Encyclopedia of Geropsychology

2017 Edition
| Editors: Nancy A. Pachana

Frontotemporal Dementia (FTD)

  • Maxime Bertoux
  • Claire O’Callaghan
  • Emma Flanagan
  • Michael Hornberger
Reference work entry
First Online:
DOI: https://doi.org/10.1007/978-981-287-082-7_311
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Synonyms

Behavioural variant frontotemporal dementia; Frontal dementia; Semantic Dementia; Progressive non-fluent aphasia; Pick disease

Definition

Frontotemporal dementia is an insidious neurodegenerative disease characterised by progressive deficits in behaviour and cognition. Three main clinical entities as well as overlaping syndromes affecting the young subject (< 65 years old) are described here on a clinical, neuropsychological and imaging point of view.

Introduction

Frontotemporal dementia (FTD) is an insidious neurodegenerative disease characterised by progressive deficits in behaviour and cognition. FTD is a common type of dementia, particularly in patients younger than 65 years. It is the second most common form of younger-onset dementia after Alzheimer’s disease (AD) (Ratnavalli et al. 2002). Despite such prevalence, FTD has received far less recognition compared to other dementias. This is surprising considering the overlap FTD shares clinically and pathologically with...

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Notes

Acknowledgments

Dr. M. Bertoux is supported by the European Commission and Dr. C. O’Callaghan by the National Health and Medical Research Council (MRC-Australia).

References

  1. Aarsland, D., Litvan, I., & Larsen, J. P. (2001). Neuropsychiatric symptoms of patients with progressive supranuclear palsy and Parkinson’s disease. The Journal of Neuropsychiatry and Clinical Neurosciences, 13(1), 42–49.PubMedCrossRefGoogle Scholar
  2. Agosta, F., Canu, E., Sarro, L., Comi, G., & Filippi, M. (2012). Neuroimaging findings in frontotemporal lobar degeneration spectrum of disorders. Cortex, 48(4), 389–413.PubMedCrossRefGoogle Scholar
  3. Ahmed, R. M., Irish, M., Kam, J., van Keizerswaard, J., Bartley, L., Samaras, K., Hodges, J. R., & Piguet, O. (2014). Quantifying the eating abnormalities in frontotemporal dementia. JAMA Neurology, 71(12), 1540–1546.PubMedCrossRefGoogle Scholar
  4. Alladi, S., Xuereb, J., Bak, T., Nestor, P., Knibb, J., Patterson, K., & Hodges, J. R. (2007). Focal cortical presentations of Alzheimer’s disease. Brain, 130(Pt 10), 2636–2645.PubMedCrossRefGoogle Scholar
  5. Armstrong, M. J., Litvan, I., Lang, A. E., Bak, T. H., Bhatia, K. P., Borroni, B., et al. (2013). Criteria for the diagnosis of corticobasal degeneration. Neurology, 80(5), 496–503.PubMedPubMedCentralCrossRefGoogle Scholar
  6. Ash, S., Moore, P., Vesely, L., Gunawardena, D., McMillan, C., Anderson, C., et al. (2009). Non-fluent speech in frontotemporal lobar degeneration. Journal of Neurolinguistics, 22(4), 370–383.PubMedPubMedCentralCrossRefGoogle Scholar
  7. Baez, S., Manes, F., Huepe, D., Torralva, T., Fiorentino, N., Richter, F., Huepe-Artigas, D., Ferrari, J., Montañes, P., Reyes, P., Matallana, D., Vigliecca, N. S., Decety, J., Ibanez, A., Baez, S., Manes, F., Huepe, D., Torralva, T., Fiorentino, N., Richter, F., Huepe-Artigas, D., Ferrari, J., Montañes, P., Reyes, P., Matallana, D., Vigliecca, N. S., Decety, J., & Ibanez, A. (2014). Primary empathy deficit in frontotemporal dementia. Frontiers in Aging Neuroscience, 6, 262.PubMedPubMedCentralCrossRefGoogle Scholar
  8. Bak, T. H., & Hodges, J. R. (2008). Corticobasal degeneration: Clinical aspects. In Handbook of clinical neurology (pp. 509–521). Amsterdam: Elsevier.Google Scholar
  9. Bang, J., Spina, S., & Miller, B. L. (2015). Frontotemporal dementia. Lancet, 386(10004), 1672–1682.PubMedCrossRefGoogle Scholar
  10. Belliard, S., Bon, L., LeMoal, S., Jonin, P. Y., Vercelletto, M., & LeBail, B. (2007). Semantic dementia. Psychologie & Neuropsychiatrie du Vieillissement, 5(2), 127–138.Google Scholar
  11. Bertoux, M., Funkiewiez, A., O’Callaghan, C., Dubois, B., & Hornberger, M. (2013). Sensitivity and specificity of ventromedial prefrontal cortex tests in behavioural variant frontemporal dementia. Alzheimer’s and Dementia: Journal of the Alzheimer’s Association, 9(5 Suppl), S84–S94.CrossRefGoogle Scholar
  12. Bertoux, M., de Souza, L. C., Corlier, F., Lamari, F., Bottlaender, M., Dubois, B., & Sarazin, M. (2014). Two distinct amnesic profiles in behavioral variant frontotemporal dementia. Biological Psychiatry, 75(7), 582–588.PubMedCrossRefGoogle Scholar
  13. Bertoux, M., de Souza, L. C., Sarazin, M., Funkiewiez, A., Dubois, B., & Hornberger, M. (2015a). How preserved is emotion recognition in alzheimer disease compared with behavioral variant frontotemporal dementia? Alzheimer Disease and Associated Disorders, 29(2), 154–157.PubMedGoogle Scholar
  14. Bertoux, M., O’Callaghan, C., Flanagan, E., Hodges, J. R., & Hornberger, M. (2015b). Fronto-striatal atrophy in behavioral variant frontotemporal dementia and Alzheimer’s disease. Frontiers in Neurology, 6, 147.PubMedPubMedCentralCrossRefGoogle Scholar
  15. Bertoux, M., de Souza, L. C., Zamith, P., Dubois, B., & Bourgeois-Gironde, S. (2015c). Discounting of future rewards in behavioural variant frontotemporal dementia and Alzheimer’s disease. Neuropsychology, 29(6), 933–939.PubMedCrossRefGoogle Scholar
  16. Bishop, D. V. (2003). Test for reception of grammar: TROG-2 version 2. London: Pearson Assessment.Google Scholar
  17. Boeve, B. F., Lang, A. E., & Litvan, I. (2003). Corticobasal degenerationa and its relationship to progressive supranuclear palsy and frontotemporal dementia. Annals of Neurology, 54(Suppl 5), S15–S19.PubMedCrossRefGoogle Scholar
  18. Bora, E., Walterfang, M., & Velakoulis, D. (2015). Theory of mind in behavioural-variant frontotemporal dementia and Alzheimer’s disease: A meta-analysis. Journal of Neurology, Neurosurgery, and Psychiatry, 86(7), 714–9. doi:10.1136/jnnp-2014-309445. pii: jnnp-2014-309445.PubMedCrossRefGoogle Scholar
  19. Bozeat, S., Gregory, C. A., Ralph, M. A., & Hodges, J. R. (2000). Which neuropsychiatric and behavioural features distinguish frontal and temporal variants of frontotemporal dementia from Alzheimer’s disease? Journal of Neurology, Neurosurgery, and Psychiatry, 69(2), 178–186.PubMedPubMedCentralCrossRefGoogle Scholar
  20. Braak, H., & Braak, E. (1991). Neuropathological stageing of Alzheimer-related changes. Acta Neuropathologica, 82(4), 239–259.PubMedCrossRefGoogle Scholar
  21. Brambati, S. M., Amici, S., Racine, C. A., Neuhaus, J., Miller, Z., Ogar, J., et al. (2015). Longitudinal gray matter contraction in three variants of primary progressive aphasia: A tenser-based morphometry study. NeuroImage: Clinical, 8, 345–355.CrossRefGoogle Scholar
  22. Brown, R. G., Lacomblez, L., Landwehrmeyer, B. G., Bak, T., Uttner, I., Dubois, B., et al. (2010). Cognitive impairment in patients with multiple system atrophy and progressive supranuclear palsy. Brain, 133(8), 2382–2393.PubMedCrossRefGoogle Scholar
  23. Bruns, M. B., & Josephs, K. A. (2013). Neuropsychiatry of corticobasal degeneration and progressive supranuclear palsy. International Review of Psychiatry, 25(2), 197–209.PubMedCrossRefGoogle Scholar
  24. Burrell, J. R., Hodges, J. R., & Rowe, J. B. (2014). Cognition in corticobasal syndrome and progressive supranuclear palsy: A review. Movement Disorders, 29(5), 684–693.PubMedCrossRefGoogle Scholar
  25. Butters, N., Granholm, E., Salmon, D. P., Grant, I., & Wolfe, J. (1987). Episodic and semantic memory: A comparison of amnesic and demented patients. Journal of Clinical and Experimental Neuropsychology, 9(5), 479–497.PubMedCrossRefGoogle Scholar
  26. Chan, D., Fox, N. C., Scahill, R. I., Crum, W. R., Whitwell, J. L., Leschziner, G., Rossor, A. M., Stevens, J. M., Cipolotti, L., & Rossor, M. N. (2001). Patterns of temporal lobe atrophy in semantic dementia and Alzheimer’s disease. Annals of Neurology, 49(4), 433–442.PubMedCrossRefGoogle Scholar
  27. Chiong, W., Wood, K. A., Beagle, A. J., Hsu, M., Kayser, A. S., Miller, B. L., & Kramer, J. H. (2016). Neuroeconomic dissociation of semantic dementia and behavioural variant frontotemporal dementia. Brain, 139(Pt 2), 578–587.PubMedCrossRefGoogle Scholar
  28. Chow, T. W., Binns, M. A., Cummings, J. L., Lam, I., Black, S. E., Miller, B. L., Freedman, M., Stuss, D. T., & van Reekum, R. (2009). Apathy symptom profile and behavioral associations in frontotemporal dementia vs dementia of Alzheimer type. Archives of Neurology, 66(7), 888–893.PubMedPubMedCentralCrossRefGoogle Scholar
  29. Davies, R. R., Kipps, C. M., Mitchell, J., Kril, J. J., Halliday, G. M., & Hodges, J. R. (2006). Progression in frontotemporal dementia: Identifying a benign behavioral variant by magnetic resonance imaging. Archives of Neurology, 63(11), 1627–1631.PubMedCrossRefGoogle Scholar
  30. Davis, M. H. (1983). Measuring individual-differences in empathy – evidence for a multidimensional approach. Journal of Personality and Social Psychology, 44, 113–126.CrossRefGoogle Scholar
  31. de Souza, L. C., Volle, E., Bertoux, M., Czernecki, V., Funkiewiez, A., Allali, G., Leroy, B., Sarazin, M., Habert, M. O., Dubois, B., Kas, A., & Levy, R. (2010). Poor creativity in frontotemporal dementia: A window into the neural bases of the creative mind. Neuropsychologia, 48(13), 3733–3742.PubMedCrossRefGoogle Scholar
  32. de Souza, L. C., Chupin, M., Bertoux, M., Lehéricy, S., Dubois, B., Lamari, F., Le Ber, I., Bottlaender, M., Colliot, O., & Sarazin, M. (2013). Is hippocampal volume a good marker to differentiate Alzheimer’s disease from frontotemporal dementia? Journal of Alzheimer’s Disease, 36(1), 57–66.PubMedGoogle Scholar
  33. DeJesus-Hernandez, M., Mackenzie, I. R., Boeve, B. F., Boxer, A. L., Baker, M., Rutherford, N. J., et al. (2011). Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron, 72(2), 245–256.PubMedPubMedCentralCrossRefGoogle Scholar
  34. Desgranges, B., Matuszewski, V., Piolino, P., Chételat, G., Mézenge, F., Landeau, B., de la Sayette, V., Belliard, S., & Eustache, F. (2007). Anatomical and functional alterations in semantic dementia: A voxel-based MRI and PET study. Neurobiology of Aging, 28(12), 1904–1913.PubMedCrossRefGoogle Scholar
  35. Dickson, D. W., Ahmed, Z., Algom, A. A., Tsuboi, Y., & Josephs, K. A. (2010). Neuropathology of variants of progressive supranuclear palsy. Current Opinion in Neurology, 23(4), 394–400.PubMedCrossRefGoogle Scholar
  36. Dickson, D. W., Kouri, N., Murray, M. E., & Josephs, K. A. (2011). Neuropathology of frontotemporal lobar degeneration-tau (FTLD-tau). Journal of Molecular Neuroscience, 45(3), 384–389.PubMedPubMedCentralCrossRefGoogle Scholar
  37. Duval, C., Bejanin, A., Piolino, P., Laisney, M., de La Sayette, V., Belliard, S., Eustache, F., & Desgranges, B. (2012). Theory of mind impairments in patients with semantic dementia. Brain, 135(Pt 1), 228–241.PubMedPubMedCentralCrossRefGoogle Scholar
  38. Edwards-Lee, T., Miller, B. L., Benson, D. F., Cummings, J. L., Russell, G. L., Boone, K., & Mena, I. (1997). The temporal variant of frontotemporal dementia. Brain, 120(Pt 6), 1027–1040.PubMedCrossRefGoogle Scholar
  39. Eslinger, P. J., Dennis, K., Moore, P., Antani, S., Hauck, R., & Grossman, M. (2005). Metacognitive deficits in frontotemporal dementia. Journal of Neurology, Neurosurgery, and Psychiatry, 76(12), 1630–1635.PubMedPubMedCentralCrossRefGoogle Scholar
  40. Evans, J. J., Heggs, A. J., Antoun, N., & Hodges, J. R. (1995). Progressive prosopagnosia associated with selective right temporal lobe atrophy. A new syndrome? Brain, 118(Pt 1), 1–13.PubMedCrossRefGoogle Scholar
  41. Flaherty-Craig, C., Eslinger, P., Stephens, B., & Simmons, Z. (2006). A rapid screening battery to identify frontal dysfunction in patients with ALS. Neurology, 67(11), 2070–2072.PubMedCrossRefGoogle Scholar
  42. Fletcher, P. D., Downey, L. E., Golden, H. L., Clark, C. N., Slattery, C. F., Paterson, R. W., Schott, J. M., Rohrer, J. D., Rossor, M. N., & Warren, J. D. (2015). Auditory hedonic phenotypes in dementia: A behavioural and neuroanatomical analysis. Cortex, 67, 95–105.PubMedPubMedCentralCrossRefGoogle Scholar
  43. Galantucci, S., Tartaglia, M. C., Wilson, S. M., Henry, M. L., Filippi, M., Agosta, F., et al. (2011). White matter damage in primary progressive aphasias: A diffusion tensor tractography study. Brain, 134(Pt 10), 3011–3029.PubMedPubMedCentralCrossRefGoogle Scholar
  44. Galton, C. J., Patterson, K., Graham, K., Lambon-Ralph, M. A., Williams, G., Antoun, N., Sahakian, B. J., & Hodges, J. R. (2001). Differing patterns of temporal atrophy in Alzheimer’s disease and semantic dementia. Neurology, 57(2), 216–225.PubMedCrossRefGoogle Scholar
  45. Golden, H. L., Downey, L. E., Fletcher, P. D., Mahoney, C. J., Schott, J. M., Mummery, C. J., Crutch, S. J., & Warren, J. D. (2015). Identification of environmental sounds and melodies in syndromes of anterior temporal lobe degeneration. Journal of Neurological Sciences, 352(1–2), 94–98.CrossRefGoogle Scholar
  46. Goodglass, H., & Kaplan, E. (1983). The assessment of aphasia and related disorders. Philadelphia, PA: Lea & Febiger.Google Scholar
  47. Gorno-Tempini, M. L., Dronkers, N. F., Rankin, K. P., Ogar, J. M., Phengrasamy, L., Rosen, H. J., Johnson, J. K., Weiner, M. W., & Miller, B. L. (2004). Cognition and anatomy in three variants of primary progressive aphasia. Annals of Neurology, 55(3), 335–346.PubMedPubMedCentralCrossRefGoogle Scholar
  48. Gorno-Tempini, M., Brambati, S., Ginex, V., Ogar, J., Dronkers, N., Marcone, A., et al. (2008). The logopenic/phonological variant of primary progressive aphasia. Neurology, 71(16), 1227–1234.PubMedPubMedCentralCrossRefGoogle Scholar
  49. Gorno-Tempini, M. L., Hillis, A. E., Weintraub, S., Kertesz, A., Mendez, M., Cappa, S. F., Ogar, J. M., Rohrer, J. D., Black, S., Boeve, B. F., Manes, F., Dronkers, N. F., Vandenberghe, R., Rascovsky, K., Patterson, K., Miller, B. L., Knopman, D. S., Hodges, J. R., Mesulam, M. M., & Grossman, M. (2011). Classification of primary progressive aphasia and its variants. Neurology, 76(11), 1006–1014.PubMedPubMedCentralCrossRefGoogle Scholar
  50. Graham, N. L., Bak, T., & Hodges, J. R. (2003). Corticobasal degeneration as a cognitive disorder. Movement Disorders, 18(11), 1224–1232.PubMedCrossRefGoogle Scholar
  51. Graham, N. L., Patterson, K., & Hodges, J. R. (2004). When more yields less: Speaking and writing deficits in nonfluent progressive aphasia. Neurocase, 10(2), 141–155.PubMedCrossRefGoogle Scholar
  52. Gregory, C. A., & Hodges, J. R. (1996). Clinical features of frontal lobe dementia in comparison to Alzheimer’s disease. Journal of Neural Transmission, Supplement, 47, 103–123.CrossRefGoogle Scholar
  53. Gregory, C., Lough, S., Stone, V., Erzinclioglu, S., Martin, L., Baron-Cohen, S., & Hodges, J. R. (2002). Theory of mind in patients with frontal variant frontotemporal dementia and Alzheimer’s disease: Theoretical and practical implications. Brain, 125(Pt 4), 752–764.PubMedCrossRefGoogle Scholar
  54. Grossman, M. (2012). The non-fluent/agrammatic variant of primary progressive aphasia. The Lancet Neurology, 11(6), 545–555.PubMedPubMedCentralCrossRefGoogle Scholar
  55. Grossman, M., Mickanin, J., Onishi, K., Hughes, E., D’Esposito, M., Ding, X.-S., et al. (1996). Progressive nonfluent aphasia: Language, cognitive, and PET measures contrasted with probable Alzheimer’s disease. Journal of Cognitive Neuroscience, 8(2), 135–154.PubMedCrossRefGoogle Scholar
  56. Grossman, M., Libon, D. J., Forman, M. S., Massimo, L., Wood, E., Moore, P., Anderson, C., Farmer, J., Chatterjee, A., Clark, C. M., Coslett, H. B., Hurtig, H. I., Lee, V. M., & Trojanowski, J. Q. (2007). Distinct antemortem profiles in patients with pathologically defined frontotemporal dementia. Archives of Neurology, 64(11), 1601–1609.PubMedCrossRefGoogle Scholar
  57. Gunawardena, D., Ash, S., McMillan, C., Avants, B., Gee, J., & Grossman, M. (2010). Why are patients with progressive nonfluent aphasia nonfluent? Neurology, 75(7), 588–594.PubMedPubMedCentralCrossRefGoogle Scholar
  58. Harciarek, M., & Jodzio, K. (2005). Neuropsychological differences between frontotemporal dementia and Alzheimer’s disease: A review. Neuropsychology Review, 15(3), 131–145.PubMedCrossRefGoogle Scholar
  59. Hardy, C. J., Buckley, A. H., Downey, L. E., Lehmann, M., Zimmerer, V. C., Varley, R. A., Crutch, S. J., Rohrer, J. D., Warrington, E. K., & Warren, J. D. (2015). The language profile of behavioral variant frontotemporal dementia. Journal of Alzheimer’s Disease, 50(2), 359–371.PubMedCentralCrossRefGoogle Scholar
  60. Hauw, J.-J., Daniel, S. E., Dickson, D., Horoupian, D. S., Jellinger, K., Lantos, P. L., et al. (1994). Preliminary NINDS neuropathologic criteria for Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology, 44(11), 2015–2019.PubMedCrossRefGoogle Scholar
  61. Henry, J. D., Phillips, L. H., & von Hippel, C. (2014). A meta-analytic review of theory of mind difficulties in behavioural-variant frontotemporal dementia. Neuropsychologia, 56, 53–62.PubMedCrossRefGoogle Scholar
  62. Hodges, J. R. (2006). Alzheimer’s centennial legacy: Origins, landmarks and the current status of knowledge concerning cognitive aspects. Brain, 129, 2811–2822.PubMedCrossRefGoogle Scholar
  63. Hodges, J. R. (2011). Overview of frontotemporal dementia. In J. R. Hodges (Ed.), Frontotemporal dementia syndromes. Cambridge, UK: Cambridge University Press.Google Scholar
  64. Hodges, J. R., & Patterson, K. (1996). Non-fluent progressive aphasia and semantic dementia: A comparative neuropsychological study. Journal of the International Neuropsychological Society, 2, 511–524.PubMedCrossRefGoogle Scholar
  65. Hodges, J. R., & Patterson, K. (2007). Semantic dementia: A unique clinicopathological syndrome. Lancet Neurology, 6(11), 1004–1014.PubMedCrossRefGoogle Scholar
  66. Hodges, J. R., Patterson, K., Oxbury, S., & Funnell, E. (1992). Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy. Brain, 115(Pt 6), 1783–1806.PubMedCrossRefGoogle Scholar
  67. Hodges, J. R., Patterson, K., Ward, R., Garrard, P., Bak, T., Perry, R., & Gregory, C. (1999). The differentiation of semantic dementia and frontal lobe dementia (temporal and frontal variants of frontotemporal dementia) from early Alzheimer's disease: A comparative neuropsychological study. Neuropsychology, 13(1), 31–40.PubMedCrossRefGoogle Scholar
  68. Hodges, J. R., Davies, R., Xuereb, J., Kril, J., & Halliday, G. (2003). Survival in frontotemporal dementia. Neurology, 61(3), 349–354.PubMedCrossRefGoogle Scholar
  69. Hodges, J. R., Davies, R. R., Xuereb, J. H., Casey, B., Broe, M., Bak, T. H., Kril, J. J., & Halliday, G. M. (2004). Clinicopathological correlates in frontotemporal dementia. Annals of Neurology, 56(3), 399–406.PubMedCrossRefGoogle Scholar
  70. Hornberger, M., Shelley, B. P., Kipps, C. M., Piguet, O., & Hodges, J. R. (2009). Can progressive and non-progressive behavioural variant frontotemporal dementia be distinguished at presentation? Journal of Neurology, Neurosurgery, and Psychiatry, 80(6), 591–593.PubMedCrossRefGoogle Scholar
  71. Hornberger, M., Piguet, O., Graham, A. J., Nestor, P. J., & Hodges, J. R. (2010). How preserved is episodic memory in behavioral variant frontotemporal dementia? Neurology, 74, 472–479.PubMedPubMedCentralCrossRefGoogle Scholar
  72. Hornberger, M., Geng, J., & Hodges, J. R. (2011). Convergent grey and white matter evidence of orbitofrontal cortex changes related to disinhibition in behavioural variant frontotemporal dementia. Brain, 134(Pt 9), 2502–2512.PubMedCrossRefGoogle Scholar
  73. Hornberger, M., Wong, S., Tan, R., Irish, M., Piguet, O., Kril, J., Hodges, J. R., & Halliday, G. (2012). In vivo and post-mortem memory circuit integrity in frontotemporal dementia and Alzheimer’s disease. Brain, 135(Pt 10), 3015–3025.PubMedCrossRefGoogle Scholar
  74. Hornberger, M., Yew, B., Gilardoni, S., Mioshi, E., Gleichgerrcht, E., Manes, F., & Hodges, J. R. (2014). Ventromedial-frontopolar prefrontal cortex atrophy correlates with insight loss in frontotemporal dementia and Alzheimer’s disease. Human Brain Mapping, 35(2), 616–626.PubMedCrossRefGoogle Scholar
  75. Hsieh, S., Hornberger, M., Piguet, O., & Hodges, J. R. (2012). Brain correlates of musical and facial emotion recognition: Evidence from the dementias. Neuropsychologia, 50(8), 1814–1822.PubMedCrossRefGoogle Scholar
  76. Irish, M., Hornberger, M., Lah, S., Miller, L., Pengas, G., Nestor, P. J., Hodges, J. R., & Piguet, O. (2011). Profiles of recent autobiographical memory retrieval in semantic dementia, behavioural-variant frontotemporal dementia, and Alzheimer’s disease. Neuropsychologia, 49(9), 2694–2702.PubMedCrossRefGoogle Scholar
  77. Irish, M., Hodges, J. R., & Piguet, O. (2014). Right anterior temporal lobe dysfunction underlies theory of mind impairments in semantic dementia. Brain, 137(Pt 4), 1241–1253.PubMedCrossRefGoogle Scholar
  78. Irish, M., Bunk, S., Tu, S., Kamminga, J., Hodges, J. R., Hornberger, M., & Piguet, O. (2016). 4.Preservation of episodic memory in semantic dementia: The importance of regions beyond the medial temporal lobes. Neuropsychologia, 81, 50–60.PubMedCrossRefGoogle Scholar
  79. Jack, C. R. (2012). Alzheimer disease: New concepts on its neurobiology and the clinical role imaging will play. Radiology, 263(2), 344–361.PubMedPubMedCentralCrossRefGoogle Scholar
  80. Jefferies, E., Patterson, K., & Ralph, M. A. (2008). Deficits of knowledge versus executive control in semantic cognition: Insights from cued naming. Neuropsychologia, 46(2), 649–658.PubMedCrossRefGoogle Scholar
  81. Jeong, Y., Cho, S. S., Park, J. M., Kang, S. J., Lee, J. S., Kang, E., Na, D. L., & Kim, S. E. (2005). 18F-FDG PET findings in frontotemporal dementia: An SPM analysis of 29 patients. Journal of Nuclear Medicine, 46(2), 233–239.PubMedGoogle Scholar
  82. Johnen, A., Frommeyer, J., Modes, F., Wiendl, H., Duning, T., & Lohmann, H. (2015). Dementia Apraxia Test (DATE): A brief tool to differentiate behavioral variant frontotemporal dementia from Alzheimer’s dementia based on Apraxia profiles. Journal of Alzheimer’s Disease, 49(3), 593–605.CrossRefGoogle Scholar
  83. Johnson, J. K., Diehl, J., Mendez, M. F., Neuhaus, J., Shapira, J. S., Forman, M., et al. (2005). Frontotemporal lobar degeneration: Demographic characteristics of 353 patients. Archives of Neurology, 62(6), 925–930.PubMedCrossRefGoogle Scholar
  84. Josephs, K. A., Duffy, J. R., Strand, E. A., Whitwell, J. L., Layton, K. F., Parisi, J. E., et al. (2006). Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Brain, 129(6), 1385–1398.PubMedPubMedCentralCrossRefGoogle Scholar
  85. Josephs, K. A., Hodges, J. R., Snowden, J. S., Mackenzie, I. R., Neumann, M., Mann, D. M., et al. (2011). Neuropathological background of phenotypical variability in frontotemporal dementia. Acta Neuropathologica, 122(2), 137–153.PubMedPubMedCentralCrossRefGoogle Scholar
  86. Kamminga, J., Kumfor, F., Burrell, J. R., Piguet, O., Hodges, J. R., & Irish, M. (2015). Differentiating between right-lateralised semantic dementia and behavioural-variant frontotemporal dementia: An examination of clinical characteristics and emotion processing. Journal of Neurology, Neurosurgery, and Psychiatry, 86(10), 1082–1088.PubMedCrossRefGoogle Scholar
  87. Kertesz, A. (1982). Western aphasia battery test manual. San Antonio, TX: Psychological Corp.Google Scholar
  88. Kertesz, A., & McMonagle, P. (2010). Behavior and cognition in corticobasal degeneration and progressive supranuclear palsy. Journal of the Neurological Sciences, 289(1), 138–143.PubMedCrossRefGoogle Scholar
  89. Kertesz, A., Martinez-Lage, P., Davidson, W., & Munoz, D. (2000). The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia. Neurology, 55(9), 1368–1375.PubMedCrossRefGoogle Scholar
  90. Kertesz, A., McMonagle, P., Blair, M., Davidson, W., & Munoz, D. G. (2005). The evolution and pathology of frontotemporal dementia. Brain, 128(9), 1996–2005.PubMedCrossRefGoogle Scholar
  91. Kiernan, M. C., Vucic, S., Cheah, B. C., Turner, M. R., Eisen, A., Hardiman, O., et al. (2011). Amyotrophic lateral sclerosis. The Lancet, 377(9769), 942–955.CrossRefGoogle Scholar
  92. Kobylecki, C., Jones, M., Thompson, J. C., Richardson, A. M., Neary, D., Mann, D. M. A., et al. (2015). Cognitive–behavioural features of progressive supranuclear palsy syndrome overlap with frontotemporal dementia. Journal of Neurology, 262(4), 916–922.PubMedCrossRefGoogle Scholar
  93. Kopelman, M. D. (1985). Rates of forgetting in Alzheimer-type dementia and Korsakoff’s syndrome. Neuropsychologia, 23(5), 623–638.PubMedCrossRefGoogle Scholar
  94. Kouri, N., Murray, M. E., Hassan, A., Rademakers, R., Uitti, R. J., Boeve, B. F., et al. (2011). Neuropathological features of corticobasal degeneration presenting as corticobasal syndrome or Richardson syndrome. Brain, 134(Pt 11), 3264–3275.PubMedPubMedCentralCrossRefGoogle Scholar
  95. La Joie, R., Perrotin, A., de La Sayette, V., Egret, S., Doeuvre, L., Belliard, S., Eustache, F., Desgranges, B., & Chételat, G. (2013). Hippocampal subfield volumetry in mild cognitive impairment, Alzheimer’s disease and semantic dementia. Neuroimage Clinical, 3, 155–162.PubMedPubMedCentralCrossRefGoogle Scholar
  96. Laisney, M., Matuszewski, V., Mézenge, F., Belliard, S., de la Sayette, V., Eustache, F., & Desgranges, B. (2009). The underlying mechanisms of verbal fluency deficit in frontotemporal dementia and semantic dementia. Journal of Neurology, 256(7), 1083–1094.PubMedCrossRefGoogle Scholar
  97. Lavenu, I., Pasquier, F., Lebert, F., Petit, H., & Van der Linden, M. (1999). Perception of emotion in frontotemporal dementia and Alzheimer disease. Alzheimer Disease and Associated Disorders, 13(2), 96–101.PubMedCrossRefGoogle Scholar
  98. Le Ber, I. (2013). Genetics of frontotemporal lobar degeneration: An up-date and diagnosis algorithm. Rev Neurol (Paris), 169(10), 811–819.CrossRefGoogle Scholar
  99. Le Ber, I., Guedj, E., Gabelle, A., Verpillat, P., Volteau, M., Thomas-Anterion, C., Decousus, M., Hannequin, D., Véra, P., Lacomblez, L., Camuzat, A., Didic, M., Puel, M., Lotterie, J. A., Golfier, V., Bernard, A. M., Vercelletto, M., Magne, C., Sellal, F., Namer, I., Michel, B. F., Pasquier, J., Salachas, F., Bochet, J., French research network on FTD/FTD-MND, Brice, A., Habert, M. O., & Dubois, B. (2006). Demographic, neurological and behavioural characteristics and brain perfusion SPECT in frontal variant of frontotemporal dementia. Brain, 129(Pt 11), 3051–3065.PubMedCrossRefGoogle Scholar
  100. Leyton, C. E., Villemagne, V. L., Savage, S., Pike, K. E., Ballard, K. J., Piguet, O., et al. (2011). Subtypes of progressive aphasia: Application of the international consensus criteria and validation using β-amyloid imaging. Brain, 134(10), 3030–3043.PubMedCrossRefGoogle Scholar
  101. Leyton, C. E., Piguet, O., Savage, S., Burrell, J., & Hodges, J. R. (2012). The neural basis of logopenic progressive aphasia. Journal of Alzheimer’s Disease, 32(4), 1051.PubMedGoogle Scholar
  102. Leyton, C. E., Hsieh, S., Mioshi, E., & Hodges, J. R. (2013). Cognitive decline in logopenic aphasia more than losing words. Neurology, 80(10), 897–903.PubMedCrossRefGoogle Scholar
  103. Leyton, C. E., Savage, S., Irish, M., Schubert, S., Piguet, O., Ballard, K. J., et al. (2014). Verbal repetition in primary progressive aphasia and Alzheimer’s disease. Journal of Alzheimer’s Disease, 41(2), 575–585.PubMedGoogle Scholar
  104. Libon, D. J., Xie, S. X., Moore, P., Farmer, J., Antani, S., McCawley, G., Cross, K., & Grossman, M. (2007). Patterns of neuropsychological impairment in frontotemporal dementia. Neurology, 68(5), 369–375.PubMedCrossRefGoogle Scholar
  105. Lillo, P., Mioshi, E., Burrell, J. R., Kiernan, M. C., Hodges, J. R., & Hornberger, M. (2012a). Grey and white matter changes across the amyotrophic lateral sclerosis-frontotemporal dementia continuum. PLoS One, 7(8), e43993.PubMedPubMedCentralCrossRefGoogle Scholar
  106. Lillo, P., Savage, S., Mioshi, E., Kiernan, M. C., & Hodges, J. R. (2012b). Amyotrophic lateral sclerosis and frontotemporal dementia: A behavioural and cognitive continuum. Amyotrophic Lateral Sclerosis, 13(1), 102–109.PubMedCrossRefGoogle Scholar
  107. Lindau, M., Almkvist, O., Kushi, J., Boone, K., Johansson, S. E., Wahlund, L. O., Cummings, J. L., & Miller, B. L. (2000). First symptoms--frontotemporal dementia versus Alzheimer’s disease. Dementia and Geriatric Cognitive Disorders, 11(5), 286–293.PubMedCrossRefGoogle Scholar
  108. Litvan, I., Agid, Y., Calne, D., Campbell, G., Dubois, B., Duvoisin, R. C., et al. (1996a). Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): Report of the NINDS-SPSP International Workshop*. Neurology, 47(1), 1–9.PubMedCrossRefGoogle Scholar
  109. Litvan, I., Mega, M., Cummings, J., & Fairbanks, L. (1996b). Neuropsychiatric features of progressive supranuclear palsy. Neurology, 47, 1184–1189.PubMedCrossRefGoogle Scholar
  110. Lomen-Hoerth, C., Anderson, T., & Miller, B. (2002). The overlap of amyotrophic lateral sclerosis and frontotemporal dementia. Neurology, 59(7), 1077–1079.PubMedCrossRefGoogle Scholar
  111. Lough, S., Kipps, C. M., Treise, C., Watson, P., Blair, J. R., Hodges, J. R., Lough, S., Kipps, C. M., Treise, C., Watson, P., Blair, J. R., & Hodges, J. R. (2006). Social reasoning, emotion and empathy in frontotemporal dementia. Neuropsychologia, 44(6), 950–958.PubMedCrossRefGoogle Scholar
  112. Lu, P. H., Khanlou, N., & Cummings, J. L. (2006). Frontotemporal dementia and the orbitofrontal cortex. In D. H. Zald & S. L. Rauch (Eds.), The orbitofrontal cortex. New York: Oxford Press.Google Scholar
  113. Luzzi, S., Cafazzo, V., Damora, A., Fabi, K., Fringuelli, F. M., Ascoli, G., Silvestrini, M., Provinciali, L., & Reverberi, C. (2015). The neural correlates of road sign knowledge and route learning in semantic dementia and Alzheimer’s disease. Journal of Neurology, Neurosurgery, and Psychiatry, 86(6), 595–602.PubMedCrossRefGoogle Scholar
  114. Mackenzie, I. R., Rademakers, R., & Neumann, M. (2010). TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia. Lancet Neurology, 9(10), 995–1007.PubMedCrossRefGoogle Scholar
  115. Mackenzie, I. R., Neumann, M., Baborie, A., Sampathu, D. M., Du Plessis, D., Jaros, E., Perry, R. H., Trojanowski, J. Q., Mann, D. M., & Lee, V. M. (2011). A harmonized classification system for FTLD-TDP pathology. Acta Neuropathologica, 122(1), 111–113.PubMedPubMedCentralCrossRefGoogle Scholar
  116. Mahoney, C. J., Simpson, I. J., Nicholas, J. M., Fletcher, P. D., Downey, L. E., Golden, H. L., Clark, C. N., Schmitz, N., Rohrer, J. D., Schott, J. M., Zhang, H., Ourselin, S., Warren, J. D., & Fox, N. C. (2015). Longitudinal diffusion tensor imaging in frontotemporal dementia. Annals of Neurology, 77(1), 33–46.PubMedCrossRefGoogle Scholar
  117. Manes, F. F., Torralva, T., Roca, M., Gleichgerrcht, E., Bekinschtein, T. A., & Hodges, J. R. (2010). Frontotemporal dementia presenting as pathological gambling. Nature Reviews Neurology, 6(6), 347–352.PubMedCrossRefGoogle Scholar
  118. McMonagle, P., Blair, M., & Kertesz, A. (2006). Corticobasal degeneration and progressive aphasia. Neurology, 67(8), 1444–1451.PubMedCrossRefGoogle Scholar
  119. Mesulam, M. M. (2001). Primary progressive aphasia. Annals of Neurology, 49, 425–432.PubMedCrossRefGoogle Scholar
  120. Mesulam, M., Wicklund, A., Johnson, N., Rogalski, E., Léger, G. C., Rademaker, A., et al. (2008). Alzheimer and frontotemporal pathology in subsets of primary progressive aphasia. Annals of Neurology, 63(6), 709–719.PubMedPubMedCentralCrossRefGoogle Scholar
  121. Mioshi, E., Lillo, P., Yew, B., Hsieh, S., Savage, S., Hodges, J. R., et al. (2013). Cortical atrophy in ALS is critically associated with neuropsychiatric and cognitive changes. Neurology, 80(12), 1117–1123.PubMedCrossRefGoogle Scholar
  122. Mitchell, J. D., & Borasio, G. D. (2007). Amyotrophic lateral sclerosis. The Lancet, 369(9578), 2031–2041.CrossRefGoogle Scholar
  123. Neary, D., Snowden, J. S., Bowen, D. M., Sims, N. R., Mann, D. M., Yates, P. O., & Davison, A. N. (1986). Cerebral biopsy in the investigation of presenile dementia due to cerebral atrophy. Journal of Neurology, Neurosurgery, and Psychiatry, 49(2), 157–162.PubMedPubMedCentralCrossRefGoogle Scholar
  124. 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., & Benson, D. F. (1998). Frontotemporal lobar degeneration: A consensus on clinical diagnostic criteria. Neurology, 51(6), 1546–1554.PubMedCrossRefGoogle Scholar
  125. Nestor, P. J., Graham, N. L., Fryer, T. D., Williams, G. B., Patterson, K., & Hodges, J. R. (2003). Progressive non-fluent aphasia is associated with hypometabolism centred on the left anterior insula. Brain, 126, 2406–2618.PubMedCrossRefGoogle Scholar
  126. Neumann, M., Sampathu, D. M., Kwong, L. K., Truax, A. C., Micsenyi, M. C., Chou, T. T., et al. (2006). Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science, 314(5796), 130–133.PubMedCrossRefGoogle Scholar
  127. 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), S23–S30.CrossRefGoogle Scholar
  128. Peelle, J. E., Troiani, V., Gee, J., Moore, P., McMillan, C., Vesely, L., et al. (2008). Sentence comprehension and voxel-based morphometry in progressive nonfluent aphasia, semantic dementia, and nonaphasic frontotemporal dementia. Journal of Neurolinguistics, 21(5), 418–432.PubMedPubMedCentralCrossRefGoogle Scholar
  129. Pengas, G., Patterson, K., Arnold, R. J., Bird, C. M., Burgess, N., & Nestor, P. J. (2010). Lost and found: Bespoke memory testing for Alzheimer’s disease and semantic dementia. Journal of Alzheimer’s Disease, 21(4), 1347–1365.PubMedGoogle Scholar
  130. Perry, R. J., & Hodges, J. R. (2000). Differentiating frontal and temporal variant frontotemporal dementia from Alzheimer’s disease. Neurology, 54(12), 2277–2284.PubMedCrossRefGoogle Scholar
  131. Perry, R. J., Watson, P., & Hodges, J. R. (2000). The nature and staging of attention dysfunction in early (minimal and mild) Alzheimer’s disease: Relationship to episodic and semantic memory impairment. Neuropsychologia, 38(3), 252–271.PubMedCrossRefGoogle Scholar
  132. Perry, R. J., Graham, A., Williams, G., Rosen, H., Erzinçlioglu, S., Weiner, M., Miller, B., & Hodges, J. (2006). Patterns of frontal lobe atrophy in frontotemporal dementia: A volumetric MRI study. Dementia and Geriatric Cognitive Disorders, 22(4), 278–287.PubMedCrossRefGoogle Scholar
  133. Phukan, J., Pender, N. P., & Hardiman, O. (2007). Cognitive impairment in amyotrophic lateral sclerosis. The Lancet Neurology, 6(11), 994–1003.PubMedCrossRefGoogle Scholar
  134. Pick, A. (1892). Uber die Beziehunge der senilen Hirnatrophie zur Aphasie. Prager Medische Wochenschrift, 17, 165–167.Google Scholar
  135. Piguet, O., Hornberger, M., Mioshi, E., & Hodges, J. R. (2011a). Behavioural-variant frontotemporal dementia: Diagnosis, clinical staging, and management. Lancet Neurology, 10(2), 162–172. doi: 10.1016/S1474-4422(10)70299-4.Google Scholar
  136. Piguet, O., Petersén, A., Yin Ka Lam, B., Gabery, S., Murphy, K., Hodges, J. R., & Halliday, G. M. (2011b). Eating and hypothalamus changes in behavioral-variant frontotemporal dementia. Annals of Neurology, 69(2), 312–319.PubMedPubMedCentralCrossRefGoogle Scholar
  137. 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., & Gorno-Tempini, M. L. (2008). Abeta amyloid and glucose metabolism in three variants of primary progressive aphasia. Annals of Neurology, 64(4), 388–401.PubMedPubMedCentralCrossRefGoogle Scholar
  138. Rabinovici, G. D., Rosen, H. J., Alkalay, A., Kornak, J., Furst, A. J., Agarwal, N., Mormino, E. C., O’Neil, J. P., Janabi, M., Karydas, A., Growdon, M. E., Jang, J. Y., Huang, E. J., Dearmond, S. J., Trojanowski, J. Q., Grinberg, L. T., Gorno-Tempini, M. L., Seeley, W. W., Miller, B. L., & Jagust, W. J. (2011). Amyloid vs FDG-PET in the differential diagnosis of AD and FTLD. Neurology, 77(23), 2034–2042.PubMedPubMedCentralCrossRefGoogle Scholar
  139. Rahman, S., Sahakian, B. J., Hodges, J. R., Rogers, R. D., & Robbins, T. W. (1999). Specific cognitive deficits in mild frontal variant frontotemporal dementia. Brain, 122(Pt 8), 1469–1493.PubMedCrossRefGoogle Scholar
  140. Rankin, K. P., Salazar, A., Gorno-Tempini, M. L., Sollberger, M., Wilson, S. M., Pavlic, D., Stanley, C. M., Glenn, S., Weiner, M. W., & Miller, B. L. (2009). Detecting sarcasm from paralinguistic cues: Anatomic and cognitive correlates in neurodegenerative disease. NeuroImage, 47(4), 2005–2015.PubMedPubMedCentralCrossRefGoogle Scholar
  141. Rascovsky, K., Hodges, J. R., Knopman, D., Mendez, M. F., Kramer, J. H., Neuhaus, J., van Swieten, J. C., Seelaar, H., Dopper, E. G., Onyike, C. U., Hillis, A. E., Josephs, K. A., Boeve, B. F., Kertesz, A., Seeley, W. W., Rankin, K. P., Johnson, J. K., Gorno-Tempini, M. L., Rosen, H., Prioleau-Latham, C. E., Lee, A., Kipps, C. M., Lillo, P., Piguet, O., Rohrer, J. D., Rossor, M. N., Warren, J. D., Fox, N. C., Galasko, D., Salmon, D. P., Black, S. E., Mesulam, M., Weintraub, S., Dickerson, B. C., Diehl-Schmid, J., Pasquier, F., Deramecourt, V., Lebert, F., Pijnenburg, Y., Chow, T. W., Manes, F., Grafman, J., Cappa, S. F., Freedman, M., Grossman, M., & Miller, B. L. (2011). Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain, 134(Pt 9), 2456–2477.PubMedPubMedCentralCrossRefGoogle Scholar
  142. Ratnavalli, E., Brayne, C., Dawson, K., & Hodges, J. R. (2002). The prevalence of frontotemporal dementia. Neurology, 58(11), 1615–1621.PubMedCrossRefGoogle Scholar
  143. Renton Alan, E., Majounie, E., Waite, A., Simón-Sánchez, J., Rollinson, S., Gibbs, J. R., et al. (2011). A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron, 72(2), 257–268.PubMedPubMedCentralCrossRefGoogle Scholar
  144. Riley, D., Lang, A., Lewis, A., Resch, L., Ashby, P., Hornykiewicz, O., et al. (1990). Cortical-basal ganglionic degeneration. Neurology, 40(8), 1203.PubMedCrossRefGoogle Scholar
  145. Ringholz, G., Appel, S., Bradshaw, M., Cooke, N., Mosnik, D., & Schulz, P. (2005). Prevalence and patterns of cognitive impairment in sporadic ALS. Neurology, 65(4), 586–590.PubMedCrossRefGoogle Scholar
  146. Rogalski, E., Cobia, D., Harrison, T., Wieneke, C., Weintraub, S., & Mesulam, M.-M. (2011). Progression of language decline and cortical atrophy in subtypes of primary progressive aphasia. Neurology, 76(21), 1804–1810.PubMedPubMedCentralCrossRefGoogle Scholar
  147. Rogers, T. T., Patterson, K., Jefferies, E., & Ralph, M. A. (2015). Disorders of representation and control in semantic cognition: Effects of familiarity, typicality, and specificity. Neuropsychologia, 76, 220–239.PubMedPubMedCentralCrossRefGoogle Scholar
  148. Rohrer, J. D., & Warren, J. D. (2010). Phenomenology and anatomy of abnormal behaviours in primary progressive aphasia. Journal of the Neurological Sciences, 293(1), 35–38.PubMedPubMedCentralCrossRefGoogle Scholar
  149. Rohrer, J. D., Ridgway, G. R., Crutch, S. J., Hailstone, J., Goll, J. C., Clarkson, M. J., et al. (2010). Progressive logopenic/phonological aphasia: Erosion of the language network. NeuroImage, 49(1), 984–993.PubMedPubMedCentralCrossRefGoogle Scholar
  150. Rohrer, J. D., Rossor, M. N., & Warren, J. D. (2012). Alzheimer’s pathology in primary progressive aphasia. Neurobiology of Aging, 33(4), 744–752.PubMedPubMedCentralCrossRefGoogle Scholar
  151. Rosen, H. J., Gorno-Tempini, M. L., Goldman, W. P., Perry, R. J., Schuff, N., Weiner, M., Feiwell, R., Kramer, J. H., & Miller, B. L. (2002). Patterns of brain atrophy in frontotemporal dementia and semantic dementia. Neurology, 58(2), 198–208.PubMedCrossRefGoogle Scholar
  152. Savage, S., Hsieh, S., Leslie, F., Foxe, D., Piguet, O., & Hodges, J. R. (2013). Distinguishing subtypes in primary progressive aphasia: Application of the Sydney language battery. Dementia and Geriatric Cognitive Disorders, 35(3–4), 208–218.PubMedCrossRefGoogle Scholar
  153. Savage, S. A., Piguet, O., & Hodges, J. R. (2015). “Knowing what you don’t know”: Language insight in semantic dementia. Journal of Alzheimer’s Disease, 46(1), 187–198.PubMedCrossRefGoogle Scholar
  154. Schwindt, G. C., Graham, N. L., Rochon, E., Tang-Wai, D. F., Lobaugh, N. J., Chow, T. W., et al. (2013). Whole-brain white matter disruption in semantic and nonfluent variants of primary progressive aphasia. Human Brain Mapping, 34(4), 973–984.PubMedCrossRefGoogle Scholar
  155. Seelaar, H., Rohrer, J. D., Pijnenburg, Y. A., Fox, N. C., & van Swieten, J. C. (2011). Clinical, genetic and pathological heterogeneity of frontotemporal dementia: A review. Journal of Neurology, Neurosurgery, and Psychiatry, 82(5), 476–486.PubMedCrossRefGoogle Scholar
  156. Seeley, W. W., Bauer, A. M., Miller, B. L., Gorno-Tempini, M. L., Kramer, J. H., Weiner, M., & Rosen, H. J. (2005). The natural history of temporal variant frontotemporal dementia. Neurology, 64(8), 1384–1390.PubMedPubMedCentralCrossRefGoogle Scholar
  157. Seeley, W. W., Crawford, R., Rascovsky, K., Kramer, J. H., Weiner, M., Miller, B. L., & Gorno-Tempini, M. L. (2008). Frontal paralimbic network atrophy in very mild behavioral variant frontotemporal dementia. Archives of Neurology, 65(2), 249–255.PubMedPubMedCentralCrossRefGoogle Scholar
  158. Sieben, A., Van Langenhove, T., Engelborghs, S., Martin, J. J., Boon, P., Cras, P., De Deyn, P. P., Santens, P., Van Broeckhoven, C., & Cruts, M. (2012). The genetics and neuropathology of frontotemporal lobar degeneration. Acta Neuropathologica, 124(3), 353–372.PubMedPubMedCentralCrossRefGoogle Scholar
  159. Snowden, J. S., Goulding, P. J., & Neary, D. (1989). Semantic dementia: A form of circumscribed cerebral atrophy. Behavioural Neurology, 2, 167–182.Google Scholar
  160. Snowden, J. S., Neary, D., & Mann, D. M. A. (1996). Fronto-temporal lobar degeneration: Fronto-temporal dementia, progressive aphasia, semantic dementia. New York: Churchill-Livingstone.Google Scholar
  161. Snowden, J. S., Bathgate, D., Varma, A., Blackshaw, A., Gibbons, Z. C., & Neary, D. (2001). Distinct behavioural profiles in frontotemporal dementia and semantic dementia. Journal of Neurology, Neurosurgery, and Psychiatry, 70(3), 323–332.PubMedPubMedCentralCrossRefGoogle Scholar
  162. Stopford, C. L., Thompson, J. C., Neary, D., Richardson, A. M., & Snowden, J. S. (2012). Working memory, attention, and executive function in Alzheimer’s disease and frontotemporal dementia. Cortex, 48(4), 429–446.PubMedCrossRefGoogle Scholar
  163. Tan, R. H., Kril, J. J., Fatima, M., McGeachie, A., McCann, H., Shepherd, C., Forrest, S. L., Affleck, A., Kwok, J. B., Hodges, J. R., Kiernan, M. C., & Halliday, G. M. (2015). TDP-43 proteinopathies: Pathological identification of brain regions differentiating clinical phenotypes. Brain, 138(Pt 10), 3110–3122.PubMedCrossRefGoogle Scholar
  164. Thompson, C. K., Ballard, K. J., Tait, M. E., Weintraub, S., & Mesulam, M. (1997). Patterns of language decline in non-fluent primary progressive aphasia. Aphasiology, 11(4–5), 297–321.CrossRefGoogle Scholar
  165. Thompson, S. A., Patterson, K., & Hodges, J. R. (2003). Left/right asymmetry of atrophy in semantic dementia: Behavioral-cognitive implications. Neurology, 61(9), 1196–1203.PubMedCrossRefGoogle Scholar
  166. Tu, S., Wong, S., Hodges, J. R., Irish, M., Piguet, O., & Hornberger, M. (2015). Lost in spatial translation – A novel tool to objectively assess spatial disorientation in Alzheimer’s disease and frontotemporal dementia. Cortex, 67, 83–94.PubMedCrossRefGoogle Scholar
  167. Warren, J. D., Fletcher, P. C., & Golden, H. L. (2012). The paradox of syndromic diversity in Alzheimer disease. Nature Reviews Neurology, 8, 451–464.PubMedGoogle Scholar
  168. Williams, D. R., & Lees, A. J. (2009). Progressive supranuclear palsy: Clinicopathological concepts and diagnostic challenges. Lancet Neurology, 8(3), 270–279.PubMedCrossRefGoogle Scholar
  169. Wilson, S. M., Dronkers, N. F., Ogar, J. M., Jang, J., Growdon, M. E., Agosta, F., et al. (2010a). Neural correlates of syntactic processing in the nonfluent variant of primary progressive aphasia. The Journal of Neuroscience, 30(50), 16845–16854.PubMedPubMedCentralCrossRefGoogle Scholar
  170. Wilson, S. M., Henry, M. L., Besbris, M., Ogar, J. M., Dronkers, N. F., Jarrold, W., et al. (2010b). Connected speech production in three variants of primary progressive aphasia. Brain, 133(7), 2069–2088.PubMedPubMedCentralCrossRefGoogle Scholar
  171. Woolley, J. D., Gorno-Tempini, M. L., Seeley, W. W., Rankin, K., Lee, S. S., Matthews, B. R., & Miller, B. L. (2007). Binge eating is associated with right orbitofrontal-insular-striatal atrophy in frontotemporal dementia. Neurology, 69(14), 1424–1433.PubMedCrossRefGoogle Scholar
  172. Woolley, J. D., Khan, B. K., Murthy, N. K., Miller, B. L., & Rankin, K. P. (2011). The diagnostic challenge of psychiatric symptoms in neurodegenerative disease: Rates of and risk factors for prior psychiatric diagnosis in patients with early neurodegenerative disease. Journal of Clinical Psychiatry, 72(2), 126–133.PubMedPubMedCentralCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media Singapore 2017

Authors and Affiliations

  • Maxime Bertoux
    • 1
    • 2
  • Claire O’Callaghan
    • 3
  • Emma Flanagan
    • 1
  • Michael Hornberger
    • 1
  1. 1.Norwich Medical SchoolUniversity of East AngliaNorfolkUK
  2. 2.Department of Clinical NeurosciencesUniversity of CambridgeCambridgeUK
  3. 3.Behavioral and Clinical Neurosciences InstituteUniversity of CambridgeCambridgeUK