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Cited by 419Dementia and Cognitive Impairment ResearchAmyotrophic Lateral Sclerosis ResearchAlzheimer's disease research and treatments

Frontotemporal dementia

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Olney, Nicholas T., Spina, Salvatore, Miller, Bruce L.·2017·Neurologic Clinics
DOI 10.1016/j.ncl.2017.01.008PMC PMC5472209
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Abstract

Frontotemporal dementia (FTD) is a heterogeneous disorder with distinct clinical phenotypes associated with multiple neuropathologic entities. Presently, the term FTD encompasses clinical disorders that include changes in behavior, language, executive control, and often motor symptoms. The core FTD spectrum disorders include behavioral variant FTD, nonfluent/agrammatic variant primary progressive aphasia, and semantic variant PPA. Related FTD disorders include frontotemporal dementia with motor neuron disease, progressive supranuclear palsy syndrome, and corticobasal syndrome. In this article, the authors discuss the clinical presentation, diagnostic criteria, neuropathology, genetics, and treatments of these disorders.

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  • Frontotemporal dementia is caused by frontotemporal lobar degeneration (FTLD), which features abnormal aggregation of tau (FTLD-tau) or transactivating DNA-binding protein 43.

    “Frontotemporal dementia is caused by frontotemporal lobar degeneration (FTLD), which features abnormal aggregation of the microtubule associated protein consisting of tau (FTLD-tau) or the transactivating DNA-binding protein 43.16,18–20”

    From Association of Cognitive and Behavioral Features Between Adults With Tuberous Sclerosis and Frontotemporal Dementia (2020) · cites this paper as [16]

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  1. Ahmed Z Bigio EH Budka H Dickson DW Ferrer I Ghetti B Kovacs GG 2013 Globular glial tauopathies (GGT): consensus recommendations Acta Neuropathol 126 4 537 544 10.1007/s00401-013-1171-0 23995422 PMC3914659 · DOI
  2. Ames D Cummings JL Wirshing WC Quinn B Mahler M 1994 Repetitive and compulsive behavior in frontal lobe degenerations Journal of Neuropsychiatry and Clinical Neurosciences 6 2 100 113 8044031
  3. Arai T Hasegawa M Akiyama H Ikeda K Nonaka T Mori H Oda T 2006 TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis Biochem Biophys Res Commun 351 3 602 611 17084815
  4. Armstrong MJ Litvan I Lang AE Bak TH Bhatia KP Borroni B Weiner WJ 2013 Criteria for the diagnosis of corticobasal degeneration Neurology 80 5 496 503 10.1212/WNL.0b013e31827f0fd1 23359374 PMC3590050 · DOI · Paper page
  5. Baborie A Griffiths TD Jaros E Perry R McKeith IG Burn DJ Mann DM 2015 Accumulation of dipeptide repeat proteins predates that of TDP-43 in frontotemporal lobar degeneration associated with hexanucleotide repeat expansions in C9ORF72 gene Neuropathol Appl Neurobiol 41 5 601 612 10.1111/nan.12178 25185840 PMC4934135 · DOI
  6. Baloh RH 2012 How do the RNA-binding proteins TDP-43 and FUS relate to amyotrophic lateral sclerosis and frontotemporal degeneration, and to each other? Curr Opin Neurol 25 6 701 707 10.1097/WCO.0b013e32835a269b 23041957 · DOI
  7. Baralle M Buratti E Baralle FE 2013 The role of TDP-43 in the pathogenesis of ALS and FTLD Biochem Soc Trans 41 6 1536 1540 10.1042/BST20130186 24256250 · DOI
  8. Bigio EH Brown DF White CL 1999 Progressive supranuclear palsy with dementia: cortical pathology J Neuropathol Exp Neurol 58 4 359 364 10218631
  9. Boeve BF Boylan KB Graff-Radford NR DeJesus-Hernandez M Knopman DS Pedraza O Rademakers R 2012 Characterization of frontotemporal dementia and/or amyotrophic lateral sclerosis associated with the GGGGCC repeat expansion in C9ORF72 Brain 135 Pt 3 765 783 doi:aws004 [pii] 10.1093/brain/aws004 22366793 PMC3286335 · DOI
  10. Borghero G Floris G Cannas A Marrosu MG Murru MR Costantino E Chio A 2011 A patient carrying a homozygous p.A382T TARDBP missense mutation shows a syndrome including ALS, extrapyramidal symptoms, and FTD Neurobiol Aging 32 12 2327 e2321 2325 10.1016/j.neurobiolaging.2011.06.009 PMC3192246 21803454 · DOI
  11. Boxer AL Geschwind MD Belfor N Gorno-Tempini ML Schauer GF Miller BL Rosen HJ 2006 Patterns of brain atrophy that differentiate corticobasal degeneration syndrome from progressive supranuclear palsy Arch Neurol 63 1 81 86 16401739
  12. Boxer AL Gold M Huey E Gao FB Burton EA Chow T Cummings J 2013 Frontotemporal degeneration, the next therapeutic frontier: molecules and animal models for frontotemporal degeneration drug development Alzheimers Dement 9 2 176 188 10.1016/j.jalz.2012.03.002 S1552-5260(12)01750-5 [pii] 23043900 PMC3542408 · DOI
  13. Boxer AL Gold M Huey E Hu WT Rosen H Kramer J Cummings JL 2013 The advantages of frontotemporal degeneration drug development (part 2 of frontotemporal degeneration: the next therapeutic frontier) Alzheimers Dement 9 2 189 198 10.1016/j.jalz.2012.03.003 23062850 PMC3562382 · DOI
  14. Brooks BR 1994 El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. Subcommittee on Motor Neuron Diseases/Amyotrophic Lateral Sclerosis of the World Federation of Neurology Research Group on Neuromuscular Diseases and the El Escorial “Clinical limits of amyotrophic lateral sclerosis” workshop contributors J Neurol Sci 124 Suppl 96 107 7807156
  15. Brun A 1993 Frontal lobe degeneration of non-Alzheimer type revisited Dementia 4 126 131 8401779
  16. Buratti E Baralle FE 2009 The molecular links between TDP-43 dysfunction and neurodegeneration Adv Genet 66 1 34 10.1016/S0065-2660(09)66001-6 19737636 · DOI
  17. Cairns NJ Bigio EH Mackenzie IR Neumann M Lee VM Hatanpaa KJ Mann DM 2007 Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration Acta Neuropathol (Berl) 114 1 5 22 17579875 PMC2827877
  18. Cairns NJ Neumann M Bigio EH Holm IE Troost D Hatanpaa KJ Mackenzie IR 2007 TDP-43 in familial and sporadic frontotemporal lobar degeneration with ubiquitin inclusions Am J Pathol 171 1 227 240 17591968 PMC1941578
  19. Carvalho MD Swash M 2009 Awaji diagnostic algorithm increases sensitivity of El Escorial criteria for ALS diagnosis Amyotroph Lateral Scler 10 1 53 57 10.1080/17482960802521126 18985466 · DOI
  20. Chambers CB Lee JM Troncoso JC Reich S Muma NA 1999 Overexpression of four-repeat tau mRNA isoforms in progressive supranuclear palsy but not in Alzheimer’s disease Ann Neurol 46 3 325 332 10482263
  21. Cheng ST Chow PK Song YQ Yu EC Chan AC Lee TM Lam JH 2014 Mental and physical activities delay cognitive decline in older persons with dementia Am J Geriatr Psychiatry 22 1 63 74 10.1016/j.jagp.2013.01.060 23582750 · DOI
  22. Chow TW Binns MA Cummings JL Lam I Black SE Miller BL van Reekum R 2009 Apathy symptom profile and behavioral associations in frontotemporal dementia vs dementia of Alzheimer type Arch Neurol 66 7 888 893 10.1001/archneurol.2009.92 19597092 PMC2875777 · DOI
  23. Chow TW Mendez MF 2002 Goals in symptomatic pharmacologic management of frontotemporal lobar degeneration Am J Alzheimers Dis Other Demen 17 5 267 272 12392261 PMC5841918
  24. Chow TW Miller BL Hayashi VN Geschwind DH 1999 Inheritance of frontotemporal dementia Arch Neurol 56 7 817 822 10404983 PMC5786271
  25. Clark LN Poorkaj P Wszolek Z Geschwind DH Nasreddine ZS Miller B Wilhelmsen KC 1998 Pathogenic implications of mutations in the tau gene in pallido-ponto-nigral degeneration and related neurodegenerative disorders linked to chromosome 17 Proceedings of the National Academy of Sciences of the United States of America 95 22 13103 13107 9789048 PMC23724
  26. Clinical and neuropathological criteria for frontotemporal dementia. The Lund and Manchester Groups 1994 J Neurol Neurosurg Psychiatry 57 4 416 418 8163988 PMC1072868
  27. Conrad C Andreadis A Trojanowski JQ Dickson DW Kang D Chen X Saitoh T 1997 Genetic evidence for the involvement of tau in progressive supranuclear palsy [see comments] Ann Neurol 41 2 277 281 9029080
  28. Constantinidis J Richard J Tissot R 1974 Pick’s disease: histological and clinical correlations European Neurology 11 208 217 4137107
  29. Coppola G Chinnathambi S Lee JJ Dombroski BA Baker MC Soto-Ortolaza AI Geschwind DH 2012 Evidence for a role of the rare p.A152T variant in MAPT in increasing the risk for FTD-spectrum and Alzheimer’s diseases Hum Mol Genet 21 15 3500 3512 10.1093/hmg/dds161 dds161 [pii] 22556362 PMC3392107 · DOI · Paper page
  30. Coppola G Karydas A Rademakers R Wang Q Baker M Hutton M Geschwind DH 2008 Gene expression study on peripheral blood identifies progranulin mutations Ann Neurol 64 1 92 96 10.1002/ana.21397 18551524 PMC2773201 · DOI
  31. Coyle-Gilchrist IT Dick KM Patterson K Vazquez Rodriquez P Wehmann E Wilcox A Rowe JB 2016 Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes Neurology 86 18 1736 1743 10.1212/WNL.0000000000002638 27037234 PMC4854589 · DOI
  32. Cruts M Gijselinck I van der Zee J Engelborghs S Wils H Pirici D Van Broeckhoven C 2006 Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21 Nature 442 7105 920 924 10.1038/nature05017 16862115 · DOI · Paper page
  33. Cruz M Marinho V Fontenelle LF Engelhardt E Laks J 2008 Topiramate may modulate alcohol abuse but not other compulsive behaviors in frontotemporal dementia: case report Cogn Behav Neurol 21 2 104 106 10.1097/WNN.0b013e31816bdf73 18541987 · DOI
  34. Deng HX Chen W Hong ST Boycott KM Gorrie GH Siddique N Siddique T 2011 Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia Nature 477 7363 211 215 10.1038/nature10353 21857683 PMC3169705 · DOI · Paper page
  35. Dickson DW Bergeron C Chin SS Duyckaerts C Horoupian D Ikeda K Litvan I 2002 Office of Rare Diseases neuropathologic criteria for corticobasal degeneration J Neuropathol Exp Neurol 61 11 935 946 12430710
  36. Diehl-Schmid J Perneczky R Koch J Nedopil N Kurz A 2013 Guilty by suspicion? Criminal behavior in frontotemporal lobar degeneration Cogn Behav Neurol 26 2 73 77 10.1097/WNN.0b013e31829cff11 23812170 · DOI
  37. Donker Kaat L Boon AJ Kamphorst W Ravid R Duivenvoorden HJ van Swieten JC 2007 Frontal presentation in progressive supranuclear palsy Neurology 69 8 723 729 10.1212/01.wnl.0000267643.24870.26 17709703 · DOI
  38. Edwards-Lee T Miller BL Benson DF Cummings JL Russell GL Boone K Mena I 1997 The temporal variant of frontotemporal dementia Brain 120 Pt 6 1027 1040 9217686
  39. Feany MB Dickson DW 1996 Neurodegenerative Disorders with Extensive Tau Pathology: A Comparative Study and Review Annals of Neurology 40 2 139 148 8773594
  40. Fecto F Yan J Vemula SP Liu E Yang Y Chen W Siddique T 2011 SQSTM1 mutations in familial and sporadic amyotrophic lateral sclerosis Arch Neurol 68 11 1440 1446 10.1001/archneurol.2011.250 22084127 · DOI · Paper page
  41. Finger EC 2011 New potential therapeutic approaches in frontotemporal dementia: oxytocin, vasopressin, and social cognition J Mol Neurosci 45 3 696 701 10.1007/s12031-011-9550-2 21618004 · DOI
  42. Flanagan EP Baker MC Perkerson RB Duffy JR Strand EA Whitwell JL Josephs KA 2015 Dominant frontotemporal dementia mutations in 140 cases of primary progressive aphasia and speech apraxia Dement Geriatr Cogn Disord 39 5–6 281 286 10.1159/000375299 25765123 PMC4879710 · DOI
  43. Fujino Y Wang DS Thomas N Espinoza M Davies P Dickson DW 2005 Increased frequency of argyrophilic grain disease in Alzheimer disease with 4R tau-specific immunohistochemistry J Neuropathol Exp Neurol 64 3 209 214 15804052
  44. Geevasinga N Loy CT Menon P de Carvalho M Swash M Schrooten M Vucic S 2016 Awaji criteria improves the diagnostic sensitivity in amyotrophic lateral sclerosis: A systematic review using individual patient data Clin Neurophysiol 127 7 2684 2691 10.1016/j.clinph.2016.04.005 27212114 · DOI
  45. Ghetti B Oblak AL Boeve BF Johnson KA Dickerson BC Goedert M 2015 Invited review: Frontotemporal dementia caused by microtubule-associated protein tau gene (MAPT) mutations: a chameleon for neuropathology and neuroimaging Neuropathology and applied neurobiology 41 1 24 46 10.1111/nan.12213 25556536 PMC4329416 · DOI
  46. Goldman JS Farmer JM Wood EM Johnson JK Boxer A Neuhaus J Miller BL 2005 Comparison of family histories in FTLD subtypes and related tauopathies Neurology 65 11 1817 1819 16344531
  47. Gorno-Tempini ML Dronkers NF Rankin KP Ogar JM Phengrasamy L Rosen HJ Miller BL 2004 Cognition and anatomy in three variants of primary progressive aphasia Ann Neurol 55 3 335 346 14991811 PMC2362399
  48. Gorno-Tempini ML Hillis AE Weintraub S Kertesz A Mendez M Cappa SF Grossman M 2011 Classification of primary progressive aphasia and its variants Neurology 76 11 1006 1014 21325651 PMC3059138
  49. Gorno-Tempini ML Ogar JM Brambati SM Wang P Jeong JH Rankin KP Miller BL 2006 Anatomical correlates of early mutism in progressive nonfluent aphasia Neurology 67 10 1849 1851 10.1212/01.wnl.0000237038.55627.5b 16931509 · DOI
  50. Graff-Radford NR Damasio AR Hyman BT Hart MN Tranel D Damasio H Rezai K 1990 Progressive aphasia in a patient with Pick’s disease: a neuropsychological, radiologic, and anatomic study Neurology 40 620 626 2320235
  51. Gregory CA Serra-Mestres J Hodges JR 1999 Early diagnosis of the frontal variant of frontotemporal dementia: how sensitive are standard neuroimaging and neuropsychologic tests? Neuropsychiatry, Neuropsychology, and Behavioral Neurology 12 2 128 135 10223261
  52. Grinberg LT Wang X Wang C Sohn PD Theofilas P Sidhu M Seeley WW 2013 Argyrophilic grain disease differs from other tauopathies by lacking tau acetylation Acta neuropathologica 125 4 581 593 10.1007/s00401-013-1080-2 23371364 PMC3692283 · DOI
  53. Grover A Houlden H Baker M Adamson J Lewis J Prihar G Hutton M 1999 5’ splice site mutations in tau associated with the inherited dementia FTDP-17 affect a stem-loop structure that regulates alternative splicing of exon 10 J Biol Chem 274 21 15134 15143 10329720
  54. Hassan A Whitwell JL Boeve BF Jack CR Jr Parisi JE Dickson DW Josephs KA 2010 Symmetric corticobasal degeneration (S-CBD) Parkinsonism Relat Disord 16 3 208 214 10.1016/j.parkreldis.2009.11.013 20018548 PMC2941264 · DOI
  55. Herrmann N Black SE Chow T Cappell J Tang-Wai DF Lanctot KL 2012 Serotonergic function and treatment of behavioral and psychological symptoms of frontotemporal dementia Am J Geriatr Psychiatry 20 9 789 797 10.1097/JGP.0b013e31823033f3 21878805 · DOI
  56. Hodges JR Davies R Xuereb J Kril J Halliday G 2003 Survival in frontotemporal dementia Neurology 61 3 349 354 12913196
  57. Hodges JR Mitchell J Dawson K Spillantini MG Xuereb JH McMonagle P Patterson K 2010 Semantic dementia: demography, familial factors and survival in a consecutive series of 100 cases Brain 133 Pt 1 300 306 10.1093/brain/awp248 19805492 · DOI
  58. Hogan DB Jette N Fiest KM Roberts JI Pearson D Smith EE Maxwell CJ 2016 The Prevalence and Incidence of Frontotemporal Dementia: a Systematic Review Can J Neurol Sci 43 Suppl 1 S96 S109 10.1017/cjn.2016.25 27307130 · DOI
  59. Holm IE Isaacs AM Mackenzie IR 2009 Absence of FUS-immunoreactive pathology in frontotemporal dementia linked to chromosome 3 (FTD-3) caused by mutation in the CHMP2B gene Acta Neuropathol 118 5 719 720 10.1007/s00401-009-0593-1 19844732 · DOI
  60. Hosler BA Siddique T Sapp PC Sailor W Huang MC Hossain A Brown RH Jr 2000 Linkage of familial amyotrophic lateral sclerosis with frontotemporal dementia to chromosome 9q21–q22 JAMA 284 13 1664 1669 11015796
  61. Hutton M Lendon CL Rizzu P Baker M Froelich S Houlden H 1998 Association of missense and 5’-splice-site mutations in tau with the inherited dementia FTDP-17 Nature 393 6686 702 705 9641683
  62. Ikeda M Shigenobu K Fukuhara R Hokoishi K Maki N Nebu A Tanabe H 2004 Efficacy of fluvoxamine as a treatment for behavioral symptoms in frontotemporal lobar degeneration patients Dement Geriatr Cogn Disord 17 3 117 121 10.1159/000076343 14739531 · DOI
  63. Ingvar DH Gustafson L 1970 Regional cerebral blood flow in organic dementia with early onset Acta Neurol Scand 46 Suppl 43 42+ 5457859
  64. Irwin DJ 2016 Tauopathies as clinicopathological entities Parkinsonism Relat Disord 22 Suppl 1 S29 33 10.1016/j.parkreldis.2015.09.020 26382841 PMC4662611 · DOI
  65. Irwin DJ Brettschneider J McMillan CT Cooper F Olm C Arnold SE Trojanowski JQ 2016 Deep clinical and neuropathological phenotyping of Pick disease Ann Neurol 79 2 272 287 10.1002/ana.24559 26583316 PMC4755803 · DOI
  66. Ishihara K Araki S Ihori N Shiota J Kawamura M Yoshida M Nakano I 2005 Argyrophilic grain disease presenting with frontotemporal dementia: a neuropsychological and pathological study of an autopsied case with presenile onset Neuropathology 25 2 165 170 15875911
  67. Jellinger KA 2007 The enigma of vascular cognitive disorder and vascular dementia Acta Neuropathol 113 4 349 388 10.1007/s00401-006-0185-2 17285295 · DOI
  68. Jenkins SM Johnson GV 1998 Tau complexes with phospholipase C-gamma in situ NeuroReport 9 1 67 71 9592050
  69. Jesso S Morlog D Ross S Pell MD Pasternak SH Mitchell DG Finger EC 2011 The effects of oxytocin on social cognition and behaviour in frontotemporal dementia Brain 134 Pt 9 2493 2501 10.1093/brain/awr171 21859765 · DOI
  70. Johnson JO Mandrioli J Benatar M Abramzon Y Van Deerlin VM Trojanowski JQ Traynor BJ 2010 Exome sequencing reveals VCP mutations as a cause of familial ALS Neuron 68 5 857 864 10.1016/j.neuron.2010.11.036 21145000 PMC3032425 · DOI · Paper page
  71. Josephs KA Hodges JR Snowden JS Mackenzie IR Neumann M Mann DM Dickson DW 2011 Neuropathological background of phenotypical variability in frontotemporal dementia Acta Neuropathol 122 2 137 153 10.1007/s00401-011-0839-6 21614463 PMC3232515 · DOI
  72. Josephs KA Petersen RC Knopman DS Boeve BF Whitwell JL Duffy JR Dickson DW 2006 Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP Neurology 66 1 41 48 16401843
  73. Josephs KA Whitwell JL Jack CR Jr 2008 Anatomic correlates of stereotypies in frontotemporal lobar degeneration Neurobiol Aging 29 12 1859 1863 10.1016/j.neurobiolaging.2007.04.027 17574708 PMC2745642 · DOI
  74. Karakaya T Fusser F Prvulovic D Hampel H 2012 Treatment options for tauopathies Curr Treat Options Neurol 14 2 126 136 10.1007/s11940-012-0168-7 22307450 · DOI
  75. Katzman R 1976 Editorial: The prevalence and malignancy of Alzheimer disease. A major killer Arch Neurol 33 4 217 218 1259639
  76. Kertesz A Davidson W McCabe P Takagi K Munoz D 2003 Primary progressive aphasia: diagnosis, varieties, evolution J Int Neuropsychol Soc 9 5 710 719 10.1017/S1355617703950041 12901777 · DOI
  77. Kertesz A McMonagle P 2010 Behavior and cognition in corticobasal degeneration and progressive supranuclear palsy J Neurol Sci 289 1–2 138 143 10.1016/j.jns.2009.08.036 19733862 · DOI
  78. Kertesz A McMonagle P Blair M Davidson W Munoz DG 2005 The evolution and pathology of frontotemporal dementia Brain 128 Pt 9 1996 2005 16033782
  79. Khan BK Yokoyama JS Takada LT Sha SJ Rutherford NJ Fong JC Miller BL 2012 Atypical, slowly progressive behavioural variant frontotemporal dementia associated with C9ORF72 hexanucleotide expansion J Neurol Neurosurg Psychiatry 83 4 358 364 doi:jnnp-2011-301883 [pii] 10.1136/jnnp-2011-301883 22399793 PMC3388906 · DOI · Paper page
  80. Kim YE Kang SY Ma HI Ju YS Kim YJ 2015 A Visual Rating Scale for the Hummingbird Sign with Adjustable Diagnostic Validity J Parkinsons Dis 5 3 605 612 10.3233/JPD-150537 26406141 · DOI
  81. Kimura T Takamatsu J 2013 Pilot study of pharmacological treatment for frontotemporal dementia: risk of donepezil treatment for behavioral and psychological symptoms Geriatr Gerontol Int 13 2 506 507 10.1111/j.1447-0594.2012.00956.x 23551349 · DOI
  82. Kipps CM Hodges JR Hornberger M 2010 Nonprogressive behavioural frontotemporal dementia: recent developments and clinical implications of the ‘bvFTD phenocopy syndrome’ Curr Opin Neurol 23 6 628 632 10.1097/WCO.0b013e3283404309 20962637 · DOI
  83. Kovacs GG Murrell JR Horvath S Haraszti L Majtenyi K Molnar MJ Spina S 2009 TARDBP variation associated with frontotemporal dementia, supranuclear gaze palsy, and chorea Mov Disord 24 12 1843 1847 10.1002/mds.22697 19609911 PMC12399026 · DOI
  84. Kramer JH Jurik J Sha SJ Rankin KP Rosen HJ Johnson JK Miller BL 2003 Distinctive neuropsychological patterns in frontotemporal dementia, semantic dementia, and Alzheimer disease Cogn Behav Neurol 16 4 211 218 14665820
  85. Kwiatkowski TJ Jr Bosco DA Leclerc AL Tamrazian E Vanderburg CR Russ C Brown RH Jr 2009 Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis Science 323 5918 1205 1208 10.1126/science.1166066 323/5918/1205 [pii] 19251627 · DOI
  86. Lagier-Tourenne C Baughn M Rigo F Sun S Liu P Li HR Ravits J 2013 Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration Proc Natl Acad Sci U S A 110 47 E4530 4539 10.1073/pnas.1318835110 24170860 PMC3839752 · DOI
  87. Lanata SC Miller BL 2016 The behavioural variant frontotemporal dementia (bvFTD) syndrome in psychiatry J Neurol Neurosurg Psychiatry 87 5 501 511 10.1136/jnnp-2015-310697 26216940 PMC4755931 · DOI
  88. Le Ber I Camuzat A Hannequin D Pasquier F Guedj E Rovelet-Lecrux A Brice A 2008 Phenotype variability in progranulin mutation carriers: a clinical, neuropsychological, imaging and genetic study Brain 131 Pt 3 732 746 10.1093/brain/awn012 awn012 [pii] 18245784 · DOI
  89. Lebert F Stekke W Hasenbroekx C Pasquier F 2004 Frontotemporal dementia: a randomised, controlled trial with trazodone Dement Geriatr Cogn Disord 17 4 355 359 10.1159/000077171 15178953 · DOI
  90. Lee EB Russ J Jung H Elman LB Chahine LM Kremens D McCluskey LF 2013 Topography of FUS pathology distinguishes late-onset BIBD from aFTLD-U Acta Neuropathol Commun 1 9 1 11 10.1186/2051-5960-1-9 PMC3767453 24027631 · DOI
  91. Lee SE Khazenzon AM Trujillo AJ Guo CC Yokoyama JS Sha SJ Seeley WW 2014 Altered network connectivity in frontotemporal dementia with C9orf72 hexanucleotide repeat expansion Brain 137 Pt 11 3047 3060 10.1093/brain/awu248 25273996 PMC4208465 · DOI
  92. Lee SE Rabinovici GD Mayo MC Wilson SM Seeley WW DeArmond SJ Miller BL 2011 Clinicopathological correlations in corticobasal degeneration Ann Neurol 70 2 327 340 10.1002/ana.22424 21823158 PMC3154081 · DOI · Paper page
  93. Leugers CJ Lee G 2010 Tau potentiates nerve growth factor-induced mitogen-activated protein kinase signaling and neurite initiation without a requirement for microtubule binding J Biol Chem 285 25 19125 19134 10.1074/jbc.M110.105387 20375017 PMC2885191 · DOI
  94. Liljegren M Naasan G Temlett J Perry DC Rankin KP Merrilees J Miller BL 2015 Criminal behavior in frontotemporal dementia and Alzheimer disease JAMA Neurol 72 3 295 300 10.1001/jamaneurol.2014.3781 25559744 PMC4432918 · DOI
  95. Litvan I Agid Y Calne D Campbell G Dubois B Duvoisin RC Zee DS 1996 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 8710059
  96. Litvan I Hauw JJ Bartko JJ Lantos PL Daniel SE Horoupian DS Anderson DW 1996 Validity and reliability of the preliminary NINDS neuropathologic criteria for progressive supranuclear palsy and related disorders J Neuropathol Exp Neurol 55 1 97 105 8558176
  97. Liu A Werner K Roy S Trojanowski JQ Morgan-Kane U Miller BL Rankin KP 2009 A case study of an emerging visual artist with frontotemporal lobar degeneration and amyotrophic lateral sclerosis Neurocase 15 3 235 247 10.1080/13554790802633213 19274573 PMC2829114 · DOI
  98. Lomen-Hoerth C 2011 Clinical phenomenology and neuroimaging correlates in ALS-FTD J Mol Neurosci 45 3 656 662 10.1007/s12031-011-9636-x 21971978 · DOI
  99. Lomen-Hoerth C Anderson T Miller B 2002 The overlap of amyotrophic lateral sclerosis and frontotemporal dementia Neurology 59 7 1077 1079 12370467
  100. Lu CH Macdonald-Wallis C Gray E Pearce N Petzold A Norgren N Malaspina A 2015 Neurofilament light chain: A prognostic biomarker in amyotrophic lateral sclerosis Neurology 84 22 2247 2257 10.1212/WNL.0000000000001642 25934855 PMC4456658 · DOI · Paper page
  101. Mackenzie IR 2007a The neuropathology and clinical phenotype of FTD with progranulin mutations Acta Neuropathol 114 1 49 54 10.1007/s00401-007-0223-8 17458552 · DOI
  102. Mackenzie IR 2007b The neuropathology of FTD associated With ALS Alzheimer Dis Assoc Disord 21 4 S44 49 10.1097/WAD.0b013e31815c3486 00002093-200710000-00016 [pii] 18090423 · DOI
  103. Mackenzie IR Feldman HH 2005 Ubiquitin immunohistochemistry suggests classic motor neuron disease, motor neuron disease with dementia, and frontotemporal dementia of the motor neuron disease type represent a clinicopathologic spectrum J Neuropathol Exp Neurol 64 8 730 739 16106222
  104. Mackenzie IR Munoz DG Kusaka H Yokota O Ishihara K Roeber S Neumann M 2011 Distinct pathological subtypes of FTLD-FUS Acta Neuropathol 121 2 207 218 10.1007/s00401-010-0764-0 21052700 · DOI
  105. Mackenzie IR Neumann M 2012 FET proteins in frontotemporal dementia and amyotrophic lateral sclerosis Brain Res 1462 40 43 10.1016/j.brainres.2011.12.010 22261247 · DOI
  106. Mackenzie IR Neumann M 2016 Molecular neuropathology of frontotemporal dementia: insights into disease mechanisms from postmortem studies J Neurochem 10.1111/jnc.13588 27306735 · DOI
  107. Mackenzie IR Neumann M Baborie A Sampathu DM Du Plessis D Jaros E Lee VM 2011 A harmonized classification system for FTLD-TDP pathology Acta Neuropathol 122 1 111 113 10.1007/s00401-011-0845-8 21644037 PMC3285143 · DOI
  108. Mackenzie IR Neumann M Bigio EH Cairns NJ Alafuzoff I Kril J Mann DM 2009 Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations Acta Neuropathol 117 1 15 18 10.1007/s00401-008-0460-5 19015862 PMC2710877 · DOI
  109. Mackenzie IR Neumann M Bigio EH Cairns NJ Alafuzoff I Kril J Mann DM 2010 Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update Acta Neuropathol 119 1 1 4 10.1007/s00401-009-0612-2 19924424 PMC2799633 · DOI · Paper page
  110. Mackenzie IR Rademakers R Neumann M 2010 TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia Lancet Neurol 9 10 995 1007 S1474-4422(10)70195-2 [pii] 10.1016/S1474-4422(10)70195-2 20864052 · DOI
  111. Mandelkow EM Mandelkow E 2012 Biochemistry and cell biology of tau protein in neurofibrillary degeneration Cold Spring Harb Perspect Med 2 7 a006247 10.1101/cshperspect.a006247 22762014 PMC3385935 · DOI
  112. Mann DM 2015 Dipeptide repeat protein toxicity in frontotemporal lobar degeneration and in motor neurone disease associated with expansions in C9ORF72-a cautionary note Neurobiol Aging 36 2 1224 1226 10.1016/j.neurobiolaging.2014.10.011 25457023 · DOI
  113. McGeer PL Schwab C McGeer EG Haddock RL Steele JC 1997 Familial nature and continuing morbidity of the amyotrophic lateral sclerosis-parkinsonism dementia complex of Guam Neurology 49 2 400 409 9270568
  114. Mendez MF Shapira JS 2011 Loss of emotional insight in behavioral variant frontotemporal dementia or “frontal anosodiaphoria” Conscious Cogn 20 4 1690 1696 S1053-8100(11)00213-3 [pii] 10.1016/j.concog.2011.09.005 21959203 PMC3199289 · DOI
  115. Mendez MF Shapira JS McMurtray A Licht E 2007 Preliminary findings: behavioral worsening on donepezil in patients with frontotemporal dementia Am J Geriatr Psychiatry 15 1 84 87 10.1097/01.JGP.0000231744.69631.33 17194818 · DOI · Paper page
  116. Mendez MF Shapira JS Miller BL 2005 Stereotypical movements and frontotemporal dementia Mov Disord 20 6 742 745 15786492
  117. Merrilees J 2007 A model for management of behavioral symptoms in frontotemporal lobar degeneration Alzheimer Dis Assoc Disord 21 4 S64 69 10.1097/WAD.0b013e31815bf774 18090427 · DOI
  118. Merrilees J Hubbard E Mastick J Miller BL Dowling GA 2009 Rest-activity and behavioral disruption in a patient with frontotemporal dementia Neurocase 15 6 515 526 10.1080/13554790903061371 914594846 [pii] 19736599 PMC2829101 · DOI
  119. Mesulam MM 1987 Primary progressive aphasia: differentiation from Alzheimer’s disease Ann Neurol 37 1448 1553 3324947
  120. Mesulam MM 1982 Slowly progressive aphasia without generalized dementia Annals of Neurology 11 6 592 598 7114808
  121. Mesulam MM 2001 Primary progressive aphasia Ann Neurol 49 4 425 432 11310619
  122. Miller BL 2014 Frontotemporal Dementia Oxford University Press
  123. Miller BL Boone K Cummings JL Read SL Mishkin F 2000 Functional correlates of musical and visual ability in frontotemporal dementia Br J Psychiatry 176 458 463 10912222
  124. Miller BL Chang L Mena I Boone K Lesser IM 1993 Progressive right frontotemporal degeneration: clinical, neuropsychological and SPECT characteristics Dementia 4 3–4 204 213 8401793
  125. Miller BL Cummings J Mishkin F Boone K Prince F Ponton M Cotman C 1998 Emergence of artistic talent in frontotemporal dementia Neurology 51 4 978 982 9781516
  126. Miller BL Darby AL Swartz JR Yener GG Mena I 1995 Dietary changes, compulsions and sexual behavior in frontotemporal degeneration Dementia 6 4 195 199 7550598
  127. Miller BL Ponton M Benson DF Cummings JL Mena I 1996 Enhanced artistic creativity with temporal lobe degeneration [letter] Lancet 348 9043 1744 1745 8973469
  128. Mosca L Lunetta C Tarlarini C Avemaria F Maestri E Melazzini M Penco S 2012 Wide phenotypic spectrum of the TARDBP gene: homozygosity of A382T mutation in a patient presenting with amyotrophic lateral sclerosis, Parkinson’s disease, and frontotemporal lobar degeneration, and in neurologically healthy subject Neurobiol Aging 33 8 1846 e1841 1844 10.1016/j.neurobiolaging.2012.01.108 22398199 · DOI
  129. Munoz DG Neumann M Kusaka H Yokota O Ishihara K Terada S Mackenzie IR 2009 FUS pathology in basophilic inclusion body disease Acta Neuropathol 118 5 617 627 10.1007/s00401-009-0598-9 19830439 · DOI
  130. Myers AJ Pittman AM Zhao AS Rohrer K Kaleem M Marlowe L Hardy J 2007 The MAPT H1c risk haplotype is associated with increased expression of tau and especially of 4 repeat containing transcripts Neurobiol Dis 25 3 561 570 10.1016/j.nbd.2006.10.018 17174556 · DOI
  131. Narvid J Gorno-Tempini ML Slavotinek A Dearmond SJ Cha YH Miller BL Rankin K 2009 Of brain and bone: the unusual case of Dr. A Neurocase 15 3 190 205 10.1080/13554790802632967 20183548 PMC2997763 · DOI
  132. Neary D Snowden JS Gustafson L Passant U Stuss D Black S Benson DF 1998 Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria Neurology 51 6 1546 1554 9855500
  133. Neumann M Bentmann E Dormann D Jawaid A DeJesus-Hernandez M Ansorge O Mackenzie IR 2011 FET proteins TAF15 and EWS are selective markers that distinguish FTLD with FUS pathology from amyotrophic lateral sclerosis with FUS mutations Brain 134 Pt 9 2595 2609 10.1093/brain/awr201 21856723 PMC3170539 · DOI
  134. Neumann M Mackenzie IR Cairns NJ Boyer PJ Markesbery WR Smith CD Forman MS 2007 TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations J Neuropathol Exp Neurol 66 2 152 157 10.1097/nen.0b013e31803020b9 17279000 · DOI
  135. Neumann M Rademakers R Roeber S Baker M Kretzschmar HA Mackenzie IR 2009 A new subtype of frontotemporal lobar degeneration with FUS pathology Brain 132 Pt 11 2922 2931 doi:awp214 [pii] 10.1093/brain/awp214 19674978 PMC2768659 · DOI · Paper page
  136. Neumann M Roeber S Kretzschmar HA Rademakers R Baker M Mackenzie IR 2009 Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease Acta Neuropathol 118 5 605 616 10.1007/s00401-009-0581-5 19669651 PMC2864784 · DOI
  137. Neumann M Sampathu DM Kwong LK Truax AC Micsenyi MC Chou TT Lee VM 2006 Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis Science 314 5796 130 133 10.1126/science.1134108 17023659 · DOI · Paper page
  138. Ogar J Willock S Baldo J Wilkins D Ludy C Dronkers N 2006 Clinical and anatomical correlates of apraxia of speech Brain Lang 97 3 343 350 10.1016/j.bandl.2006.01.008 16516956 · DOI
  139. Olney RK Murphy J Forshew D Garwood E Miller BL Langmore S Lomen-Hoerth C 2005 The effects of executive and behavioral dysfunction on the course of ALS Neurology 65 11 1774 1777 16344521
  140. Perry DC Whitwell JL Boeve BF Pankratz VS Knopman DS Petersen RC Josephs KA 2012 Voxel-based morphometry in patients with obsessive-compulsive behaviors in behavioral variant frontotemporal dementia Eur J Neurol 19 6 911 917 10.1111/j.1468-1331.2011.03656.x 22284815 PMC3351534 · DOI
  141. Petkau TL Leavitt BR 2014 Progranulin in neurodegenerative disease Trends Neurosci 37 7 388 398 10.1016/j.tins.2014.04.003 24800652 · DOI
  142. Piguet O 2011 Eating disturbance in behavioural-variant frontotemporal dementia J Mol Neurosci 45 3 589 593 10.1007/s12031-011-9547-x 21584651 · DOI
  143. Pijnenburg YA Sampson EL Harvey RJ Fox NC Rossor MN 2003 Vulnerability to neuroleptic side effects in frontotemporal lobar degeneration Int J Geriatr Psychiatry 18 1 67 72 10.1002/gps.774 12497558 · DOI
  144. Poorkaj P Bird TD Wijsman E Nemens E Garruto RM Anderson L Schellenberg GD 1998 Tau is a candidate gene for chromosome 17 frontotemporal dementia Annals of neurology 43 6 815 825 10.1002/ana.410430617 9629852 · DOI · Paper page
  145. Pottier C Ravenscroft TA Sanchez-Contreras M Rademakers R 2016 Genetics of FTLD: overview and what else we can expect from genetic studies J Neurochem 138 Suppl 1 32 53 10.1111/jnc.13622 27009575 · DOI · Paper page
  146. Proudfoot M Gutowski NJ Edbauer D Hilton DA Stephens M Rankin J Mackenzie IR 2014 Early dipeptide repeat pathology in a frontotemporal dementia kindred with C9ORF72 mutation and intellectual disability Acta Neuropathol 127 3 451 458 10.1007/s00401-014-1245-7 24445903 · DOI
  147. Rabinovici GD Jagust WJ Furst AJ Ogar JM Racine CA Mormino EC Gorno-Tempini ML 2008 Abeta amyloid and glucose metabolism in three variants of primary progressive aphasia Ann Neurol 64 4 388 401 18991338 PMC2648510
  148. Rabinovici GD Rosen HJ Alkalay A Kornak J Furst AJ Agarwal N Jagust WJ 2011 Amyloid vs FDG-PET in the differential diagnosis of AD and FTLD Neurology 77 23 2034 2042 doi:WNL.0b013e31823b9c5e [pii] 10.1212/WNL.0b013e31823b9c5e 22131541 PMC3236517 · DOI · Paper page
  149. Ranasinghe KG Rankin KP Lobach IV Kramer JH Sturm VE Bettcher BM Miller BL 2016 Cognition and neuropsychiatry in behavioral variant frontotemporal dementia by disease stage Neurology 86 7 600 610 10.1212/WNL.0000000000002373 26802093 PMC4762418 · DOI · Paper page
  150. Rankin KP Baldwin E Pace-Savitsky C Kramer JH Miller BL 2005 Self awareness and personality change in dementia J Neurol Neurosurg Psychiatry 76 5 632 639 15834018 PMC1739614
  151. Rankin KP Mayo MC Seeley WW Lee S Rabinovici G Gorno-Tempini ML Miller BL 2011 Behavioral variant frontotemporal dementia with corticobasal degeneration pathology: phenotypic comparison to bvFTD with Pick’s disease J Mol Neurosci 45 3 594 608 10.1007/s12031-011-9615-2 21881831 PMC3208125 · DOI
  152. Rascovsky K Hodges JR Knopman D Mendez MF Kramer JH Neuhaus J Miller BL 2011 Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia Brain 134 Pt 9 2456 2477 doi:awr179 [pii] 10.1093/brain/awr179 21810890 PMC3170532 · DOI · Paper page
  153. Rebeiz JJ Kolodny EH Richardson EP Jr 1968 Corticodentatonigral degeneration with neuronal achromasia Arch Neurol 18 1 20 33 5634369
  154. Rohrer JD Rosen HJ 2013 Neuroimaging in frontotemporal dementia Int Rev Psychiatry 25 2 221 229 10.3109/09540261.2013.778822 23611351 · DOI
  155. Rohrer JD Warren JD 2011 Phenotypic signatures of genetic frontotemporal dementia Curr Opin Neurol 24 6 542 549 10.1097/WCO.0b013e32834cd442 21986680 · DOI
  156. Rohrer JD Woollacott IO Dick KM Brotherhood E Gordon E Fellows A Zetterberg H 2016 Serum neurofilament light chain protein is a measure of disease intensity in frontotemporal dementia Neurology 10.1212/WNL.0000000000003154 PMC5047041 27581216 · DOI
  157. Rojas JC Karydas A Bang J Tsai RM Blennow K Liman V Boxer AL 2016 Plasma neurofilament light chain predicts progression in progressive supranuclear palsy Ann Clin Transl Neurol 3 3 216 225 10.1002/acn3.290 27042681 PMC4774256 · DOI · Paper page
  158. Roman G 2003 Vascular dementia: a historical background Int Psychogeriatr 15 Suppl 1 11 13 10.1017/S1041610203008901 16191211 · DOI
  159. Rosen HJ Allison SC Schauer GF Gorno-Tempini ML Weiner MW Miller BL 2005 Neuroanatomical correlates of behavioural disorders in dementia Brain 128 Pt 11 2612 2625 16195246 PMC1820861
  160. Rosen HJ Gorno-Tempini ML Goldman WP Perry RJ Schuff N Weiner M Miller BL 2002 Patterns of brain atrophy in frontotemporal dementia and semantic dementia Neurology 58 2 198 208 11805245
  161. Rosen HJ Lengenfelder J Miller B 2000 Frontotemporal dementia Neurol Clin 18 4 979 992 11072270
  162. Rosen HJ Perry RJ Murphy J Kramer JH Mychack P Schuff N Miller BL 2002 Emotion comprehension in the temporal variant of frontotemporal dementia Brain 125 Pt 10 2286 2295 12244085
  163. Rosso SM Roks G Stevens M de Koning I Tanghe HLJ Kamphorst W van Swieten JC 2001 Complex compulsive behaviour in the temporal variant of frontotemporal dementia J Neurol 248 11 965 970 11757960
  164. Ryan NS Rossor MN Fox NC 2015 Alzheimer’s disease in the 100 years since Alzheimer’s death Brain 138 Pt 12 3816 3821 10.1093/brain/awv316 26541346 · DOI · Paper page
  165. Schneider A Mandelkow E 2008 Tau-based treatment strategies in neurodegenerative diseases Neurotherapeutics 5 3 443 457 10.1016/j.nurt.2008.05.006 18625456 PMC5084246 · DOI
  166. Schroeter ML Raczka K Neumann J von Cramon DY 2008 Neural networks in frontotemporal dementia–a meta-analysis Neurobiol Aging 29 3 418 426 10.1016/j.neurobiolaging.2006.10.023 17140704 · DOI
  167. Seeley WW 2008 Selective functional, regional, and neuronal vulnerability in frontotemporal dementia Curr Opin Neurol 21 6 701 707 18989116 PMC2909835
  168. Seeley WW Bauer AM Miller BL Gorno-Tempini ML Kramer JH Weiner M Rosen HJ 2005 The natural history of temporal variant frontotemporal dementia Neurology 64 8 1384 1390 10.1212/01.WNL.0000158425.46019.5C 15851728 PMC2376750 · DOI
  169. Seeley WW Carlin DA Allman JM Macedo MN Bush C Miller BL Dearmond SJ 2006 Early frontotemporal dementia targets neurons unique to apes and humans Ann Neurol 60 6 660 667 17187353
  170. Seeley WW Zhou J Kim EJ 2012 Frontotemporal Dementia: What Can the Behavioral Variant Teach Us about Human Brain Organization? Neuroscientist 18 4 373 385 doi:1073858411410354 [pii] 10.1177/1073858411410354 21670424 · DOI
  171. Sha SJ Takada LT Rankin KP Yokoyama JS Rutherford NJ Fong JC Boxer AL 2012 Frontotemporal dementia due to C9ORF72 mutations: Clinical and imaging features Neurology 79 10 1002 1011 doi:WNL.0b013e318268452e [pii] 10.1212/WNL.0b013e318268452e 22875087 PMC3430713 · DOI
  172. Smith KR Damiano J Franceschetti S Carpenter S Canafoglia L Morbin M Berkovic SF 2012 Strikingly different clinicopathological phenotypes determined by progranulin-mutation dosage Am J Hum Genet 90 6 1102 1107 10.1016/j.ajhg.2012.04.021 22608501 PMC3370276 · DOI · Paper page
  173. Snowden JS Adams J Harris J Thompson JC Rollinson S Richardson A Pickering-Brown S 2015 Distinct clinical and pathological phenotypes in frontotemporal dementia associated with MAPT, PGRN and C9orf72 mutations Amyotroph Lateral Scler Frontotemporal Degener 16 7–8 497 505 10.3109/21678421.2015.1074700 26473392 · DOI
  174. Snowden JS Hu Q Rollinson S Halliwell N Robinson A Davidson YS Mann DM 2011 The most common type of FTLD-FUS (aFTLD-U) is associated with a distinct clinical form of frontotemporal dementia but is not related to mutations in the FUS gene Acta Neuropathol 122 1 99 110 10.1007/s00401-011-0816-0 21424531 · DOI
  175. Snowden JS Neary D Mann DM 2002 Frontotemporal dementia Br J Psychiatry 180 140 143 11823324
  176. Spillantini MG Murrell JR Goedert M Farlow MR Klug A Ghetti B 1998 Mutation in the tau gene in familial multiple system tauopathy with presenile dementia Proceedings of the National Academy of Sciences of the United States of America 95 13 7737 7741 9636220 PMC22742
  177. Steele JC Richardson JC Olszewski J 1964 Progressive Supranuclear Palsy Arch Neurol 10 4 333 360 14107684
  178. Suarez J Tartaglia MC Vitali P Erbetta A Neuhaus J Laluz V Miller BL 2009 Characterizing radiology reports in patients with frontotemporal dementia Neurology 73 13 1073 1074 10.1212/WNL.0b013e3181b9c8a6 19786700 PMC2754327 · DOI
  179. Swartz JR Miller BL Lesser IM Darby AL 1997 Frontotemporal dementia: treatment response to serotonin selective reuptake inhibitors J Clin Psychiatry 58 5 212 216 9184615
  180. Tacik P DeTure M Hinkle KM Lin WL Sanchez-Contreras M Carlomagno Y Dickson DW 2015 A Novel Tau Mutation in Exon 12, p.Q336H, Causes Hereditary Pick Disease Journal of neuropathology and experimental neurology 74 11 1042 1052 10.1097/NEN.0000000000000248 26426266 PMC4607649 · DOI
  181. Tekin S Cummings JL 2002 Frontal-subcortical neuronal circuits and clinical neuropsychiatry: an update J Psychosom Res 53 2 647 654 12169339
  182. Thal DR Schultz C Botez G Del Tredici K Mrak RE Griffin WS Ghebremedhin E 2005 The impact of argyrophilic grain disease on the development of dementia and its relationship to concurrent Alzheimer’s disease-related pathology Neuropathol Appl Neurobiol 31 3 270 279 10.1111/j.1365-2990.2005.00635.x 15885064 · DOI · Paper page
  183. Thibodeau MP Miller BL 2013 ‘Limits and current knowledge of Pick’s disease: its differential diagnosis’. A translation of the 1957 Delay, Brion, Escourolle article Neurocase 19 5 417 422 10.1080/13554794.2012.667133 22554132 · DOI
  184. Tranel D Bechara A Denburg NL 2002 Asymmetric functional roles of right and left ventromedial prefrontal cortices in social conduct, decision-making, and emotional processing Cortex 38 4 589 612 12465670
  185. Tsai RM Boxer AL 2016 Therapy and clinical trials in frontotemporal dementia: past, present, and future J Neurochem 138 Suppl 1 211 221 10.1111/jnc.13640 27306957 PMC5217534 · DOI
  186. Tsuchiya K Mitani K Arai T Yamada S Komiya T Esaki Y Ikeda K 2001 Argyrophilic grain disease mimicking temporal Pick’s disease: a clinical, radiological, and pathological study of an autopsy case with a clinical course of 15 years Acta Neuropathol 102 2 195 199 11563637
  187. Urwin H Josephs KA Rohrer JD Mackenzie IR Neumann M Authier A Isaacs AM 2010 FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration Acta Neuropathol 120 1 33 41 20490813 PMC2887939
  188. Van Damme P Van Hoecke A Lambrechts D Vanacker P Bogaert E van Swieten J Robberecht W 2008 Progranulin functions as a neurotrophic factor to regulate neurite outgrowth and enhance neuronal survival J Cell Biol 181 1 37 41 10.1083/jcb.200712039 18378771 PMC2287280 · DOI
  189. van Swieten JC Heutink P 2008 Mutations in progranulin (GRN) within the spectrum of clinical and pathological phenotypes of frontotemporal dementia Lancet Neurol 7 10 965 974 10.1016/S1474-4422(08)70194-7 18771956 · DOI
  190. Vance C Rogelj B Hortobagyi T De Vos KJ Nishimura AL Sreedharan J Shaw CE 2009 Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6 Science 323 5918 1208 1211 10.1126/science.1165942 19251628 PMC4516382 · DOI
  191. Vatsavayai SC Yoon SJ Gardner RC Gendron TF Vargas JN Trujillo A Seeley WW 2016 Timing and significance of pathological features in C9orf72 expansion-associated frontotemporal dementia Brain 10.1093/brain/aww250 PMC5790143 27797809 · DOI
  192. Vercelletto M Boutoleau-Bretonniere C Volteau C Puel M Auriacombe S Sarazin M French research network on Frontotemporal, d. 2011 Memantine in behavioral variant frontotemporal dementia: negative results J Alzheimers Dis 23 4 749 759 10.3233/JAD-2010-101632 21157021 · DOI
  193. Wadia PM Lang AE 2007 The many faces of corticobasal degeneration Parkinsonism Relat Disord 13 Suppl 3 S336 340 10.1016/S1353-8020(08)70027-0 18267261 · DOI
  194. Walker LC Jucker M 2015 Neurodegenerative diseases: expanding the prion concept Annu Rev Neurosci 38 87 103 10.1146/annurev-neuro-071714-033828 25840008 PMC4803040 · DOI · Paper page
  195. Ward ME Miller BL 2011 Potential mechanisms of progranulin-deficient FTLD J Mol Neurosci 45 3 574 582 10.1007/s12031-011-9622-3 21892758 PMC3767571 · DOI
  196. Watts GD Thomasova D Ramdeen SK Fulchiero EC Mehta SG Drachman DA Kimonis VE 2007 Novel VCP mutations in inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia Clin Genet 72 5 420 426 10.1111/j.1399-0004.2007.00887.x 17935506 · DOI
  197. Weingarten MD Lockwood AH Hwo SY Kirschner MW 1975 A protein factor essential for microtubule assembly Proc Natl Acad Sci U S A 72 5 1858 1862 1057175 PMC432646
  198. Whitwell JL Jack CR Jr Boeve BF Parisi JE Ahlskog JE Drubach DA Josephs KA 2010 Imaging correlates of pathology in corticobasal syndrome Neurology 75 21 1879 1887 10.1212/WNL.0b013e3181feb2e8 21098403 PMC2995388 · DOI
  199. Whitwell JL Jack CR Jr Boeve BF Senjem ML Baker M Rademakers R Josephs KA 2009 Voxel-based morphometry patterns of atrophy in FTLD with mutations in MAPT or PGRN Neurology 72 9 813 820 10.1212/01.wnl.0000343851.46573.67 72/9/813 [pii] 19255408 PMC2677544 · DOI
  200. Whitwell JL Sampson EL Loy CT Warren JE Rossor MN Fox NC Warren JD 2007 VBM signatures of abnormal eating behaviours in frontotemporal lobar degeneration Neuroimage 35 1 207 213 10.1016/j.neuroimage.2006.12.006 17240166 · DOI

200 of 208 shown

Cited by

Sorted by citations

Authors
Olney, N. T., Spina, S., & Miller, B. L.
Affiliations
University of California, San Francisco (US)
Corresponding author
Olney, Nicholas T.
ORCID
Venue
Neurologic Clinics
Publisher
Elsevier BV
Published
2017-05
Volume
35
Issue
2
Pages
339-374
ISSN
0733-8619 (ISSN-L)
DOI
10.1016/j.ncl.2017.01.008
PMC
PMC5472209
OpenAlex
W4210983111
Cited by
419
References
26
Citations by year
2026: 32 · 2025: 48 · 2024: 57 · 2023: 57 · 2022: 50 · 2021: 63 · 2020: 52 · 2019: 34 · 2018: 21 · 2017: 3 · 2014: 1
FWCI
4.57
Type
journal article
Language
en
Open access
Licence
www.elsevier.com/tdm/userlicense/1.0/ (tdm)
Locations
  • Neurologic Clinics (publishedVersion)
  • PubMed (publishedVersion)
  • Europe PMC (PubMed Central) (acceptedVersion)
  • PubMed Central (acceptedVersion)
  • eScholarship (California Digital Library) (submittedVersion)
Funders
National Institute on Aging (K08AG052648, K24AG045333, P01AG019724, T32AG023481, P50AG023501)
Topics
  • Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
  • Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
  • Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
Keywords
frontotemporal dementia, behavioral variant FTD, primary progressive aphasia, semantic variant PPA, progressive supranuclear palsy, corticobasal syndrome, clinical phenotype, diagnostic criteria, neuropathology, treatment
MeSH terms
Brain, Humans, Supranuclear Palsy, Progressive, History, 20th Century, Frontotemporal Dementia

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