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Chapter 9 - Frontotemporal dementia

Published online by Cambridge University Press:  01 December 2016

By
Shirley Y. Pang  and
Bruce L. Miller
Edited by
Bruce L. Miller  and
Bradley F. Boeve
Bruce L. Miller
Affiliation:
University of California, San Francisco
Bradley F. Boeve
Affiliation:
Mayo Clinic, Minnesota
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The Behavioral Neurology of Dementia , pp. 143 - 155
Publisher: Cambridge University Press
Print publication year: 2016

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References

Knopman, DS, Roberts, RO. Estimating the number of persons with frontotemporal lobar degeneration in the US population. J Mol Neurosci 2011; 45(3): 330–5.CrossRefGoogle ScholarPubMed
Knopman, DS, Petersen, RC, Edland, SD, et al. The incidence of frontotemporal lobar degeneration in Rochester, Minnesota, 1990 through 1994. Neurology 2004; 62(3): 506–8.CrossRefGoogle ScholarPubMed
Neary, D, Snowden, JS, Northen, B, et al. Dementia of frontal lobe type. J Neurol Neurosurg Psychiatry 1988; 51(3): 353–61.CrossRefGoogle ScholarPubMed
Brun, A. Frontal lobe degeneration of non-Alzheimer type. I. Neuropathol Arch Gerontol Geriatr 1987; 6(3): 193–208.CrossRefGoogle ScholarPubMed
Goldman, JS, Farmer, J, Wood, EM, et al. Comparison of family histories in FTLD subtypes and related tauopathies. Neurology 2005; 65(11): 1817–9.CrossRefGoogle ScholarPubMed
Pick, A. Uber die Beziehungen der senilen Hirnatrophie zur Aphasie. Prager Med Wochensch 1892; 17: 165–7.Google Scholar
Alzheimer, A. Uber eigenartige Krankheitsfalle des spateren Alters. Z Ges Neurol Psychiatr 1911; 4: 356–85.CrossRefGoogle Scholar
Neary, D, Snowden, JS, Bowen, DM, et al. Neuropsychological syndromes in presenile dementia due to cerebral atrophy. J Neurol Neurosurg Psychiatry 1986; 49(2): 163–74.Google ScholarPubMed
Gustafson, L. Frontal lobe degeneration of non-Alzheimer type. II. Clinical picture and differential diagnosis. Arch Gerontol Geriatr 1987; 6(3): 209–23.CrossRefGoogle ScholarPubMed
Mann, DMA, South, PW, Snowden, JS, et al. Dementia of frontal lobe type: neuropathology and immunohistochemistry. J Neurol Neurosurg Psychiatry 1993; 56: 605–14.CrossRefGoogle ScholarPubMed
Knopman, DS, Mastri, AR, Frey, WHD, et al. Dementia lacking distinctive histologic features: a common non-Alzheimer degenerative dementia. Neurology 1990; 40(2): 251–6.CrossRefGoogle ScholarPubMed
Jagust, WJ, Reed, BR, Seab, JP, et al. Clinical–physiologic correlates of Alzheimer’s disease and frontal lobe dementia. Am J Physiol Imaging 1989; 4: 89–96.Google ScholarPubMed
Miller, BL, Cummings, JL, Villanueva-Meyer, J, et al. Frontal lobe degeneration: clinical, neuropsychological, and SPECT characteristics. Neurology 1991; 41(9): 1374–82.CrossRefGoogle ScholarPubMed
Varma, AR, Snowden, JS, Lloyd, JJ, et al. Evaluation of the NINCDS–ADRDA criteria in the differentiation of Alzheimer’s disease and frontotemporal dementia. J Neurol Neurosurg Psychiatry 1999; 66(2): 184–8.CrossRefGoogle ScholarPubMed
de Leon, MJ, Convit, A, DeSanti, S, et al. Contribution of structural neuroimaging to the early diagnosis of Alzheimer’s disease. Int Psychogeriatr 1997; 9(Suppl 1): 183–90; discussion 247–52.CrossRefGoogle Scholar
Thompson, PM, Hayashi, KM, de Zubicaray, G, et al. Dynamics of gray matter loss in Alzheimer’s disease. J Neurosci 2003; 23(3): 994–1005.CrossRefGoogle ScholarPubMed
Kitagaki, H, Mori, E, Yamaji, S, et al. Frontotemporal dementia and Alzheimer disease: evaluation of cortical atrophy with automated hemispheric surface display generated with MR images. Radiology 1998; 208(2): 431–9.CrossRefGoogle ScholarPubMed
Read, SL, Miller, BL, Mena, I, et al. SPECT in dementia: clinical and pathological correlation. J Am Geriatr Soc 1995; 43(11): 1243–7.CrossRefGoogle ScholarPubMed
Miller, BL. Clinical advances in degenerative dementias. [See comments.] Br J Psychiatry 1997; 171(18): 1–3.CrossRefGoogle ScholarPubMed
Neary, D, Snowden, JS, Gustafson, L, et al. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 1998; 51(6): 1546–54.CrossRefGoogle ScholarPubMed
Rascovsky, K, Hodges, JR, Knopman, D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 2011; 134(Pt 9): 2456–77.CrossRefGoogle ScholarPubMed
Gorno-Tempini, ML, Hillis, AE, Weintraub, S, et al. Classification of primary progressive aphasia and its variants. Neurology 2011; 76(11): 1006–14.CrossRefGoogle ScholarPubMed
Roberson, ED, Hesse, JH, Rose, KD, et al. Frontotemporal dementia progresses to death faster than Alzheimer disease. Neurology 2005; 65(5): 719–25.CrossRefGoogle ScholarPubMed
Johnson, JK, Diehl, J, Mendez, MF, et al. Frontotemporal lobar degeneration: demographic characteristics of 353 patients. Arch Neurol 2005; 62(6): 925–30.CrossRefGoogle ScholarPubMed
Lomen-Hoerth, C, Anderson, T, Miller, B. The overlap of amyotrophic lateral sclerosis and frontotemporal dementia. Neurology 2002; 59(7): 1077–9.CrossRefGoogle ScholarPubMed
Murphy, JM, Henry, RG, Langmore, S, et al. Continuum of frontal lobe impairment in amyotrophic lateral sclerosis. Arch Neurol 2007; 64(4): 530–4.Google ScholarPubMed
Seeley, WW, Bauer, AM, Miller, BL, et al. The natural history of temporal variant frontotemporal dementia. Neurology 2005; 64(8): 1384–90.CrossRefGoogle ScholarPubMed
Josephs, KA, Petersen, RC, Knopman, DS, et al. Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP. Neurology 2006; 66(1): 41–8.CrossRefGoogle ScholarPubMed
Mathuranath, PS, Xuereb, JH, Bak, T, et al. Corticobasal ganglionic degeneration and/or frontotemporal dementia? A report of two overlap cases and review of literature. J Neurol Neurosurg Psychiatry 2000; 68(3): 304–12.CrossRefGoogle ScholarPubMed
Kertesz, A, Munoz, DG. Diagnostic controversies: is CBD part of the “Pick complex.” Adv Neurol 2000; 82: 223–31.Google ScholarPubMed
Mann, DM, South, PW. The topographic distribution of brain atrophy in frontal lobe dementia. Acta Neuropathol 1993; 85(3): 334–40.CrossRefGoogle ScholarPubMed
Seeley, WW, Carlin, DA, Allman, JM, et al. Early frontotemporal dementia targets neurons unique to apes and humans. Ann Neurol, 2006; 60(6): 660–7.CrossRefGoogle ScholarPubMed
Krill, JJ, Halliday, GM. Pathological staging of frontotemporal lobar degeneration. J Mol Neurosci 2011; 45(3): 379–83.Google Scholar
Mandelkow, EM, Mandelkow, E. Biochemistry and cell biology of tau protein in neurofibrillary degeneration. Cold Spring Harb Perspect Med 2012; 2(7): a006247.CrossRefGoogle ScholarPubMed
Kovacs, GG, Rozemuller, AJ, van Swieten, JC, et al. Neuropathology of the hippocampus in FTLD-tau with Pick bodies: a study of the BrainNet Europe Consortium. Neuropathol Appl Neurobiol 2013; 39(2): 166–78.CrossRefGoogle ScholarPubMed
Brun, A. Frontal lobe degeneration of non-Alzheimer type. I. Neuropathology. Arch Gerontol Geriatr 1987; 6(3): 193–208.CrossRefGoogle ScholarPubMed
Boxer, AL, Geschwind, MD, Belfor, N, et al. Patterns of brain atrophy that differentiate corticobasal degeneration syndrome from progressive supranuclear palsy. Arch Neurol 2006; 63(1): 81–6.CrossRefGoogle ScholarPubMed
Fujino, Y, Wang, DS, Thomas, N, et al. Increased frequency of argyrophilic grain disease in Alzheimer disease with 4R tau-specific immunohistochemistry. J Neuropathol Exp Neurol 2005; 64(3): 209–14.CrossRefGoogle ScholarPubMed
Soma, K, Fu, YJ, Wakabayashi, K, et al. Co-occurrence of argyrophilic grain disease in sporadic amyotrophic lateral sclerosis. Neuropathol Appl Neurobiol 2012; 38(1): 54–60.CrossRefGoogle ScholarPubMed
Neumann, M, Sampathu, DM, Kwong, LK, et al. Ubiquinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 2006; 314: 130–3.CrossRefGoogle Scholar
Mackenzie, IR, Neumann, M, Baborie, A, et al. A harmonized classification system for FTLD-TDP pathology. Acta Neuropathol 2011; 122(1): 111–13.CrossRefGoogle ScholarPubMed
Miller, ZA, Rankin, KP, Graff-Radford, NR, et al. TDP-43 frontotemporal lobar degeneration and autoimmune disease. J Neurol Neurosurg Psychiatry 2013; 84(9): 956–62.CrossRefGoogle ScholarPubMed
Koppers, M, van Blitterswijk, MM, Vlam, L, et al. VCP mutations in familial and sporadic ALS. Neurobiol Aging 2012; 33: 837.e7–13.CrossRefGoogle Scholar
Neumann, M, Rademakers, R, Roeber, S, et al. A new subtype of frontotemporal lobar degeneration with FUS pathology. Brain 2009; 132(Pt 11): 2922–31.CrossRefGoogle ScholarPubMed
Urwin, H, Josephs, KA, Rohrer, JD, et al. FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration. Acta Neuropathol 2010; 120: 33–41.CrossRefGoogle ScholarPubMed
Chow, TW, Miller, BL, Hayashi, VN, et al. Inheritance of frontotemporal dementia. Arch Neurol 1999; 56(7): 817–22.CrossRefGoogle ScholarPubMed
van Swieten, JC, Stevens, M, Rosso, SM, et al. Phenotypic variation in hereditary frontotemporal dementia with tau mutations. Ann Neurol, 1999; 46(4): 617–26.3.0.CO;2-I>CrossRefGoogle ScholarPubMed
Wilhelmsen, KC, Lynch, T, Pavlou, E, et al. Localization of disinhibition–dementia–parkinsonism–amyotrophy complex to 17q21–22. Am J Hum Genetics 1994; 55: 1159–65.Google ScholarPubMed
Seelaar, H, Rohrer, JD, Pijnenburg, YA, et al. Clinical, genetic and pathological heterogeneity of frontotemporal dementia: a review. J Neurol Neurosurg Psychiatry 2011; 82: 476–86.CrossRefGoogle ScholarPubMed
Rohrer, JD, Warren, JD. Phenotypic signatures of genetic frontotemporal dementia. Curr Opin Neurol 2011; 24(6): 542–9.CrossRefGoogle ScholarPubMed
Hong, M, Zhukareva, V, Vogelsberg-Ragaglia, V, et al. Mutation-specific functional impairments in distinct tau isoforms of hereditary FTDP-17. Science 1998; 282(5395): 1914–17.CrossRefGoogle ScholarPubMed
Baker, M, Mackenzie, IR, Pickering-Brown, SM, et al. Mutations in progranulin casue tau-negative frontotemporal dementia linked to chromosome 17. Nature 2006; 442: 916–19.CrossRefGoogle Scholar
Cruts, M, Gijselinck, I, van der Zee, J, et al. Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21. Nature 2006; 442(7105): 920–4.CrossRefGoogle ScholarPubMed
Coppola, G, Karydas, A, Rademakers, R, et al. Gene expression study on peripheral blood identifies progranulin mutations. Ann Neurol 2008; 64: 92–6.CrossRefGoogle Scholar
Finch, N, Baker, M, Crook, R, et al. Plasma progranulin levels predict progranulin mutation status in frontotemporal dementia patients and asymptomatic family members. Brain 2009; 132(Pt3): 583–91.CrossRefGoogle ScholarPubMed
Finch, N, Carrasquillo, MM, Baker, M, et al. TMEM106B regulates progranulin levels and the penetrance of FTLD in GRN mutation carriers. Neurology 2011; 76: 467–74.CrossRefGoogle ScholarPubMed
Hsiung, GYR, Fok, A, Feldman, HH, et al. rs5848 polymorphism and serum progranulin level. J Neurol Sci 2011; 300(1–2): 28–32.CrossRefGoogle ScholarPubMed
Rademakers, R, Baker, M, Gass, J, et al. Phenotypic variability associated with progranulin haploinsufficiency in patients with the common 1477C➔T (Arg493X) mutation: an international initiative. Lancet Neurol 2007; 6 : 857–68.CrossRefGoogle ScholarPubMed
Cenik, B, Sephton, CF, Cenik, BK, et al. Progranulin: a proteolytically processed protein at the crossroads of inflammation and neurodegeneration. J Biol Chem 2012; 287(39): 32298–306.CrossRefGoogle Scholar
Kao, AW, Eisenhut, RJ, Martens, LH, et al. A neurodegenerative disease mutation that accelerates the clearance of apoptotic cells. Proc Natl Acad Sci USA 2011; 108(11): 4441–6.CrossRefGoogle ScholarPubMed
Ryan, CL, Baranowski, DC, Chitramuthu, BP, et al. Progranulin is expressed within motor neurons and promotes neuronal cell survival. BMC Neurosci 2009; 10 : 130.CrossRefGoogle ScholarPubMed
Lomen-Hoerth, C, Murphey, J, Langmore, S, et al. Are amyotrophic lateral sclerosis patients cognitively normal? Neurology 2003; 60(7): 1094–7.CrossRefGoogle ScholarPubMed
Lomen-Hoerth, C, Anderson, T, Miller, B. The overlap of amyotrophic lateral sclerosis and frontotemporal dementia. Neurology 2002; 59(7): 1077–9.CrossRefGoogle ScholarPubMed
Hosler, BA, Siddique, T, Sapp, PC, et al. Linkage of familial amyotrophic lateral sclerosis with frontotemporal dementia to chromosome 9q21-q22. JAMA 2000; 284: 1664–9.CrossRefGoogle ScholarPubMed
DeJesus-Hernandez, M, Mackenzie, IR, Boeve, BF, et al. Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron 2011; 72: 245–56.CrossRefGoogle ScholarPubMed
Renton, AE, Majounie, E, Waite, A, et al. A hexanucleotide repeat expansion in C9ORF72 is the casue of chromosome 9p21-linked ALS-FTD. Neuron 2011; 72: 257–68.CrossRefGoogle Scholar
Sha, S, Takada, LT, Rankin, KP, et al. Frontotemporal dementia due to C9ORF72 mutations: clinical and imaging features. Neurology 2012; 79: 1002–11.CrossRefGoogle ScholarPubMed
Takada, LT, Pimentel, MLV, DeJesus-Hernandez, M, et al. Frontotemporal dementia in a Brazilian kindred with the C9ORF72 mutation. Arch Neurol 2012; 69(9): 1149–53.CrossRefGoogle Scholar
Ash, PE, Bieniek, KF, Gendron, TF, et al. Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS. Neuron 2013; 77(4): 639–46.CrossRefGoogle ScholarPubMed
Gallagher, MD, Suh, E, Grossman, M, et al. TMEM106B is a genetic modifier of frontotemporal lobar degeneration with C9ORF72 hexanucleotide repeat expansions. Acta Neuropathol 2014; 127(3): 407–18.CrossRefGoogle ScholarPubMed
van Blitterswijk, M, Mullen, B, Nicholson, AM, et al. TMEM106B protects C9ORF72 expansion carriers against frontotemporal dementia. Acta Neuropathol 2014; 127(3): 397–406.CrossRefGoogle ScholarPubMed
Hoffman, JM, Welsh-Bohmer, KA, Hanson, M, et al. FDG PET imaging in patients with pathologically verified dementia. J Nucl Med 2000; 41(11): 1920–8.Google ScholarPubMed
Rosen, HJ, Gorno-Tempini, ML, Goldman, WP, et al. Patterns of brain atrophy in frontotemporal dementia and semantic dementia. Neurology 2002; 58(2): 198–208.CrossRefGoogle ScholarPubMed
Chan, D, Fox, NC, Jenkins, R, et al. Rates of global and regional cerebral atrophy in AD and frontotemporal dementia. Neurology 2001; 57(10): 1756–63.CrossRefGoogle ScholarPubMed
Rabinovici, GD, Furst, AJ, O’Neil, JP, et al. 11C-PIB PET imaging in Alzheimer disease and frontotemporal lobar degeneration. Neurology 2007; 68(15): 1205–12.CrossRefGoogle ScholarPubMed
Edwards-Lee, T, Miller, BL, Benson, DF, et al. The temporal variant of frontotemporal dementia. Brain 1997; 120(Pt 6): 1027–40.CrossRefGoogle ScholarPubMed
Galton, CJ, Patterson, K, Graham, K, et al. Differing patterns of temporal atrophy in Alzheimer’s disease and semantic dementia. Neurology 2001; 57(2): 216–25.CrossRefGoogle ScholarPubMed
Chan, D, Fox, NC, Scahill, RI, et al. Patterns of temporal lobe atrophy in semantic dementia and Alzheimer’s disease. Ann Neurol 2001; 49(4): 433–42.CrossRefGoogle ScholarPubMed
Mummery, CJ, Patterson, K, Wise, RJ, et al. Disrupted temporal lobe connections in semantic dementia. Brain 1999; 122(Pt 1): 61–73.CrossRefGoogle ScholarPubMed
Hodges, JR, Patterson, K. Nonfluent progressive aphasia and semantic dementia: a comparative neuropsychological study. J Int Neuropsychol Soc 1996; 2(6): 511–24.CrossRefGoogle ScholarPubMed
Rosen, HJ, Kramer, JH, Gorno-Tempini, ML, et al. Patterns of cerebral atrophy in primary progressive aphasia. Am J Geriatr Psychiatry 2002; 10(1): 89–97.CrossRefGoogle ScholarPubMed
Moretti, R, Torre, P, Antonello, RM, et al. Rivastigmine in frontotemporal dementia: an open-label study. Drugs Aging 2004; 21: 93107.CrossRefGoogle ScholarPubMed
Kertesz, A, Morlog, D, Light, M, et al. Galantamine in frontotemporal dementia and primary progressive aphasia. Dement Geriatr Cogn Disord 2008; 25: 178–85.CrossRefGoogle ScholarPubMed
Mendez, MF, Shapira, JS, McMurtray, A, et al. Preliminary findings: behavioral worsening on donepezil in patients with frontotemporal dementia. Am J Geriatr Psychiatr 2007; 15: 84–7.CrossRefGoogle ScholarPubMed
Boxer, AL, Knopman, DS, Kaufer, DI, et al. Memantine in patients with frontotemporal lobar degeneration: a multicenter, randomized, double-blind, placebo-controlled trial. Lancet Neurol 2013; 12: 149–56.CrossRefGoogle Scholar
Hermann, N, Black, SE, Chow, T, et al. Serotonergic function and treatment of behavioral and psychological symptoms of frontotemporal dementia. Am J Geriatr Psychiatr 2012; 20: 789–97.CrossRefGoogle Scholar
Swartz, JR, Miller, BL, Lesser, IM, et al. Frontotemporal dementia: treatment response to serotonin selective reuptake inhibitors. J Clin Psychiatry 1997; 58: 212–16.CrossRefGoogle ScholarPubMed
Lebert, F, Stekke, W, Hasenbroekx, C, et al. Frontotemporal dementia: a randomized, controlled trial with trazodone. Dement Geriatr Cogn Disord 2004; 17: 355–9.CrossRefGoogle ScholarPubMed
Curtis, RC, Resch, DS. Case of Pick’s central lobar atrophy with apparent stabilization of cognitive decline after treatment with risperidone. J Clin Psychopharmacol 2000; 20: 384–5.CrossRefGoogle ScholarPubMed
Fellgiebel, A, Muller, MJ, Hiemke, C, et al. Clinical improvement in a case of frontotemporal dementia under aripiprazole treatment corresponds to partial recovery of disturbed frontal glucose metabolism. World J Biol Psychiatry 2007; 8: 123–6.CrossRefGoogle Scholar
Moretti, R, Torre, P, Antonello, RM, et al. Olanzapine as a treatment of neuropsychiatric disorders of Alzheimer’s disease and other dementias: a 24-month follow-up of 68 patients. Am J Alzheimers Dis Other Demen 2003; 18: 205–14.CrossRefGoogle ScholarPubMed
Chow, TW, Mendez, MF. Goals in symptomatic pharmacologic management of frontotemporal lobar degeneration. Am J Alzheimers Dis Other Demen 2002; 17: 267–72.CrossRefGoogle ScholarPubMed
Huey, ED, Garcia, C, Wassermann, EM, et al. Stimulant treatment of frontotemporal dementia in 8 patients. J Clin Psychiatry 2008; 69: 1981–2.CrossRefGoogle ScholarPubMed

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