Skip to main content Accessibility help
×
Hostname: page-component-76fb5796d-r6qrq Total loading time: 0 Render date: 2024-04-28T11:54:55.968Z Has data issue: false hasContentIssue false

Section 1 - Introduction to and brief history of FTD

Published online by Cambridge University Press:  05 May 2016

Bradford C. Dickerson
Affiliation:
Department of Neurology, Massachusetts General Hospital
Get access
Type
Chapter
Information
Publisher: Cambridge University Press
Print publication year: 2016

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

References

Alexander, SK, Rittman, T, Xuereb, JH et al. 2014. Validation of the new consensus criteria for the diagnosis of Corticobasal Degeneration. Journal of Neurology, Neurosurgery, and Psychiatry 85:925–9.Google Scholar
Alzheimer, A. 1911. Uber eigenartige Krankheitsfalle des spateren Alters. Zeitschrift für die und Gesellschaft für Neurologie und Psychiatrie 4:356–85.Google Scholar
Bak, T, Hodges, JR. 1997. Noun-verb dissociation in three patients with motor neurone disease and aphasia. Brain and Language 60:3840.Google Scholar
Bak, T, Hodges, JR. 1999. Cognition, language and behaviour in motor neurone disease: evidence of frontotemporal dementia. Dementia and Geriatric Cognitive Disorders 10:2932.Google Scholar
Bak, TH, O'Donovan, DG, Xuereb, JH, Boniface, S, Hodges, J. 2001. Selective impairment of verb processing associated with pathological changes in the Brodmann areas 44 and 45 in the motor neurone disease/dementia/aphasia syndrome. Brain 124:103–20.Google Scholar
Bak, TH, Crawford, LM, Hearn, VC, Mathuranath, PS, Hodges, JR. 2005. Subcortical dementia revisited: similarities and differences in cognitive function between progressive supranuclear palsy (PSP), corticobasal degeneration (CBD) and multiple system atrophy (MSA). Neurocase 11: 268–73.CrossRefGoogle ScholarPubMed
Bak, T, Caine, D, Hearn, VC, Hodges, JR. 2006. Visuospatial functions in atypical parkinsonian syndromes. Journal of Neurology, Neurosurgery, and Psychiatry 77:454–6.CrossRefGoogle ScholarPubMed
Baker, M, Mackenzie, IR, Pickering-Brown, SM et al. 2006. Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17. Nature 24:916–19.Google Scholar
Bertoux, M, Volle, E, Funkiewiez, A et al. 2012. Social Cognition and Emotional Assessment (SEA) is a marker of medial and orbital frontal functions: a voxel-based morphometry study in behavioral variant of frontotemporal degeneration. Journal of the International Neuropsychological Society 18:972–85.Google Scholar
Boeve, BF, Maraganore, DM, Parisi, JE et al. 1999. Pathological heterogeneity in clinically diagnosed corticobasal degeneration. Neurology 53:795800.CrossRefGoogle ScholarPubMed
Bozeat, S, Gregory, CA, Lambon Ralph, MA, Hodges, JR. 2000. Which neuropsychiatric and behavioural features distinguish frontal and temporal variants of frontotemporal dementia from Alzheimer's disease? Journal of Neurology, Neurosurgery, and Psychiatry 69:178–86.CrossRefGoogle ScholarPubMed
Brown, J, Gydesen, S, Sorensen, SA et al. 1993. Genetic characterization of a familial non-specific dementia originating in Jutland, Denmark. Journal of Neurological Sciences 114:138–43.Google Scholar
Brun, A. 1987. Frontal lobe degeneration of non-Alzheimer's type. I. Neuropathology. Archives of Gerontology and Geriatrics 6:209–33.CrossRefGoogle Scholar
Brun, A, Englund, B, Gustafson, L et al. 1994. Clinical and neuropathological criteria for frontotemporal dementia. The Lund Manchester Groups. Journal of Neurology, Neurosurgery, and Psychiatry 57:416–18.Google Scholar
Burrell, J, Kiernan, MC, Vucic, S, Hodges, JR. 2011. Motor neuron dysfunction in frontotemporal dementia. Brain 134(Pt 9):2582–94.CrossRefGoogle ScholarPubMed
Burrell, J, Hornberger, M, Villemagne, V, Rowe, C, Hodges, JR. 2013. Clinical profile of PiB-positive corticobasal syndrome. PLoS One 8(4):e61025.CrossRefGoogle ScholarPubMed
Cannon, A, Baker, M, Boeve, BF et al. 2006. CHMP2B mutations are not a common cause of frontotemporal lobar degeneration. Neuroscience Letters 398:83–4.Google Scholar
Caselli, RJ, Windebank, AJ, Petersen, RC et al. 1993. Rapidly progressive aphasic dementia and motor neuron disease. Annals of Neurology 33:200–7.CrossRefGoogle ScholarPubMed
Chare, L, Hodges, JR, Leyton, CE et al. 2014. New criteria for frontotemporal dementia syndromes: clinical and pathological diagnostic implications. Journal of Neurology, Neurosurgery, and Psychiatry 85(8):865–70.Google Scholar
Cruts, M, Gijselinck, I, Van der Zee, J et al. 2006. Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21. Nature 442:920–4.Google Scholar
Cummings, JL, Mega, M, Gray, K et al. 1994. The neuropsychiatric inventory: comprehensive assessment of psychopathology in dementia. Neurology 44:2308–14.Google Scholar
Davies, RR, Graham, KS, Xuereb, JH, Williams, GB, Hodges, JR. 2004. The human perirhinal cortex and semantic memory. European Journal of Neuroscience 20:2441–6.Google Scholar
Davies, RR, Hodges, JR, Kril, J et al. 2005. The pathological basis of semantic dementia. Brain 128:1984–5.Google Scholar
Davies, RR, Kipps, CM, Mitchell, J et al. 2006. Progression in frontotemporal dementia: identifying a benign behavioral variant by MRI. Archives of Neurology 63: 1627–31.CrossRefGoogle Scholar
DeJesus-Hernandez, M, Mackenzie, IR, Boeve, BF et al. 2011. Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron 72(2):24556.Google Scholar
Devenney, E, Hornberger, M, Irish, M et al. 2014. Frontotemporal dementia associated with the C9ORF72 mutation: a unique clinical profile. JAMA Neurology 71(3):331–9.CrossRefGoogle ScholarPubMed
Dickson, DW, Bergeron, C, Chin, SS et al. 2002. Neuropathologic criteria for corticobasal degeneration. Journal of Neuropathology and Experimental Neurology 61:935–46.Google Scholar
Doran, M, du Plessis, DG, Enevoldson, TP et al. 2003. Pathological heterogeneity of clinically diagnosed corticobasal degeneration. Journal of Neurological Science 216:127–34.CrossRefGoogle ScholarPubMed
Dumanchin, C, Camuzat, A, Campion, D et al. 1998. Segregation of a missense mutation in the microtubule-associated protein tau gene with familial frontotemporal dementia and parkinsonism. Human Molecular Genetics 7:1825–9.Google Scholar
Edwards Lee, T, Miller, B, Benson, F et al. 1997.The temporal variant of frontotemporal dementia. Brain 120:1027–40.Google Scholar
Evans, JJ, Heggs, AJ, Antoun, N, Hodges, JR. 1995. Progressive prosopagnosia associated with selective right temporal lobe atrophy: a new syndrome? Brain 118:113.Google Scholar
Feany, MB, Dickson, DW. 1996. Neurodegenerative disorders with extensive tau pathology: a comparative study and review. Annals of Neurology 40:139–48.CrossRefGoogle Scholar
Ferraro, A, Jervis, GA. 1940. Clinicopathologic study of a case of Pick's disease. Psychiatric Quarterly (New York NY) 17:1729.Google Scholar
Forman, MS, Farmer, J, Johnson, JK et al. 2006. Frontotemporal dementia: clinicopathological correlations. Annals of Neurology 59:952–62.Google Scholar
Foster, NL, Wilhelmsen, K, Sima, AAF et al. 1997. Frontotemporal dementia and parkinsonism linked to chromosome 17: a consensus conference. Annals of Neurology 41:706–15.CrossRefGoogle ScholarPubMed
Gainotti, G, Barber, A, Marra, C. 2003. Slowly progressive defect in recognition of familiar people in a patient with right anterior temporal atrophy. Brain 126:792803.Google Scholar
Galton, CJ, Patterson, K, Graham, KS et al. 2001. Differing patterns of temporal atrophy in Alzheimer's disease and semantic dementia. Neurology 57:216–25.Google Scholar
Garrard, P, Perry, R, Hodges, JR. 1997. Disorders of semantic memory. Journal of Neurology, Neurosurgery, and Psychiatry 62:431–5.Google Scholar
Gentileschi, V, Sperber, S, Spinnler, H. 1999. Progressive defective recognition of familiar people. Neurocase 5:407–24.Google Scholar
Gentileschi, V, Sperber, S, Spinnler, H. 2001. Crossmodal agnosia for familiar people as a consequence of right infero polar temporal atrophy. Cognitive Neuropsychology 18:439–63.Google Scholar
Gibb, WRG, Luthert, PJ, Marsden, CD. 1989. Corticobasal degeneration. Brain 112:1171–92.Google Scholar
Girling, DM, Berrios, GE. 1994. On the relationship between senile cerebral atrophy and aphasia (translation of Pick A. 1892. Über die Beziehungen der senilen Hirnatrophie zur Aphasie. Prager Medicinische Wochenschrift 17:165–7). History of Psychiatry 5:542–7.Google Scholar
Girling, DM, Berrios, GE. 1997. On the symptomatology of left-sided temporal lobe atrophy (translation of Pick A. 1904. Zur Symptomatologie der linksseitigen Schäfenlappenatrophie. Monatschrift für Psychiatrie und Neurologie 16:378–88). History of Psychiatry 8:149–59.Google Scholar
Gorno-Tempini, M, Dronkers, N, Rankin, K et al. 2004. Cognition and anatomy in three variants of primary progressive aphasia. Annals of Neurology 55:335–46.Google Scholar
Gorno-Tempini, ML, Hillis, AE, Weintraub, S et al. 2011. Classification of primary progressive aphasia and its variants. Neurology 76(11):1006–14.CrossRefGoogle ScholarPubMed
Graham, AJ, Davies, R, Xuereb, J et al. 2005. Pathologically proven frontotemporal dementia presenting with severe amnesia. Brain 128:597605.CrossRefGoogle ScholarPubMed
Graham, KS, Hodges, JR. 1997. Differentiating the roles of the hippocampal complex and the neocortex in long-term memory storage: evidence from the study of semantic dementia and Alzheimer's disease. Neuropsychology 11:7789.Google Scholar
Graham, NL, Bak, T, Hodges, JR. 2003a. Corticobasal degeneration as a cognitive disorder. Movement Disorders 18:1224–32.Google Scholar
Graham, NL, Patterson, K, Bak, T, Hodges, JR. 2003b. Language function and dysfunction in corticobasal degeneration. Neurology 61:493–9.Google Scholar
Gregory, CA, Lough, S, Stone, VA et al. 2002. Theory of mind in patients with frontal variant frontotemporal dementia and Alzheimer's disease: theoretical and practical implications. Brain 125:752–64.Google Scholar
Grossman, M. 2002. Frontotemporal dementia: a review. Journal of the International Neurological Society 8:566–83.Google Scholar
Gustafson, L. 1987. Frontal lobe degeneration of non-Alzheimer's type II: clinical picture and differential diagnosis. Archives of Gerontology and Geriatrics 6:209–23.Google Scholar
Heutink, P, Stevens, M, Rizzu, P et al. 1997. Hereditary frontotemporal dementia is linked to chromosome 17q21-q22: a genetic and clinicopathological study of three Dutch families. Annals of Neurology 41:150–9.CrossRefGoogle ScholarPubMed
Hodges, JR. 2012. Familial frontotemporal dementia and amyotrophic lateral sclerosis associated with the C9ORF72 hexanucleotide repeat. Brain 135(Pt 3):652–5.Google Scholar
Hodges, JR, Graham, KS. 1998. A reversal of the temporal gradient for famous person knowledge in semantic dementia: implications for the neural organisation of long-term memory. Neuropsychologia 36:803–25.CrossRefGoogle ScholarPubMed
Hodges, JR, Patterson, K. 1996. Non-fluent progressive aphasia and semantic dementia: a comparative neuropsychological study. Journal of the International Neuropsychological Society 2:511–24.Google Scholar
Hodges, JR, Patterson, KE. 1997. Semantic memory disorders. Trends in Cognitive Science 1:6772.Google Scholar
Hodges, JR, Patterson, K, Oxbury, S, Funnell, E. 1992. Semantic dementia: progressive fluent aphasia with temporal lobe atrophy. Brain 115:1783–806.CrossRefGoogle ScholarPubMed
Hodges, JR, Patterson, K, Tyler, LK. 1994. Loss of semantic memory: implications for the modularity of mind. Cognitive Neuropsychology 11:505–42.Google Scholar
Hodges, JR, Graham, N, Patterson, K. 1995. Charting the progression in semantic dementia: implications for the organisation of semantic memory. Memory 3:463–95.Google Scholar
Hodges, JR, Garrard, P, Patterson, K. 1998. Semantic dementia. In Kertesz, A, Munoz, DG, eds. Pick's Disease and Pick Complex pp. 83104. New York: Wiley-Liss, Inc.Google Scholar
Hodges, JR, Patterson, K, Ward, R et al. 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 13:3140.Google Scholar
Hodges, JR, Davies, R, Xuereb, J et al. 2004. Clinicopathological correlates in frontotemporal dementia. Annals of Neurology 56:399406.Google Scholar
Hodges, JR, Mitchell, J, Dawson, K et al. 2010. Semantic dementia: demography, familial factors and survival in a consecutive series of 100 cases. Brain 133 (Pt 1):300–6.Google Scholar
Hornberger, M, Piguet, O, Kipps, CM, Hodges, JR. 2008. Executive function in progressive and non-progressive behavioural variant frontotemporal dementia. Neurology 71(19):1481–8.Google Scholar
Hornberger, M, Piguet, O, Graham, A, Nestor, PJ, Hodges, JR. 2010. How preserved is episodic memory in behavioral variant frontotemporal dementia? Neurology 74:472–9.Google Scholar
Hornberger, M, Geng, J, Hodges, JR. 2011. Convergent evidence of orbitofrontal cortex grey and white matter changes related to disinhibition in behavioural variant frontotemporal dementia. Brain 134 (Pt 9):2502–12.Google Scholar
Hutton, M, Lendon, CL, Rizzu, P et al. 1998. Association of missense and 5′-splice-site mutations in tau with the inherited dementia FTDP-17. Nature 393:702–5.Google Scholar
Imura, T, Nogami, Y, Asakawa, K. 1971. Aphasia in Japanese language. Nihon University Journal of Medicine 13:6990.Google Scholar
Jackson, M, Lowe, J. 1996. The new neuropathology of degenerative frontotemporal dementias. Acta Neuropathologica 91:127–34.Google Scholar
Josephs, KA, Petersen, RC, Knopman, DS et al. 2006. Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP. Neurology 66:41–8.Google Scholar
Josephs, KA, Hodges, JR, Snowden, JS et al. 2011. Neuropathological background of phenotypical variability in frontotemporal dementia. Acta Neuropathologica 122(2):137–53.Google Scholar
Kamo, H, McGeer, PL, Harrop, R et al. 1987. Positron emission tomography and histopathology in Pick's disease. Neurology 37:439–45.Google Scholar
Katzman, R. 1986. Differential diagnosis of dementing illness. Neurologic Clinic of North America 4:329–40.Google Scholar
Keane, J, Calder, AJ, Hodges, JR, Young, AW. 2002. Face and emotion processing in frontal variant frontotemporal dementia. Neuropsychologia 40:655–65.Google Scholar
Kertesz, A, Munoz, DG. 1998. Pick's Disease and Pick Complex. New York: Wiley-Liss, Inc.Google Scholar
Kertesz, A, Munoz, DG. 2003. Primary progressive aphasia and Pick complex. Journal of Neurologic Sciences 206:97107.Google Scholar
Kertesz, A, Nadkarni, N, Davidson, W, Thomas, AW. 2000. The frontal behavioral inventory in the differential diagnosis of frontotemporal dementia. Journal of the International Neuropsychological Society 6:460–8.CrossRefGoogle ScholarPubMed
Kertesz, A, McMonagle, P, Blair, M, Davidson, W, Munoz, DG. 2005. The evolution and pathology of frontotemporal dementia. Brain 128:19962005.Google Scholar
Kitchener, E, Hodges, JR. 1999. Impaired knowledge of famous people and events and intact autobiographical knowledge in a case of progressive right temporal lobe degeneration: implications for the organization of remote memory. Cognitive Neuropsychology 16:589607.Google Scholar
Kloeters, S, Bertoux, M, O'Callaghan, C, Hodges, JR, Hornberger, M. 2013. Money for nothing – Atrophy correlates of gambling decision making in behavioural variant frontotemporal dementia and Alzheimer's disease. NeuroImage: Clinical 2:263–72.Google Scholar
Knibb, JA, Xuereb, JH, Patterson, K, Hodges, JR. 2006. Clinical and pathological characterisation of progressive aphasia. Annals of Neurology 59:156–65.Google Scholar
Knopman, DS, Mastri, AR, Frey, WH, Sung, JH, Rustan, T. 1990. Dementia lacking distinctive histological features: a common non-Alzheimer degenerative disease. Neurology 40:251–6.Google Scholar
Knopman, DS, Boeve, BF, Parisi, JE et al. 2005. Antemortem diagnosis of frontotemporal lobar degeneration. Annals of Neurology 57:480–8.Google Scholar
Kumfor, F, Piguet, O. 2012. Disturbance of emotion processing in frontotemporal dementia: a synthesis of cognitive and neuroimaging findings. Neuropsychology Review 22(3):280–97.Google Scholar
Kumfor, F, Irish, M, Hodges, J, Piguet, O. 2013. Discrete neural correlates for the recognition of basic emotions in frontotemporal dementia. PLoS One 8(6):e67457.CrossRefGoogle ScholarPubMed
Leyton, CE, Villemagne, VL, Savage, S et al. 2011. Subtypes of progressive aphasia: application of the International Consensus Criteria and validation using β-amyloid imaging. Brain 134 (Pt 10):3030–43.Google Scholar
Lillo, P, Hodges, JR. 2010. Cognition and behaviour in motor neurone disease (MND). Current Opinion in Neurology 23(6):638–42.Google Scholar
Lillo, P, Garcin, B, Bak, T, Hornberger, M, Hodges, J. 2010. Neurobehavioral features in frontotemporal dementia with amyotrophic lateral sclerosis. Archives of Neurology 67(7):826–30.Google Scholar
Lillo, P, Mioshi, E, Zoing, M, Kiernan, M, Hodges, JR. 2011. How common are behavioral changes in amyotrophic lateral sclerosis? Amyotrophic Lateral Sclerosis 12(1):4551.Google Scholar
Lillo, P, Mioshi, E, Hodges, JR. 2012. Caregiver burden in amyotrophic lateral sclerosis is more dependent on patients’ behavioral changes than physical disability: a comparative study. BMC Neurology 12:156.Google Scholar
Lough, S, Kipps, CM, Treise, C et al. 2006. Social reasoning, emotion and empathy in frontotemporal dementia. Neuropsychologia 44:950–8.Google Scholar
Lӧwenberg, K, Arbor, A. 1936. Pick's disease: a clinicopathologic contribution. Archives of Neurology and Psychiatry 36:768–89.Google Scholar
Lӧwenberg, K, Boyd, DA, Salon, DD, Arbor, A. 1939. Occurrence of Pick's disease in early adult years. Archives of Neurology and Psychiatry 41:1004–20.Google Scholar
Mackenzie, IR, Baker, M, West, G et al. 2006. A family with tau-negative frontotemporal dementia and neuronal intranuclear inclusions linked to chromosome 17. Brain 129:853–67.Google Scholar
Malamud, N, Boyd, DA. 1940. Pick's disease with atrophy of the temporal lobes: a clinicopathologic study. Archives of Neurology and Psychiatry 43:210–22.Google Scholar
Mansvelt, JV. 1954. Pick's Disease: A Syndrome of Lobar Cerebral Atrophy, its Clinico-anatomical and Histopathological Types. Utrecht: Thesis.Google Scholar
Mathew, R, Bak, TH, Hodges, JR. 2012. Diagnostic criteria for corticobasal syndrome: a comparative study. Journal of Neurology, Neurosurgery, and Psychiatry 83(4):405–10.Google Scholar
Mathuranath, PS, Xuereb, JH, Bak, T, Hodges, JR. 2000. Corticobasal ganglionic degeneration and/or frontotemporal dementia? A report of two overlap cases and review of literature. Journal of Neurology, Neurosurgery, and Psychiatry 68:304–12.Google Scholar
McKhann, G, Drachman, D, Folstein, M et al. 1984. Clinical diagnosis of Alzheimer's disease: report of the NINCDS-ADRDA Work Group under the auspices of Department of Health and Human Services Task Force on Alzheimer's Disease. Neurology 34:939–44.Google Scholar
McKhann, GM, Albert, MS, Grossman, M et al. 2001. Clinical and pathological diagnosis of frontotemporal dementia: report of the Working Group on Frontotemporal Dementia and Pick's Disease. Archives of Neurology 58:1803–9.Google Scholar
Mesulam, M. 1982. Slowly progressive aphasia without generalized dementia. Annals of Neurology 11:592–8.Google Scholar
Mesulam, MM, Weintraub, S. 1992. Primary progressive aphasia. In Boller, F, ed. Heterogeneity of Alzheimer's Disease pp. 4366. Berlin: Springer-Verlag.Google Scholar
Miller, BL, Cummings, JL, Villanueva-Meyer, J et al. 1991. Frontal lobe degeneration: clinical, neuropsychological, and SPECT characteristics. Neurology 41:1374–82.Google Scholar
Miller, BL, Darby, A, Benson, F, Cummings, JL, Miller, MH. 1997. Aggressive, socially disruptive and anti-social behaviour associated with frontotemporal dementia. British Journal of Psychiatry 170:150–4.Google Scholar
Mingazzini, G. 1913. On aphasia due to atrophy of the cerebral convolutions. Brain 36:493524.Google Scholar
Mioshi, M, Caga, J, Lillo, P et al. 2014. Neuropsychiatric changes precede classical motor symptoms in ALS and do not affect survival. Neurology 82(2):149–55.Google Scholar
Mitsuyama, Y, Takamiya, S. 1979. Presenile dementia with motor neuron disease in Japan. A new entity? Archives of Neurology 36:592–3.Google Scholar
Morita, K, Kaiya, H, Ikeda, T, Namba, M. 1987. Presenile dementia combined with amyotrophy: a review of 34 Japanese cases. Archives of Gerontology and Geriatrics 6:263–77.Google Scholar
Morris, JC, Cole, M, Banker, BQ, Wright, D. 1984. Hereditary dysphasic dementia and the Pick-Alzheimer spectrum. Annals of Neurology 16:455–66.Google Scholar
Mott, RT, Dickson, DW, Trojanowski, JQ et al. 2005. Neuropathologic, biochemical and molecular characterization of the frontotemporal dementias. Journal of Neuropathology and Experimental Neurology 64:420–8.Google Scholar
Mummery, CJ, Patterson, K, Wise, RJS et al. 1999. Disrupted temporal lobe connections in semantic dementia. Brain 122:6173.Google Scholar
Mummery, C J, Patterson, K, Price, CJ et al. 2000. A voxel based morphometry study of semantic dementia: the relationship between temporal lobe atrophy and semantic dementia. Annals of Neurology 47:3645.Google Scholar
Neary, D, Snowden, JS, Bowen, DM et al. 1986. Cerebral biopsy in the investigation of presenile dementia due to cerebral atrophy. Journal of Neurology, Neurosurgery, and Psychiatry 49:157–62.Google Scholar
Neary, D, Snowdon, JS, Mann, DMA et al. 1990. Frontal lobe dementia and motor neuron disease. Journal of Neurology, Neurosurgery, and Psychiatry 53:2332.CrossRefGoogle ScholarPubMed
Neary, D, Snowden, JS, Gustafson, L et al. 1998. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 51:1546–54.Google Scholar
Neumann, MA. 1949. Pick's disease. Journal of Neuropathology and Experimental Neurology 8:255–82.Google Scholar
Neumann, M, Sampathu, DM, Kwong, LK, et al. 2006. Ubiquinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 314(5796):130–3.Google Scholar
Nichols, IC, Weigner, WC. 1938. Pick's disease: a specific type of dementia. Brain 3:237–49.Google Scholar
Onari, K, Spatz, H. 1926. Anatomische beiträge zur lehre von der pickschen umschriebenen grosshirnindenatrophie (Piscksche Krankheit). Zeitschrift für die Gesamte Neurologie und Psychiatrie 101:470511.Google Scholar
Pasquier, F, Lebert, F, Scheltens, P. 1996. Frontotemporal Dementia. The Netherlands: ICG Publications.Google Scholar
Patterson, K, Hodges, JR. 2000. Semantic dementia: one window on the structure and organisation of semantic memory. In Cermak, L, ed. Revised Handbook of Neuropsychology: Memory and its Disorders pp. 313–35. Amsterdam: Elsevier Science B.V.Google Scholar
Petersen, RB, Tabaton, M, Chen, SG et al. 1995. Familial progressive subcortical gliosis: presence of prions and linkage to chromosome 17. Neurology 45:1062–7.Google Scholar
Pick, A. 1892. Über die Beziehungen der senilen Hirnatrophie zur Aphasie. Prager Medicinische Wochenschrift 17:165–7.Google Scholar
Pick, A. 1904. Zur symptomatologie der linksseitigen Schlafenlappenatrophie. Monatsschrift für Psychiatrie und Neurologie 16:378–88.Google Scholar
Piguet, O, Hornberger, M, Mioshi, M, Hodges, JR. 2011. Behavioural-variant frontotemporal dementia: diagnosis, clinical staging and management. Lancet Neurology 10(2):162–72.Google Scholar
Poorkaj, P, Bird, TD, Wijsman, E et al. 1998. Tau is a candidate gene for chromosome 17 frontotemporal dementia. Annals of Neurology 43:815–25.Google Scholar
Rademakers, R, Neumann, M, Mackenzie, IR. 2013. Advances in understanding the molecular basis of frontotemporal dementia. Nature Review Neurology 8(8):423–34.Google Scholar
Rahman, S, Sahakian, B J, Hodges, JR, Rogers, RD, Robbins, TW. 1999. Specific cognitive deficits in mild frontal variant frontotemporal dementia. Brain 122:1469–93.CrossRefGoogle ScholarPubMed
Rakowicz, Z, Hodges, JR. 1998. Dementia and aphasia in motor neurone disease: an under recognised association. Journal of Neurology, Neurosurgery, and Psychiatry 65:881–9.Google Scholar
Rankin, KP, Kramer, JH, Mychack, P, Miller, BL. 2003. Double dissociation of social functioning in frontotemporal dementia. Neurology 60:266–71.Google Scholar
Rascovsky, K, Hodges, JR, Knopman, D et al. 2011. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 134 (Pt 9):2456–77.Google Scholar
Renton, AE, Majounie, E, Waite, A et al. 2011. Hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron 72(2):257–68.Google Scholar
Robertson, EE, Le Roux, A, Brown, JH. 1958. The clinical differentiation of Pick's disease. Journal of Mental Science 104:1000–24.Google Scholar
Rosen, HJ, Gorno-Tempini, ML, Goldman, WP et al. 2002a. Patterns of brain atrophy in frontotemporal dementia and semantic dementia. Neurology 58:198208.Google Scholar
Rosen, HJ, Hartikainen, KM, Jagust, W et al. 2002b. Utility of clinical criteria in differentiating frontotemporal lobar degeneration (FTLD) from AD. Neurology 58:1608–15.Google Scholar
Rosen, HJ, Allison, SC, Schauer, GF et al. 2005. Neuroanatomical correlates of behavioural disorders in dementia. Brain 128:2612–25.Google Scholar
Rosenfeld, M. 1909. Die partielle Gorsshirnatrophie. Journal für Psychologie und Neurologie 14:115–30.Google Scholar
Sajjadi, SA, Patterson, K, Arnold, RJ, Watson, PC, Nestor, PJ. 2012. Primary progressive aphasia: a tale of two syndromes and the rest. Neurology 78(21):1670–7.Google Scholar
Sanders, J, Schenk, VWD, van Veen, P. 1939. A family with Pick's disease. Verh kon Nederl Akad Wetensch 38:1124.Google Scholar
Sasanuma, S, Mondi, H. 1975. The syndrome of Gogi (word meaning) aphasia. Neurology 25:627–32.Google Scholar
Schenk, VWS. 1951. Maladie de Pick: etude anatomo-clinique de 8 cas. Annales Medicopsychologiques 109:574–87.Google Scholar
Schneider, C. 1927. Uber Picksche Krankheit. Monatschrift für Psychologie und Neurologie 65:230–75.Google Scholar
Schneider, C. 1929. Weitere Beitrage zur Lehre von der Pickschen Krankheit. Zeitschrift für die gesamte Neurologie und Psychiatrie 120:340–84.Google Scholar
Schneider, JA, Watts, RL, Gearing, M, Brewer, RP, Mirra, SS. 1997. Corticobasal degeneration: neuropathologic and clinical heterogeneity. Neurology 48:959–69.Google Scholar
Shelley, BP, Hodges, JR, Kipps, CM, Xuereb, JH, Bak, TH. 2009. Is the pathology of corticobasal syndrome predictable in life? Movement Disorders 24(11):1593–9.Google Scholar
Skibinski, G, Parkinson, NJ, Brown, JM et al. 2005. Mutations in the endosomal ESCRTIII-complex subunit CHMP2B in frontotemporal dementia. Nature Genetics 37:806–8.Google Scholar
Snowden, JS, Goulding, PJ, Neary, D. 1989. Semantic dementia: a form of circumscribed cerebral atrophy. Behavioural Neurology 2:167–82.Google Scholar
Snowden, JS, Griffiths, HL, Neary, D. 1996a. Progressive language disorder associated with frontal lobe degeneration. Neurocase 2:429–40.Google Scholar
Snowden, JS, Neary, D, Mann, D. 1996b. Frontotemporal Lobar Degeneration: Frontotemporal Dementia, Progressive Aphasia, Semantic Dementia. New York: Churchill Livingstone.Google Scholar
Sonty, SP, Mesulam, MM, Thompson, CK et al. 2003. Primary progressive aphasia: PPA and the language network. Annals of Neurology 53:3549.Google Scholar
Spillantini, MG, Goedert, M, Crowther, RA et al. 1997. Familial multiple system tauopathy with presenile dementia: a disease with abundant neuronal and glial tau filaments. Proceedings of the National Academy of Science USA 94:4113–18.Google Scholar
Spillantini, MG, Bird, TD, Ghetti, B. 1998a. Frontotemporal dementia and parkinsonism linked to chromosome 17: a new group of tauopathies. Brain Pathology 8:387402.Google Scholar
Spillantini, MG, Murrell, JR, Goedert, M et al. 1998b. Mutation in the tau gene in familial multiple system tauopathy with presenile dementia. Proceedings of the National Academy of Science USA 95:7737–41.Google Scholar
Stertz, G. 1926. Uber die Picksche atrophie. Zeitschrift fur die Gesamte Neurologie und Psychiatrie 101:729–47.CrossRefGoogle Scholar
Sumi, SM, Bird, TD, Nochlin, D, Raskind, MA. 1992. Familial presenile dementia with psychosis associated with cortical neurofibrillary tangles and degeneration of the amygdala. Neurology 42:120–7.Google Scholar
Tanabe, H. 1992. Personality of typical Gogi (word meaning) aphasics. Japanese Journal of Neuropsychology 8:3442.Google Scholar
Tanabe, H, Ikeda, M, Nakagawa, Y et al. 1992. Gogi (word meaning) aphasia and semantic memory for words. Higher Brain Function Research 12:153–69.Google Scholar
Thompson, SA, Patterson, K, Hodges, JR. 2003. Left/right asymmetry of atrophy in semantic dementia: behavioural cognitive implications. Neurology 61:11961203.Google Scholar
Tissot, R, Constantinidis, J, Richard, J. 1975. La Maladie de Pick. Paris: Masson.Google Scholar
Tissot, R, Constantinidis, J, Richard, J. 1985. Pick's disease. In Frederiks, JAM, ed. Handbook of Clinical Neurology: Neurobehavioural Disorders Vol. 2, pp. 233–46. Amsterdam: Elsevier Science Publishers.Google Scholar
Torralva, T, Hodges, J, Clark, L et al. 2007. The relationship between affective decision making and theory of mind in the frontal variant of frontotemporal dementia. Neuropsychologia 45:342–9.Google Scholar
Tulving, E. 1972. Episodic and semantic memory. In Tulving, E, Donaldson, W, eds. Organisation of Memory, pp. 381403. New York: Academic Press.Google Scholar
Tulving, E. 1983. Elements of Episodic Memory. New York: Oxford University Press.Google Scholar
Van der Zee, J, Rademakers, R, Engelborghs, S et al. 2006. A Belgian ancestral haplotype harbours a highly prevalent mutation for 17q21-linked tau-negative FTLD. Brain 129:841–52.Google Scholar
Warrington, EK. 1975. Selective impairment of semantic memory. Quarterly Journal of Experimental Psychology 27:635–57.Google Scholar
Wilhelmsen, KC. 1997. Frontotemporal dementia is on the MAP. Annals of Neurology 41:139–40.Google Scholar
Wilhelmsen, KC, Lynch, T, Pavlou, E, Higgins, M, Nygaard, TG. 1994. Localization of disinhibition-dementia-parkinsonism-amyotrophy complex to 17q21–22. American Journal of Human Genetics 55:1159–65.Google Scholar
Williams, GB, Nestor, PJ, Hodges, JR. 2005. The neural correlates of semantic and behavioural deficits in frontotemporal dementia. NeuroImage 24:1042–51.Google Scholar
Wszolek, ZK, Pfeiffer, RF, Bhatt, MH et al. 1992. Rapidly progressive autosomal dominant parkinsonism and dementia with pallido-ponto-nigral degeneration. Annals of Neurology 32:312–20.Google Scholar

References

Pick, A. Über die Beziehungen der senilen Hirnatrophie zur Aphasie. Prag Med Wochenschr 1892;17:165–7.Google Scholar
Pick, A. Zur symptomatologie der linksseitigen Schlafenlappenatrophie. Monatsschr Psychiatr Neurol 1904;16:378–88.Google Scholar
Snowden, J, Goulding, P, Neary, D. Semantic dementia: a form of circumscribed cerebral atrophy. Behav Neurol 1989;2:167–82.Google Scholar
Hodges, JR, Patterson, K, Oxbury, S, Funnell, E. Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy. Brain 1992;115(Pt 6):1783–806.Google Scholar
Brun, A. Frontal lobe degeneration of non-Alzheimer type. I. Neuropathology. Arch Gerontol Geriatr 1987;6(3):193208.Google Scholar
Neary, D, Snowden, JS, Northen, B, Goulding, P. Dementia of frontal lobe type. J Neurol Neurosurg Psychiatry 1988;51(3):353–61.Google Scholar
Neary, D, Snowden, JS, Gustafson, L, Passant, U, Stuss, D, Black, S, et al. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 1998;51(6):1546–54.Google Scholar
Mesulam, MM. Primary progressive aphasia – differentiation from Alzheimer's disease. Ann Neurol 1987;22(4):533–4.Google Scholar
Steele, JC, Richardson, JC, Olszewski, J. Progressive supranuclear palsy. A heterogeneous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol 1964;10:333–59.Google Scholar
Gibb, WR, Luthert, PJ, Marsden, CD. Corticobasal degeneration. Brain 1989;112(Pt 5):1171–92.Google Scholar
Constantinidis, J, Richard, J, Tissot, R. Pick's disease. Histological and clinical correlations. Eur Neurol 1974;11(4):208–17.Google Scholar
Kertesz, A, Hudson, L, Mackenzie, IR, Munoz, DG. The pathology and nosology of primary progressive aphasia. Neurology 1994;44(11):2065–72.Google Scholar
Rascovsky, K, Hodges, JR, Knopman, D, Mendez, MF, Kramer, JH, Neuhaus, J, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 2011;134(Pt 9):2456–77.Google Scholar
Gregory, CA, Hodges, JR. Clinical features of frontal lobe dementia in comparison to Alzheimer's disease. J Neural Transm Suppl 1996;47:103–23.Google Scholar
Cummings, JL, Duchen, LW. Kluver-Bucy syndrome in Pick disease: clinical and pathologic correlations. Neurology 1981;31(11):1415–22.Google Scholar
Miller, BL, Cummings, JL, Villanueva-Meyer, J, Boone, K, Mehringer, CM, Lesser, IM, et al. Frontal lobe degeneration: clinical, neuropsychological, and SPECT characteristics. Neurology 1991;41(9):1374–82.Google Scholar
Elfgren, C, Passant, U, Risberg, J. Neuropsychological findings in frontal lobe dementia. Dementia 1993;4(3–4):214–19.Google Scholar
Hodges, JR, Patterson, K, Ward, R, Garrard, P, Bak, T, Perry, R, et al. 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 1999;13(1):3140.Google Scholar
Graham, A, Davies, R, Xuereb, J, Halliday, G, Kril, J, Creasey, H, et al. Pathologically proven frontotemporal dementia presenting with severe amnesia. Brain 2005;128(Pt 3):597605.Google Scholar
Kertesz, A, Davidson, W, McCabe, P, Munoz, D. Behavioral quantitation is more sensitive than cognitive testing in frontotemporal dementia. Alzheimer Dis Assoc Disord 2003;17(4):223–9.Google Scholar
Grossman, M, Mickanin, J, Onishi, K, Hughes, E, D'Esposito, M, Ding, XS, et al. Progressive nonfluent aphasia: language, cognitive, and PET measures contrasted with probable Alzheimer's disease. J Cogn Neurosci 1996;8(2):135–54.Google Scholar
Cohen, L, Benoit, N, Van Eeckhout, P, Ducarne, B, Brunet, P. Pure progressive aphemia. J Neurol Neurosurg Psychiatry 1993;56(8):923–4.Google Scholar
Mesulam, MM, Grossman, M, Hillis, A, Kertesz, A, Weintraub, S. The core and halo of primary progressive aphasia and semantic dementia. Ann Neurol 2003;54 Suppl 5:S1114.Google Scholar
Karbe, H, Kertesz, A, Polk, M. Profiles of language impairment in primary progressive aphasia. Arch Neurol 1993;50(2):193201.Google Scholar
Weintraub, S, Rubin, NP, Mesulam, MM. Primary progressive aphasia. Longitudinal course, neuropsychological profile, and language features. Arch Neurol 1990;47(12):1329–35.Google Scholar
Gorno-Tempini, ML, Hillis, AE, Weintraub, S, Kertesz, A, Mendez, M, Cappa, SF, et al. Classification of primary progressive aphasia and its variants. Neurology 2011;76(11):1006–14.Google Scholar
Josephs, KA, Duffy, JR, Strand, EA, Machulda, MM, Senjem, ML, Master, AV, et al. Characterizing a neurodegenerative syndrome: primary progressive apraxia of speech. Brain 2012;135(Pt 5):1522–36.Google Scholar
Fukui, T, Sugita, K, Kawamura, M, Shiota, J, Nakano, I. Primary progressive apraxia in Pick's disease: a clinicopathologic study. Neurology 1996;47(2):467–73.Google Scholar
Kertesz, A, McMonagle, P, Blair, M, Davidson, W, Munoz, DG. The evolution and pathology of frontotemporal dementia. Brain 2005;128(Pt 9):19962005.Google Scholar
Appell, J, Kertesz, A, Fisman, M. A study of language functioning in Alzheimer patients. Brain Lang 1982;17(1):7391.Google Scholar
Whiteley, AM, Warrington, EK. Prosopagnosia: a clinical, psychological, and anatomical study of three patients. J Neurol Neurosurg Psychiatry 1977;40(4):395403.CrossRefGoogle ScholarPubMed
Gorno-Tempini, ML, Dronkers, NF, Rankin, KP, Ogar, JM, Phengrasamy, L, Rosen, HJ, et al. Cognition and anatomy in three variants of primary progressive aphasia. Ann Neurol 2004;55(3):335–46.Google Scholar
Mesulam, M, Weintraub, S. Primary progressive aphasia: sharpening the focus on a clinical syndrome. In: Boller, F, Forrette, F, Khachaturian, Z, Poncet, M, Christen, Y, editors. Heterogeneity of Alzheimer's Disease Berlin: Springer-Verlag; 1992. p. 4366.Google Scholar
Mesulam, M, Wicklund, A, Johnson, N, Rogalski, E, Léger, GC, Rademaker, A, et al. Alzheimer and frontotemporal pathology in subsets of primary progressive aphasia. Ann Neurol 2008;63(6):709–19.Google Scholar
Harris, JM, Gall, C, Thompson, JC, Richardson, AM, Neary, D, du Plessis, D, et al. Classification and pathology of primary progressive aphasia. Neurology 2013;81(21):1832–9.Google Scholar
Akelaitis, AJ. Atrophy of basal ganglia in Pick's disease. A clinicopathologic study. Arch Neurol Psychiatr 1944;51:2734.Google Scholar
Munoz-Garcia, D, Ludwin, SK. Classic and generalized variants of Pick's disease: a clinicopathological, ultrastructural, and immunocytochemical comparative study. Ann Neurol 1984;16(4):467–80.Google Scholar
Rebeiz, JJ, Kolodny, EH, Richardson, EP. Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol 1968;18(1):2033.Google Scholar
Lang, AE, Bergeron, C, Pollanen, MS, Ashby, P. Parietal Pick's disease mimicking cortical-basal ganglionic degeneration. Neurology 1994;44(8):1436–40.Google Scholar
Lippa, CF, Smith, TW, Fontneau, N. Corticonigral degeneration with neuronal achromasia. A clinicopathologic study of two cases. J Neurol Sci 1990;98(2–3):301–10.Google Scholar
Kertesz, A, Munoz, DG. Pick's Disease and Pick Complex New York; Chichester: J. Wiley & Sons; 1998.Google Scholar
Kertesz, A, Martinez-Lage, P, Davidson, W, Munoz, DG. The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia. Neurology 2000;55(9):1368–75.Google Scholar
Armstrong, MJ, Litvan, I, Lang, AE, Bak, TH, Bhatia, KP, Borroni, B, et al. Criteria for the diagnosis of corticobasal degeneration. Neurology 2013;80(5):496503.Google Scholar
Pillon, B, Blin, J, Vidailhet, M, Deweer, B, Sirigu, A, Dubois, B, et al. The neuropsychological pattern of corticobasal degeneration: comparison with progressive supranuclear palsy and Alzheimer's disease. Neurology 1995;45(8):1477–83.Google Scholar
Feany, MB, Mattiace, LA, Dickson, DW. Neuropathologic overlap of progressive supranuclear palsy, Pick's disease and corticobasal degeneration. J Neuropathol Exp Neurol 1996;55(1):5367.Google Scholar
Houlden, H, Baker, M, Morris, HR, MacDonald, N, Pickering-Brown, S, Adamson, J, et al. Corticobasal degeneration and progressive supranuclear palsy share a common tau haplotype. Neurology 2001;56(12):1702–6.Google Scholar
Dickson, DW, Bergeron, C, Chin, SS, Duyckaerts, C, Horoupian, D, Ikeda, K, et al. Office of Rare Diseases neuropathologic criteria for corticobasal degeneration. J Neuropathol Exp Neurol 2002;61(11):935–46.Google Scholar
Mitsuyama, Y. Presenile dementia with motor neuron disease in Japan: clinico-pathological review of 26 cases. J Neurol Neurosurg Psychiatry 1984;47(9):953–9.Google Scholar
Neary, D, Snowden, JS, Mann, DM, Northen, B, Goulding, PJ, Macdermott, N. Frontal lobe dementia and motor neuron disease. J Neurol Neurosurg Psychiatry 1990;53(1):2332.Google Scholar
Von Braumuhl, A. Picksche krankheit und amyotrophische lateralsklerose. Allgemeine Z Psychiatr Psychol Med 1932;96:364–6.Google Scholar
Lomen-Hoerth, C, Anderson, T, Miller, B. The overlap of amyotrophic lateral sclerosis and frontotemporal dementia. Neurology 2002;59(7):1077–9.Google Scholar
Strong, MJ, Lomen-Hoerth, C, Caselli, RJ, Bigio, EH, Yang, W. Cognitive impairment, frontotemporal dementia, and the motor neuron diseases. Ann Neurol 2003;54 Suppl 5:S20–3.Google Scholar
Hodges, JR, Davies, R, Xuereb, J, Kril, J, Halliday, G. Survival in frontotemporal dementia. Neurology 2003;61(3):349–54.Google Scholar
Okamoto, K, Hirai, S, Yamazaki, T, Sun, XY, Nakazato, Y. New ubiquitin-positive intraneuronal inclusions in the extra-motor cortices in patients with amyotrophic lateral sclerosis. Neurosci Lett 1991;129(2):233–6.CrossRefGoogle ScholarPubMed
Jackson, M, Lennox, G, Lowe, J. Motor neurone disease-inclusion dementia. Neurodegeneration 1996;5(4):339–50.Google Scholar
Morita, M, Al-Chalabi, A, Andersen, PM, Hosler, B, Sapp, P, Englund, E, et al. A locus on chromosome 9p confers susceptibility to ALS and frontotemporal dementia. Neurology 2006;66(6):839–44.Google Scholar
Vance, C, Al-Chalabi, A, Ruddy, D, Smith, BN, Hu, X, Sreedharan, J, et al. Familial amyotrophic lateral sclerosis with frontotemporal dementia is linked to a locus on chromosome 9p13.2–21.3. Brain 2006;129(Pt 4):868–76.Google Scholar
DeJesus-Hernandez, M, Mackenzie, IR, Boeve, BF, Boxer, AL, Baker, M, Rutherford, NJ, et al. Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron 2011;72(2):245–56.Google Scholar
Renton, AE, Majounie, E, Waite, A, Simón-Sánchez, J, Rollinson, S, Gibbs, JR, et al. A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron 2011;72(2):257–68.Google Scholar
Anderson, P. ALS and FTD: two sides of the same coin? Lancet Neurol 2013;12:937–8.Google Scholar
van Blitterswijk, M, DeJesus-Hernandez, M, Niemantsverdriet, E, Murray, ME, Heckman, MG, Diehl, NN, et al. Association between repeat sizes and clinical and pathological characteristics in carriers of C9ORF72 repeat expansions (Xpansize-72): a cross-sectional cohort study. Lancet Neurol 2013;12(10):978–88.Google Scholar
Hodges, JR, Davies, RR, Xuereb, JH, Casey, B, Broe, M, Bak, TH, et al. Clinicopathological correlates in frontotemporal dementia. Ann Neurol 2004;56(3):399406.Google Scholar
Neumann, M, Sampathu, DM, Kwong, LK, Truax, AC, Micsenyi, MC, Chou, TT, et al. Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 2006;314(5796):130–3.Google Scholar
Mackenzie, IR, Neumann, M, Baborie, A, Sampathu, DM, Du Plessis, D, Jaros, E, et al. A harmonized classification system for FTLD-TDP pathology. Acta Neuropathol 2011;122(1):111–13.Google Scholar
Munoz, DG, Neumann, M, Kusaka, H, Yokota, O, Ishihara, K, Terada, S, et al. FUS pathology in basophilic inclusion body disease. Acta Neuropathol 2009;118(5):617–27.Google Scholar
Neumann, M, Rademakers, R, Roeber, S, Baker, M, Kretzschmar, HA, Mackenzie, IR. A new subtype of frontotemporal lobar degeneration with FUS pathology. Brain 2009;132(Pt 11):2922–31.Google Scholar
Mackenzie, IR, Neumann, M, Bigio, EH, Cairns, NJ, Alafuzoff, I, Kril, J, et al. Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations. Acta Neuropathol 2009;117(1):1518.Google Scholar
Snowden, JS, Rollinson, S, Thompson, JC, Harris, JM, Stopford, CL, Richardson, AM, et al. Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations. Brain 2012;135(Pt 3):693708.Google Scholar
Roberson, ED. Mouse models of frontotemporal dementia. Ann Neurol 2012;72(6):837–49.Google Scholar
Munoz, DG, Woulfe, J, Kertesz, A. Argyrophilic thorny astrocyte clusters in association with Alzheimer's disease pathology in possible primary progressive aphasia. Acta Neuropathol 2007;114(4):347–57.Google Scholar
Josephs, KA, Hodges, JR, Snowden, JS, Mackenzie, IR, Neumann, M, Mann, DM, et al. Neuropathological background of phenotypical variability in frontotemporal dementia. Acta Neuropathol 2011;122(2):137–53.Google Scholar
Rohrer, JD, Lashley, T, Schott, JM, Warren, JE, Mead, S, Isaacs, AM, et al. Clinical and neuroanatomical signatures of tissue pathology in frontotemporal lobar degeneration. Brain 2011;134(Pt 9):2565–81.Google Scholar
Menon, R, Baborie, A, Jaros, E, Mann, DM, Ray, PS, Larner, AJ. What's in a name? Neuronal intermediate filament inclusion disease (NIFID), frontotemporal lobar degeneration-intermediate filament (FTLD-IF) or frontotemporal lobar degeneration-fused in sarcoma (FTLD-FUS)? J Neurol Neurosurg Psychiatry 2011;82(12):1412–14.Google Scholar
Mackenzie, IR, Foti, D, Woulfe, J, Hurwitz, TA. Atypical frontotemporal lobar degeneration with ubiquitin-positive, TDP-43-negative neuronal inclusions. Brain 2008;131(Pt 5):1282–93.Google Scholar
Lesage, S, Le Ber, I, Condroyer, C, Broussolle, E, Gabelle, A, Thobois, S, et al. C9orf72 repeat expansions are a rare genetic cause of parkinsonism. Brain 2013;136(Pt 2):385–91.Google Scholar
Bozeat, S, Gregory, CA, Ralph, MA, Hodges, JR. Which neuropsychiatric and behavioural features distinguish frontal and temporal variants of frontotemporal dementia from Alzheimer's disease? J Neurol Neurosurg Psychiatry 2000;69(2):178–86.Google Scholar
Harris, JM, Gall, C, Thompson, JC, Richardson, AM, Neary, D, du Plessis, D, et al. Sensitivity and specificity of FTDC criteria for behavioral variant frontotemporal dementia. Neurology 2013;80(20):1881–7.Google Scholar
Alladi, S, Xuereb, J, Bak, T, Nestor, P, Knibb, J, Patterson, K, et al. Focal cortical presentations of Alzheimer's disease. Brain 2007;130(Pt 10):2636–45.Google Scholar
Chaal, S, Rowe, J. Dopamine transporter (dat) imaging can be normal with neuropathologically confirmed corticobasal degeneration. J Neurol Neurosurg Psychiatry 2013;84(11):e2.Google Scholar
Shelley, BP, Hodges, JR, Kipps, CM, Xuereb, JH, Bak, TH. Is the pathology of corticobasal syndrome predictable in life? Mov Disord 2009;24(11):1593–9.Google Scholar
Mathew, R, Bak, TH, Hodges, JR. Diagnostic criteria for corticobasal syndrome: a comparative study. J Neurol Neurosurg Psychiatry 2012;83(4):405–10.Google Scholar
Tang-Wai, D, Lewis, P, Boeve, B, Hutton, M, Golde, T, Baker, M, et al. Familial frontotemporal dementia associated with a novel presenilin-1 mutation. Dement Geriatr Cogn Disord 2002;14(1):1321.Google Scholar
Halliday, GM, Song, YJ, Lepar, G, Brooks, WS, Kwok, JB, Kersaitis, C, et al. Pick bodies in a family with presenilin-1 Alzheimer's disease. Ann Neurol 2005;57(1):139–43.Google Scholar
Dermaut, B, Kumar-Singh, S, Engelborghs, S, Theuns, J, Rademakers, R, Saerens, J, et al. A novel presenilin 1 mutation associated with Pick's disease but not beta-amyloid plaques. Ann Neurol 2004;55(5):617–26.Google Scholar
Irwin, DJ, McMillan, CT, Toledo, JB, Arnold, SE, Shaw, LM, Wang, LS, et al. Comparison of cerebrospinal fluid levels of tau and Aβ 1–42 in Alzheimer disease and frontotemporal degeneration using 2 analytical platforms. Arch Neurol 2012;69(8):1018–25.Google Scholar
Bathgate, D, Snowden, JS, Varma, A, Blackshaw, A, Neary, D. Behaviour in frontotemporal dementia, Alzheimer's disease and vascular dementia. Acta Neurol Scand 2001;103(6):367–78.Google Scholar
Greene, JD, Hodges, JR, Ironside, JW, Warlow, CP. Progressive aphasia with rapidly progressive dementia in a 49 year old woman. J Neurol Neurosurg Psychiatry 1999;66(2):238–43.CrossRefGoogle ScholarPubMed
Forman, MS, Farmer, J, Johnson, JK, Clark, CM, Arnold, SE, Coslett, HB, et al. Frontotemporal dementia: clinicopathological correlations. Ann Neurol 2006;59(6):952–62.Google Scholar
Mead, S, Poulter, M, Beck, J, Webb, TE, Campbell, TA, Linehan, JM, et al. Inherited prion disease with six octapeptide repeat insertional mutation – molecular analysis of phenotypic heterogeneity. Brain 2006;129(Pt 9):2297–317.Google Scholar
Kipps, CM, Hodges, JR, Hornberger, M. Nonprogressive behavioural frontotemporal dementia: recent developments and clinical implications of the ‘bvFTD phenocopy syndrome’. Curr Opin Neurol 2010;23(6):628–32.Google Scholar
Piguet, O, Hornberger, M, Mioshi, E, Hodges, JR. Behavioural-variant frontotemporal dementia: diagnosis, clinical staging, and management. Lancet Neurol 2011;10(2):162–72.Google Scholar

Save book to Kindle

To save this book to your Kindle, first ensure coreplatform@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

Available formats
×