomy of language. Cognition 92 1-2 (2004) 67-99
32. Hillis A.E. Aphasia: Progress in the last quarter of a century. Neurology 10 69(2) (2007) 200-213
33. Hodges J.R., Patterson K., Oxbury S., and Funnell E. Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy. Brain 115 Pt 6 (1992) 1783-1806
34. Howard D., and Patterson K. The Pyramids and Palm Trees Test (1992), Thames Valley Test Company, Bury St Edmunds
35. Jackson M., and Warrington E.K. Arithmetic skills in patients with unilateral cerebral lesions. Cortex 22 4 (1986) 611-620
36. Jefferies E., and Lambon Ralph M.A. Semantic impairment in stroke aphasia versus semantic dementia: A case-series comparison. Brain 129 Pt 8 (2006) 2132-2147
37. Josephs K.A. Frontotemporal dementia and related disorders: Deciphering the enigma. Annals of Neurology 64 1 (2008) 4-14
38. Josephs K.A., Duffy J.R., Strand E.A., Whitwell J.L., Layton K.F., Parisi J.E., Hauser M.F., Witte R.J., Boeve B.F., Knopman D.S., Dickson D.W., Jack Jr. C.R., and Petersen R.C. Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Brain 129 Pt 6 (2006) 1385-1398
39. Josephs K.A., Stroh A., Dugger B., and Dickson DW. Evaluation of subcortical pathology and clinical correlations in FTLD-U subtypes. Acta Neuropathology (2009) May 20 [Epub ahead of print]
40. Kay J., Lesser R., and Coltheart M. Psycholinguistic Assessments of Language Processing in Aphasia (PALPA) (1992), Hove, Erlbaum
41. Kessels R.P., van Zandvoort M.J., Postma A., Kappelle L.J., and de Haan E.H. The Corsi Block-Tapping Task: Standardization and normative data. Applied Neuropsychology 7 4 (2000) 252-258
42. Knibb J.A., Xuereb J.H., Patterson K., and Hodges J.R. Clinical and pathological characterization of progressive aphasia. Annals of Neurology 59 1 (2006) 156-165
43. Lesser R. Verbal comprehension in aphasia: An English version of three Italian tests. Cortex 10 (1974) 247-263
44. Manning L., and Warrington E.K. Two routes to naming: A case study. Neuropsychologia 34 8 (1995) 809-817
45. McKenna P. Category Specific Names Test (1998), Psychology Press
46. McKenna P., and Parry R. Category and modality deficits of semantic memory in patients with left hemisphere pathology. Neuropsychological Rehabilitation 4 3 (1994) 283-305
47. McKenna P., and Warrington E.K. Testing for nominal dysphasia. Journal of Neurology Neurosurgery and Psychiatry 43 9 (1980) 781-788
48. Mesulam M.M. Slowly progressive aphasia without generalized dementia. Annals of Neurology 11 6 (1982) 592-598
49. Mesulam M.M. Primary progressive aphasia. Annals of Neurology 49 4 (2001) 425-432
50. Mesulam M.M. Primary progressive aphasia-A language-based dementia. New England Journal of Medicine 16 349(16) (2003) 1535-1542
51. Mesulam M., Johnson N., Krefft T.A., Gass J.M., Cannon A.D., Adamson J.L., Bigio E.H., Weintraub S., Dickson D.W., Hutton M.L., and Graff-Radford N.R. Progranulin mutations in primary progressive aphasia: The PPA1 and PPA3 families. Archives of Neurology 64 1 (2007) 43-47
52. Mesulam M., Wicklund A., Johnson N., Rogalski E., Léger G.C., Rademaker A., Weintraub S., and Bigio E.H. Alzheimer and frontotemporal pathology in subsets of primary progressive aphasia. Annals of Neurology 63 6 (2008) 709-719
53. Morris J.C. The Clinical Dementia Rating (CDR): Current version and scoring rules. Neurology 43 11 (1993) 2412-2414
54. Neary D., Snowden J.S., Gustafson L., Passant U., Stuss D., Black S., Freedman M., Kertesz A., Robert P.H., Albert M., Boone K., Miller B.L., Cummings J., and Benson D.F. Frontotemporal lobar degeneration: A consensus on clinical diagnostic criteria. Neurology 51 (1998) 1546-1554
55. Parisi D., and Pizzamiglio L. Syntactic comprehension in aphasia. Cortex 6 (1970) 204-205
56. Pick A. Uber die Beziehungen der senilen Hirnatrophie zue Aphasie. Prager Medicinische Wochenschrift 17 (1892) 165-167
57. Quigley S.P., Steinkamp M.W., Power D.J., and Jones B.W. Test of syntactic abilities: A guide to administration and interpretation (1978), Dormac Inc., Beaverton, Oregon
58. Rabinovici G.D., Jagust W.J., Furst A.J., Ogar J.M., Racine C.A., Mormino E.C., O'Neil J.P., Lal R.A., Dronkers N.F., Miller B.L., and Gorno-Tempini M.L. Abeta amyloid and glucose metabolism in three variants of primary progressive aphasia. Annals of Neurology 64 4 (2008) 388-401
59. Rademakers R., Baker M., Gass J., Adamson J., Huey E.D., Momeni P., Spina S., Coppola G., Karydas A.M., Stewart H., Johnson N., Hsiung G.Y., Kelley B., Kuntz K., Steinbart E., Wood E.M., Yu C.E., Josephs K., Sorenson E., Womack K.B., Weintraub S., Pickering-Brown S.M., Schofield P.R., Brooks W.S., Van Deerlin V.M., Snowden J., Clark C.M., Kertesz A., Boylan K., Ghetti B., Neary D., Schellenberg G.D., Beach T.G., Mesulam M., Mann D., Grafman J., Mackenzie I.R., Feldman H., Bird T., Petersen R., Knopman D., Boeve B., Geschwind D.H., Miller B., Wszolek Z., Lippa C., Bigio E.H., Dickson D., Graff-Radford N., and Hutton M. Phenotypic variability associated with progranulin haploinsufficiency in patients with the common 1477C → T (Arg493X) mutation: An international initiative. Lancet Neurology 6 10 (2007) 857-868
60. Reverberi C., Cherubini P., Rapisarda A., Rigamonti E., Caltagirone C., Frackowiak R.S., Macaluso E., and Paulesu E. Neural basis of generation of conclusions in elementary deduction. Neuroimage 38 4 (2007) 752-762
61. Rohrer J.D., Knight W.D., Warren J.E., Fox N.C., Rossor M.N., and Warren J.D. Word-finding difficulty: A clinical analysis of the progressive aphasias. Brain 131 Pt 1 (2008) 8-38
62. Rohrer J.D., Warren J.D., Barnes J., Mead S., Beck J., Pepple T., Boyes R., Omar R., Collinge J., Stevens J.M., Warrington E.K., Rossor M.N., and Fox N.C. Mapping the progression of progranulin-associated frontotemporal lobar degeneration. Nature Clinical Practice Neurology 4 8 (2008) 455-460
63. Rosen H.J., Allison S.C., Ogar J.M., Amici S., Rose K., Dronkers N., Miller B.L., and Gorno-Tempini M.L. Behavioral features in semantic dementia vs other forms of progressive aphasias. Neurology 67 10 (2006) 1752-1756
64. Saur D., Kreher B.W., Schnell S., Kümmerer D., Kellmeyer P., Vry M.S., Umarova R., Musso M., Glauche V., Abel S., Huber W., Rijntjes M., Hennig J., and Weiller C. Ventral and dorsal pathways for language. Proceedings of the National Academy of Sciences USA 105 46 (2008) 18035-18040
65. Scott S.K., and Johnsrude I.S. The neuroanatomical and functional organization of speech perception. Trends in Neurosciences 26 2 (2003) 100-107
66. Seeley W.W., Crawford R.K., Zhou J., Miller B.L., and Greicius M.D. Neurodegenerative diseases target large-scale human brain networks. Neuron 62 1 (2009) 42-52
67. Shallice T., and Butterworth B. Short term memory impairment and spontaneous speech. Neuropsychologia 15 (1977) 729-735
68. Shi J., Shaw C.L., Du Plessis D., Richardson A.M., Bailey K.L., Julien C., Stopford C., Thompson J., Varma A., Craufurd D., Tian J., Pickering-Brown S., Neary D., Snowden J.S., and Mann D.M. Histopathological changes underlying frontotemporal lobar degeneration with clinicopathological correlation. Acta Neuropathologica 110 5 (2005) 501-512
69. Snowden J.S., Goulding P.J., and Neary D. Semantic dementia: A form of circumscribed cerebral atrophy. Behavioural Neurology 2 (1989) 167-182
70. Snowden J.S., and Neary D. Progressive anomia with preserved oral spelling and automatic speech. Neurocase 9 1 (2003) 27-34
71. Snowden J., Neary D., and Mann D. Frontotemporal lobar degeneration: Clinical and pathological relationships. Acta Neuropathologica 114 1 (2007) 31-38
72. Snowden J.S., Pickering-Brown S.M., Du Plessis D., Mackenzie I.R., Varma A., Mann D.M., and Neary D. Progressive anomia revisited: Focal degeneration associated with progranulin gene mutation. Neurocase 13 5 (2007) 366-377
73. Snowden J.S., Pickering-Brown S.M., Mackenzie I.R., Richardson A.M., Varma A., Neary D., and Mann D.M. Progranulin gene mutations associated with frontotemporal dementia and progressive non-fluent aphasia. Brain 129 Pt 11 (2006) 3091-3102
74. Snowling M.J., Stothard S.E., and McLean J. Graded nonword reading test, Thames Valley Test (1996), Bury St. Edmunds, UK
75. Spitsyna G., Warren J.E., Scott S.K., Turkheimer F.E., and Wise R.J. Converging language streams in the human temporal lobe. Journal of Neuroscience 26 28 (2006) 7328-7336
76. Vigneau M., Beaucousin V., Hervé P.Y., Duffau H., Crivello F., Houdé O., Mazoyer B., and Tzourio-Mazoyer N. Meta-analyzing left hemisphere language areas: Phonology, semantics, and sentence processing. Neuroimage 30 4 (2006) 1414-1432
77. Warren J.D., Warren J.E., Fox N.C., and Warrington E.K. Nothing to say, something to sing: Primary progressive dynamic aphasia. Neurocase 9 2 (2003) 140-155
78. Warren J.E., Wise R.J., and Warren J.D. Sounds do-able: Auditory-motor transformations and the posterior temporal plane. Trends in Neurosciences 28 12 (2005) 636-643
79. Warrington E.K. The selective impairment of semantic memory. Quarterly Journal of Experimental Psychology 27 (1975) 635-657
80. Warrington E.K. The Camden Memory Tests (1996), Psychology Press
81. Warrington E.K., and Crutch S.J. A within-modality test of semantic knowledge: The Size/Weight Attribute Test. Neuropsychology 21 6 (2007) 803-811
82. Warrington E.K., and James M. The visual object and space perception battery (1991), Thames Valley Test Company, Bury St Edmunds, UK
83. Warrington E.K., McKenna P., and Orpwood L. Single word comprehension: A concrete and abstract word synonym test. Neuropsychological Rehabilitation 8 (1998) 143-154
84. Wechsler D. Manual for the Wechsler Adult intelligence scale. Revised (1981), Psychological Corporation, New York