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Highlights & Basics
- Frontotemporal dementia manifests primarily as disruption in personality and social conduct, or as a primary language disorder.
- Almost 50% of affected people display parkinsonism; a smaller subset may have motor neuron disease.
- Clinical diagnosis derives primarily from examination and brain imaging.
- Diagnostic confirmation is based on pathologic exam or identification of gene mutation.
- Treatment is supportive, combining pharmacotherapy with nonpharmacologic and behavioral interventions, caregiver guidance, community services, and social work involvement.
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History & Exam
Key Factors
Other Factors
Diagnostics Tests
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Definition
Epidemiology
Etiology
Pathophysiology
Images
Clinical features aiding the differential diagnosis between behavioral variant FTD (bvFTD) and primary psychiatric illness
Neuroimaging patterns associated with behavioural variant FTD (bvFTD) and nonfluent variant primary progressive aphasia (nfvPPA). Structural MRI and FDG-PET demonstrating the variability in patterns of atrophy and hypometabolism in FTD. In the case of bvFTD, significant bilateral frontal lobe atrophy and hypometabolism is seen. In the case of nfvPPA, atrophy and hypometabolism is lateralised and is greatly impacting the left frontal lobe more so than the right.
Coronal T1-weighted MRI (a, b) and fused FDG-PET MRI (c) in a patient with semantic dementia
Citations
Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain. 2011 Sep;134(Pt 9):2456-77.[Abstract][Full Text]
American College of Radiology. ACR appropriateness criteria: dementia. 2024 [internet publication].[Full Text]
Buoli M, Serati M, Caldiroli A, et al. Pharmacological management of psychiatric symptoms in frontotemporal dementia: a systematic review. J Geriatr Psychiatry Neurol. 2017 May;30(3):162-9.[Abstract]
Reus VI, Fochtmann LJ, Eyler AE, et al. The American Psychiatric Association practice guideline on the use of antipsychotics to treat agitation or psychosis in patients with dementia. Am J Psychiatry. 2016 May 1;173(5):543-6.[Abstract][Full Text]
1. Ratnavalli E, Brayne C, Dawson K, et al. The prevalence of frontotemporal dementia. Neurology. 2002 Jun 11;58(11):1615-21.[Abstract]
2. Rosso SM, Donker Kaat L, Baks T, et al. Frontotemporal dementia in the Netherlands: patient characteristics and prevalence estimates from a population-based study. Brain. 2003 Sep;126(Pt 9):2016-22.[Abstract][Full Text]
3. Johnson JK, Diehl J, Mendez MF, et al. Frontotemporal lobar degeneration: demographic characteristics of 353 patients. Arch Neurol. 2005 Jun;62(6):925-30.[Abstract][Full Text]
4. Coyle-Gilchrist IT, Dick KM, Patterson K, et al. Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes. Neurology. 2016 May 3;86(18):1736-43.[Abstract][Full Text]
5. Piguet O, Hornberger M, Mioshi E, et al. Behavioral-variant frontotemporal dementia: diagnosis, clinical staging and management. Lancet Neurol. 2011 Feb;10(2):162-72.[Abstract]
6. Boeve BF, Boxer AL, Kumfor F, et al. Advances and controversies in frontotemporal dementia: diagnosis, biomarkers, and therapeutic considerations. Lancet Neurol. 2022 Mar;21(3):258-72.[Abstract]
7. Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain. 2011 Sep;134(Pt 9):2456-77.[Abstract][Full Text]
8. Harris JM, Gall C, Thompson JC, et al. Sensitivity and specificity of FTDC criteria for behavioral variant frontotemporal dementia. Neurology. 2013 May 14;80(20):1881-7.[Abstract]
9. Warren JD, Rohrer JD, Rossor MN. Clinical review. Frontotemporal dementia. BMJ. 2013 Aug 6;347:f4827.[Abstract][Full Text]
10. Barker MS, Gottesman RT, Manoochehri M, et al. Proposed research criteria for prodromal behavioural variant frontotemporal dementia. Brain. 2022 Apr 29;145(3):1079-97.[Abstract][Full Text]
11. Gorno-Tempini ML, Hillis AE, Weintraub S, et al. Classification of primary progressive aphasia and its variants. Neurology. 2011 Mar 15;76(11):1006-14.[Abstract][Full Text]
12. Mesulam M, Wicklund A, Johnson N, et al. Alzheimer and frontotemporal pathology in subsets of primary progressive aphasia. Ann Neurol. 2008 Jun;63(6):709-19.[Abstract][Full Text]
13. Sajjadi SA, Patterson K, Arnold RJ, et al. Primary progressive aphasia: a tale of two syndromes and the rest. Neurology. 2012 May 22;78(21):1670-7.[Abstract][Full Text]
14. Harris JM, Gall C, Thompson JC, et al. Classification and pathology of primary progressive aphasia. Neurology. 2013 Nov 19;81(21):1832-9.[Abstract]
15. Diehl-Schmid J, Schūlte-Overberg J, Hartmann J, et al. Extrapyramidal signs, primitive reflexes and incontinence in fronto-temporal dementia. Eur J Neurol. 2007 Aug;14(8):860-4.[Abstract]
16. Strong MJ, Abrahams S, Goldstein LH, et al. Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): revised diagnostic criteria. Amyotroph Lateral Scler Frontotemporal Degener. 2017 May;18(3-4):153-74.[Abstract][Full Text]
17. Armstrong MJ, Litvan I, Lang AE, et al. Criteria for the diagnosis of corticobasal degeneration. Neurology. 2013 Jan 29;80(5):496-503.[Abstract][Full Text]
18. Höglinger GU, Respondek G, Stamelou M, et al. Clinical diagnosis of progressive supranuclear palsy: the movement disorder society criteria. Mov Disord. 2017 Jun;32(6):853-64.[Abstract][Full Text]
19. Josephs KA. Frontotemporal dementia and related disorders: deciphering the enigma. Ann Neurol. 2008 Jul;64(1):4-14.[Abstract]
20. Bang J, Spina S, Miller BL. Frontotemporal dementia. Lancet. 2015 Oct 24;386(10004):1672-82.[Abstract][Full Text]
21. Mackenzie IR, Neumann M, Bigio EH, et al. Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update. Acta Neuropathol. 2010 Jan;119(1):1-4.[Abstract][Full Text]
22. American Psychiatric Association. Diagnostic and statistical manual of mental disorders, 5th ed., text revision (DSM-5-TR). Washington, DC: American Psychiatric Publishing; 2022.[Full Text]
23. American Psychiatric Association. DSM-5-TR neurocognitive disorders supplement. Updated excerpts for delirium codes major and mild neurocognitive disorders. October 2022.[Full Text]
24. Hogan DB, Jetté N, Fiest KM, et al. The prevalence and incidence of frontotemporal dementia: a systematic review. Can J Neurol Sci. 2016 Apr;43 Suppl 1:S96-S109.[Abstract][Full Text]
25. Snowden JS, Neary D, Mann DM. Frontotemporal dementia. Br J Psychiatry. 2002 Feb;180:140-3.[Abstract]
26. Knopman DS, Mastri AR, Frey WH 2nd, et al. Dementia lacking distinctive histologic features: a common non-Alzheimer degenerative dementia. Neurology. 1990 Feb;40(2):251-6.[Abstract]
27. Barker WW, Luis CA, Kashuba A, et al. Relative frequencies of Alzheimer disease, Lewy body, vascular and frontotemporal dementia, and hippocampal sclerosis in the State of Florida Brain Bank. Alzheimer Dis Assoc Disord. 2002 Oct-Dec;16(4):203-12.[Abstract]
28. Ducharme S, Dols A, Laforce R, et al. Recommendations to distinguish behavioural variant frontotemporal dementia from psychiatric disorders. Brain. 2020 Jun 1;143(6):1632-50.[Abstract][Full Text]
29. Baborie A, Griffiths TD, Jaros E, et al. Frontotemporal dementia in elderly individuals. Arch Neurol. 2012 Aug;69(8):1052-60.[Abstract]
30. Moore KM, Nicholas J, Grossman M, et al. Age at symptom onset and death and disease duration in genetic frontotemporal dementia: an international retrospective cohort study. Lancet Neurol. 2020 Feb;19(2):145-56.[Abstract][Full Text]
31. Gilberti N, Turla M, Alberici A, et al. Prevalence of frontotemporal lobar degeneration in an isolated population: the Vallecamonica study. Neurol Sci. 2012 Aug;33(4):899-904.[Abstract]
32. Hou CE, Yaffe K, Pérez-Stable EJ, et al. Frequency of dementia etiologies in four ethnic groups. Dement Geriatr Cogn Disord. 2006;22(1):42-7.[Abstract]
33. Jin HA, McMillan CT, Yannatos I, et al. Racial differences in clinical presentation in individuals diagnosed with frontotemporal dementia. JAMA Neurol. 2023 Nov 1;80(11):1191-8.[Abstract][Full Text]
34. Rohrer JD, Guerriro R, Vandrocova J, et al. The hereditability and genetics of frontotemporal lobar degeneration. Neurology. 2009 Nov 3;73(18):1451-6.[Abstract][Full Text]
35. Greaves CV, Rohrer JD. An update on genetic frontotemporal dementia. J Neurol. 2019 Aug;266(8):2075-86.[Abstract][Full Text]
36. Wood EM, Falcone D, Suh E, et al. Development and validation of pedigree classification criteria for frontotemporal lobar degeneration. JAMA Neurol. 2013 Nov;70(11):1411-7.[Abstract][Full Text]
37. Hutton M, Lendon CL, Rizzu P, et al. Association of missense and 5'-splice-site mutations in tau with the inherited dementia FTDP-17. Nature. 1998 Jun 18;393(6686):702-5.[Abstract]
38. Baker M, Mackenzie IR, Pickering-Brown SM, et al. Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17. Nature. 2006 Aug 24;442(7105):916-9.[Abstract]
39. 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 Aug 24;442(7105):920-4.[Abstract]
40. 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 Oct 20;72(2):245-56.[Abstract][Full Text]
41. Byrne S, Elamin M, Bede P, et al. Cognitive and clinical characteristics of patients with amyotrophic lateral sclerosis carrying a C9orf72 repeat expansion: a population-based cohort study. Lancet Neurol. 2012 Mar;11(3):232-40.[Abstract][Full Text]
42. Balendra R, Isaacs AM. C9orf72-mediated ALS and FTD: multiple pathways to disease. Nat Rev Neurol. 2018 Sep;14(9):544-58.[Abstract][Full Text]
43. Mol MO, van Rooij JGJ, Wong TH, et al. Underlying genetic variation in familial frontotemporal dementia: sequencing of 198 patients. Neurobiol Aging. 2021 Jan;97:148.e9-148.e16.[Abstract][Full Text]
44. Borroni B, Bonvicini C, Galimberti D, et al. Founder effect and estimation of the age of the Progranulin Thr272fs mutation in 14 Italian pedigrees with frontotemporal lobar degeneration. Neurobiol Aging. 2011 Mar;32(3):555.e1-8.[Abstract]
45. Deutsch MB, Mendez MF, Teng E. Interactions between traumatic brain injury and frontotemporal degeneration. Dement Geriatr Cogn Disord. 2015;39(3-4):143-53.[Abstract][Full Text]
46. Wang HK, Lee YC, Huang CY, et al. Traumatic brain injury causes frontotemporal dementia and TDP-43 proteolysis. Neuroscience. 2015 Aug 6;300:94-103.[Abstract]
47. Huang CH, Lin CW, Lee YC, et al. Is traumatic brain injury a risk factor for neurodegeneration? A meta-analysis of population-based studies. BMC Neurol. 2018 Nov 5;18(1):184.[Abstract][Full Text]
48. Günak MM, Billings J, Carratu E, et al. Post-traumatic stress disorder as a risk factor for dementia: systematic review and meta-analysis. Br J Psychiatry. 2020 Nov;217(5):600-8.[Abstract][Full Text]
49. Bonanni L, Franciotti R, Martinotti G, et al. Post traumatic stress disorder heralding the onset of semantic frontotemporal dementia. J Alzheimers Dis. 2018;63(1):203-15.[Abstract][Full Text]
50. Josephs KA, Hodges JR, Snowden JS, et al. Neuropathological background of phenotypical variability in frontotemporal dementia. Acta Neuropathol. 2011 Aug;122(2):137-53.[Abstract][Full Text]
51. Mackenzie IR, Neumann M, Baborie A, et al. A harmonized classification system for FTLD-TDP pathology. Acta Neuropathol. 2011 Jul;122(1):111-3.[Abstract][Full Text]
52. Neumann M, Mackenzie IRA. Review: Neuropathology of non-tau frontotemporal lobar degeneration. Neuropathol Appl Neurobiol. 2019 Feb;45(1):19-40.[Abstract]
53. Mackenzie IR, Neumann M. Molecular neuropathology of frontotemporal dementia: insights into disease mechanisms from postmortem studies. J Neurochem. 2016 Aug;138(suppl 1):54-70.[Abstract][Full Text]
54. Lee G, Leugers CJ. Tau and tauopathies. Prog Mol Biol Transl Sci. 2012;107:263-93.[Abstract][Full Text]
55. Rademakers R, Neumann M, Mackenzie IR. Advances in understanding the molecular basis of frontotemporal dementia. Nat Rev Neurol. 2012 Aug;8(8):423-34.[Abstract][Full Text]
56. Paloneva J, Kestilä M, Wu J, et al. Loss-of-function mutations in TYROBP (DAP12) result in a presenile dementia with bone cysts. Nat Genet. 2000 Jul;25(3):357-61.[Abstract]
57. Paloneva J, Manninen T, Christman G, et al. Mutations in two genes encoding different subunits of a receptor signaling complex result in an identical disease phenotype. Am J Hum Genet. 2002 Sep;71(3):656-62.[Abstract][Full Text]
58. Kennedy DP, Adolphs R. The social brain in psychiatric and neurological disorders. Trends Cogn Sci. 2012 Nov;16(11):559-72.[Abstract][Full Text]
59. Boeve BF. Links between frontotemporal lobar degeneration, corticobasal degeneration, progressive supranuclear palsy, and amyotrophic lateral sclerosis. Alzheimer Dis Assoc Disord. 2007 Oct-Dec;21(4):S31-8.[Abstract]
60. Antonioni A, Raho EM, Lopriore P, et al. Frontotemporal dementia, where do we stand? a narrative review. Int J Mol Sci. 2023 Jul 21;24(14):11732.[Abstract][Full Text]
61. LoBue C, Wilmoth K, Cullum CM, et al. Traumatic brain injury history is associated with earlier age of onset of frontotemporal dementia. J Neurol Neurosurg Psychiatry. 2016 Aug;87(8):817-20.[Abstract][Full Text]
62. Livingston G, Huntley J, Liu KY, et al. Dementia prevention, intervention, and care: 2024 report of the Lancet standing Commission. Lancet. 2024 Aug 10;404(10452):572-628.[Abstract]
63. Ma Y, Ajnakina O, Steptoe A, et al. Higher risk of dementia in english older individuals who are overweight or obese. Int J Epidemiol. 2020 Aug 1;49(4):1353-65.[Abstract][Full Text]
64. Cantuaria ML, Pedersen ER, Waldorff FB, et al. Hearing loss, hearing aid use, and risk of dementia in older adults. JAMA Otolaryngol Head Neck Surg. 2024 Feb 1;150(2):157-64.[Abstract][Full Text]
65. Grenier B, Berr C, Goldberg M, et al. Hearing loss, hearing aids, and cognition. JAMA Netw Open. 2024 Oct 1;7(10):e2436723.[Abstract][Full Text]
66. Royal College of Ophthalmologists. Cataract surgery in patients living with dementia. Nov 2023 [internet publication].[Full Text]
67. Del Pozo Cruz B, Ahmadi M, Naismith SL, et al. Association of daily step count and intensity with incident dementia in 78 430 adults living in the UK. JAMA Neurol. 2022 Oct 1;79(10):1059-63.[Abstract][Full Text]
68. Iso-Markku P, Aaltonen S, Kujala UM, et al. Physical activity and cognitive decline among older adults: a systematic review and meta-analysis. JAMA Netw Open. 2024 Feb 5;7(2):e2354285.[Abstract][Full Text]
69. Tessier AJ, Cortese M, Yuan C, et al. Consumption of olive oil and diet quality and risk of dementia-related death. JAMA Netw Open. 2024 May 1;7(5):e2410021.[Abstract][Full Text]
70. Vyas CM, Manson JE, Sesso HD, et al. Effect of multivitamin-mineral supplementation versus placebo on cognitive function: results from the clinic subcohort of the COcoa Supplement and Multivitamin Outcomes Study (COSMOS) randomized clinical trial and meta-analysis of 3 cognitive studies within COSMOS. Am J Clin Nutr. 2024 Mar;119(3):692-701.[Abstract][Full Text]
71. Castro CB, Costa LM, Dias CB, et al. Multi-domain interventions for dementia prevention - a systematic review. J Nutr Health Aging. 2023;27(12):1271-80.[Abstract][Full Text]
72. Johnen A, Bertoux M. Psychological and cognitive markers of behavioral variant frontotemporal dementia-a clinical neuropsychologist's view on diagnostic criteria and beyond. Front Neurol. 2019 Jun 7;10:594.[Abstract][Full Text]
73. NHMRC Partnership Centre for Dealing with Cognitive and Related Functional Decline in Older People. Clinical practice guidelines and principles of care for people with dementia. Feb 2016 [internet publication].[Full Text]
74. Olney NT, Spina S, Miller BL. Frontotemporal dementia. Neurol Clin. 2017 May;35(2):339-74.[Abstract][Full Text]
75. Antonioni A, Raho EM, Granieri E, et al. Frontotemporal dementia. How to deal with its diagnostic complexity? Expert Rev Neurother. 2025 Feb 7:1-35.[Abstract][Full Text]
76. Ducharme S, Price BH, Larvie M, et al. Clinical approach to the differential diagnosis between behavioral variant frontotemporal dementia and primary psychiatric disorders. Am J Psychiatry. 2015 Sep 1;172(9):827-37.[Full Text]
77. Pressman PS, Matlock D, Ducharme S. Distinguishing behavioral variant frontotemporal dementia from primary psychiatric disorders: a review of recently published consensus recommendations from the neuropsychiatric international consortium for frontotemporal dementia. J Neuropsychiatry Clin Neurosci. 2021 Spring;33(2):152-6.[Abstract][Full Text]
78. Laganà V, Bruno F, Altomari N, et al. Neuropsychiatric or behavioral and psychological symptoms of dementia (BPSD): focus on prevalence and natural history in alzheimer's disease and frontotemporal dementia. Front Neurol. 2022;13:832199.[Abstract][Full Text]
79. Schwertner E, Pereira JB, Xu H, et al. Behavioral and psychological symptoms of dementia in different dementia disorders: a large-scale study of 10,000 individuals. J Alzheimers Dis. 2022;87(3):1307-18.[Abstract][Full Text]
80. Liljegren M, Landqvist Waldö M, Frizell Santillo A, et al. Association of neuropathologically confirmed frontotemporal dementia and alzheimer disease with criminal and socially inappropriate behavior in a Swedish cohort. JAMA Netw Open. 2019 Mar 1;2(3):e190261.[Abstract][Full Text]
81. Tayim N, Panicker J, Foley J, et al. Impact of hypersexuality on spousal carers of patients with Parkinson's disease and frontotemporal dementia: a qualitative study. BMJ Open. 2025 Apr 10;15(4):e090870.[Abstract][Full Text]
82. Chatterjee A, Hirsch-Reinshagen V, Scott I, et al. A systematic review of the genetics and pathology of psychosis in frontotemporal dementia. Can J Neurol Sci. 2024 May;51(3):369-78.[Abstract][Full Text]
83. Goldman JS, Rademakers R, Huey ED, et al. An algorithm for genetic testing of frontotemporal lobar degeneration. Neurology. 2011 Feb 1;76(5):475-83.[Abstract][Full Text]
84. Davis DH, Creavin ST, Yip JL, et al. Montreal Cognitive Assessment for the detection of dementia. Cochrane Database Syst Rev. 2021 Jul 13;(7):CD010775.[Abstract][Full Text]
85. Coleman KK, Coleman BL, MacKinley JD, et al. Detection and differentiation of frontotemporal dementia and related disorders from alzheimer disease using the Montreal cognitive assessment. Alzheimer Dis Assoc Disord. 2016 Jul-Sep;30(3):258-63.[Abstract]
86. Hsieh S, Schubert S, Hoon C, et al. Validation of the Addenbrooke's Cognitive Examination III in frontotemporal dementia and alzheimer's disease. Dement Geriatr Cogn Disord. 2013;36(3-4):242-50.[Abstract]
87. Dubois B, Slachevsky A, Litvan I, et al. The FAB: a frontal assessment battery at bedside. Neurology. 2000 Dec 12;55(11):1621-6.[Abstract]
88. Royall DR, Mahurin RK, Gray KF. Bedside assessment of executive cognitive impairment: the executive interview. J Am Geriatr Soc. 1992 Dec;40(12):1221-6.[Abstract]
89. Larson EB, Heinemann AW. Rasch analysis of the Executive Interview (The EXIT-25) and introduction of an abridged version (The Quick EXIT). Arch Phys Med Rehabil. 2010 Mar;91(3):389-94.[Abstract][Full Text]
90. Kertesz A, Davidson W, Fox H. Frontal behavioral inventory: diagnostic criteria for frontal lobe dementia. Can J Neurol Sci. 1997 Feb;24(1):29-36.[Abstract]
91. Clinical and neuropathological criteria for frontotemporal dementia. The Lund and Manchester Groups. J Neurol Neurosurg Psychiatry. 1994 Apr;57(4):416-8.[Full Text]
92. Wear HJ, Wedderburn CJ, Mioshi E, et al. The Cambridge behavioural inventory revised. Dement Neuropsychol. 2008 Apr-Jun;2(2):102-7.[Abstract][Full Text]
93. Savage S, Hsieh S, Leslie F, et al. Distinguishing subtypes in primary progressive aphasia: application of the Sydney language battery. Dement Geriatr Cogn Disord. 2013;35(3-4):208-18.[Abstract]
94. Janssen N, Roelofs A, van den Berg E, et al. The diagnostic value of language screening in primary progressive aphasia: validation and application of the Sydney language battery. J Speech Lang Hear Res. 2022 Jan 12;65(1):200-14.[Abstract][Full Text]
95. Gallée J, Volkmer A. Role of the speech-language therapist/pathologist in primary progressive aphasia. Neurol Clin Pract. 2023 Aug;13(4):e200178.[Abstract][Full Text]
96. Mathias JL, Morphett K. Neurobehavioral differences between Alzheimer's disease and frontotemporal dementia: a meta-analysis. J Clin Exp Neuropsychol. 2010 Aug;32(7):682-98.[Abstract]
97. Mioshi E, Hsieh S, Savage S, et al. Clinical staging and disease progression in frontotemporal dementia. Neurology. 2010 May 18;74(20):1591-7.[Abstract]
98. Diehl-Schmid J, Pohl C, Ruprecht C, et al. The Ekman 60 faces test as a diagnostic instrument in frontotemporal dementia. Arch Clin Neuropsychol. 2007 May;22(4):459-64.[Abstract]
99. Kumfor F, Piguet O. Disturbance of emotional processing in frontotemporal dementia: a synthesis of cognitive and neuroimaging findings. Neuropsychol Rev. 2012 Sep;22(3):280-97.[Abstract]
100. Bora E, Velakoulis D, Walterfang M. Meta-analysis of facial emotion recognition in behavioral variant frontotemporal dementia: comparison with alzheimer disease and healthy controls. J Geriatr Psychiatry Neurol. 2016 Jul;29(4):205-11.[Abstract]
101. Stam D, Rosseel S, De Winter FL, et al. Facial expression recognition deficits in frontotemporal dementia and alzheimer's disease: a meta-analytic investigation of effects of phenotypic variant, task modality, geographical region and symptomatic specificity. J Neurol. 2023 Dec;270(12):5731-55.[Abstract]
102. Torralva T, Roca M, Gleichgerrcht E, et al. A neuropsychological battery to detect specific executive and social cognitive impairments in early frontotemporal dementia. Brain. 2009 May;132(pt 5):1299-309.[Abstract]
103. Whitwell JL. FTD spectrum: neuroimaging across the FTD spectrum. Prog Mol Biol Transl Sci. 2019;165:187-223.[Abstract][Full Text]
104. American College of Radiology. ACR appropriateness criteria: dementia. 2024 [internet publication].[Full Text]
105. Agosta F, Galantucci S, Magnani G, et al. MRI signatures of the frontotemporal lobar degeneration continuum. Hum Brain Mapp. 2015 Jul;36(7):2602-14.[Abstract]
106. Frisoni GB, Festari C, Massa F, et al. European intersocietal recommendations for the biomarker-based diagnosis of neurocognitive disorders. Lancet Neurol. 2024 Mar;23(3):302-12.[Abstract]
107. Madhavan A, Whitwell JL, Weigand SD, et al. FDG PET and MRI in logopenic primary progressive aphasia versus dementia of the Alzheimer's type. PLoS One. 2013;8(4):e62471.[Abstract][Full Text]
108. Zhao P, Zhang B, Gao S, et al. Clinical, MRI and 18F-FDG-PET/CT analysis of progressive supranuclear palsy. J Clin Neurosci. 2020 Oct;80:318-23.[Abstract]
109. Hauser RA, Murtaugh FR, Akhter K, et al. Magnetic resonance imaging of corticobasal degeneration. J Neuroimaging. 1996 Oct;6(4):222-6.[Abstract]
110. Savoiardo M, Grisoli M, Girotti F. Magnetic resonance imaging in CBD, related atypical parkinsonian disorders, and dementias. Adv Neurol. 2000;82:197-208.[Abstract]
111. Grijalvo-Perez AM, Litvan I. Corticobasal degeneration. Semin Neurol. 2014 Apr;34(2):160-73.[Abstract][Full Text]
112. Whitwell JL, Jack CR Jr, Senjem ML, et al. Patterns of atrophy in pathologically confirmed FTLD with and without motor neuron degeneration. Neurology. 2006 Jan 10;66(1):102-4.[Abstract][Full Text]
113. Bruun M, Koikkalainen J, Rhodius-Meester HFM, et al. Detecting frontotemporal dementia syndromes using MRI biomarkers. Neuroimage Clin. 2019;22:101711.[Abstract][Full Text]
114. Perry A, Wen W, Lord A, et al. The organisation of the elderly connectome. Neuroimage. 2015 Jul 1;114:414-26.[Abstract]
115. Lam BY, Halliday GM, Irish M, et al. Longitudinal white matter changes in frontotemporal dementia subtypes. Hum Brain Mapp. 2014 Jul;35(7):3547-57.[Abstract]
116. Goldman J. New approaches to genetic counseling and testing for Alzheimer's disease and frontotemporal degeneration. Curr Neurol Neurosci Rep. 2012 Oct;12(5):502-10.[Abstract][Full Text]
117. Burgunder JM, Finsterer J, Szolnoki Z, et al. EFNS guidelines on the molecular diagnosis of channelopathies, epilepsies, migraine, stroke, and dementias. Eur J Neurol. 2010 May;17(5):641-8.[Abstract][Full Text]
118. Fong JC, Karydas AM, Goldman JS. Genetic counseling for FTD/ALS caused by the C9ORF72 hexanucleotide expansion. Alzheimers Res Ther. 2012;4(4):27.[Abstract][Full Text]
119. Quaid KA. Genetic counseling for frontotemporal dementias. J Mol Neurosci. 2011 Nov;45(3):706-9.[Abstract]
120. Hermann P, Zerr I. Rapidly progressive dementias - aetiologies, diagnosis and management. Nat Rev Neurol. 2022 Jun;18(6):363-76.[Abstract][Full Text]
121. Smith SJ. EEG in neurological conditions other than epilepsy: when does it help, what does it add? J Neurol Neurosurg Psychiatry. 2005 Jun;76 Suppl 2(suppl 2):ii8-12.[Full Text]
122. Wieser HG, Schindler K, Zumsteg D. EEG in Creutzfeldt-Jakob disease. Clin Neurophysiol. 2006 May;117(5):935-51.[Abstract]
123. Sen A, Capelli V, Husain M. Cognition and dementia in older patients with epilepsy. Brain. 2018 Jun 1;141(6):1592-608.[Abstract][Full Text]
124. Abbott G, Praschan N, Newhouse A, et al. Biomarkers in frontotemporal dementia: Current landscape and future directions. Biomarkers in Neuropsychiatry. 2023 Jun 1;100065.[Full Text]
125. Miltiadous A, Tzimourta KD, Giannakeas N, et al. Alzheimer's disease and frontotemporal dementia: a robust classification method of EEG signals and a comparison of validation methods. Diagnostics (Basel). 2021 Aug 9;11(8):1437.[Abstract][Full Text]
126. Turner MR, UK MND Clinical Studies Group. Diagnosing ALS: the gold coast criteria and the role of EMG. Pract Neurol. 2022 Jun;22(3):176-8.[Abstract][Full Text]
127. Cairns NJ, Bigio EH, Mackenzie IR, et al. Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration. Acta Neuropathol. 2007 Jul;114(1):5-22.[Abstract][Full Text]
128. McKhann GM, Albert MS, Grossman M, et al. Clinical and pathological diagnosis of frontotemporal dementia: report of the Work Group on Frontotemporal Dementia and Pick's Disease. Arch Neurol. 2001 Nov;58(11):1803-9.[Abstract]
129. Chaudhry A, Houlden H, Rizig M. Novel fluid biomarkers to differentiate frontotemporal dementia and dementia with Lewy bodies from Alzheimer's disease: a systematic review. J Neurol Sci. 2020 Aug 15;415:116886.[Abstract]
130. Casoli T, Paolini S, Fabbietti P, et al. Cerebrospinal fluid biomarkers and cognitive status in differential diagnosis of frontotemporal dementia and Alzheimer's disease. J Int Med Res. 2019 Oct;47(10):4968-80.[Abstract][Full Text]
131. Palmqvist S, Janelidze S, Quiroz YT, et al. Discriminative accuracy of plasma phospho-tau217 for Alzheimer disease vs other neurodegenerative disorders. JAMA. 2020 Aug 25;324(8):772-81.[Abstract][Full Text]
132. Thijssen EH, La Joie R, Wolf A, et al. Diagnostic value of plasma phosphorylated tau181 in alzheimer's disease and frontotemporal lobar degeneration. Nat Med. 2020 Mar;26(3):387-97.[Abstract][Full Text]
133. Borroni B, Benussi A, Archetti S, et al. Csf p-tau181/tau ratio as biomarker for TDP pathology in frontotemporal dementia. Amyotroph Lateral Scler Frontotemporal Degener. 2015 Mar;16(1-2):86-91.[Abstract]
134. Ikeda M, Brown J, Holland AJ, et al. Changes in appetite, food preference, and eating habits in frontotemporal dementia and Alzheimer's disease. J Neurol Neurosurg Psychiatry. 2002 Oct;73(4):371-6.[Abstract][Full Text]
135. Moheb N, Charuworn K, Ashla MM, et al. Repetitive behaviors in frontotemporal dementia: compulsions or impulsions? J Neuropsychiatry Clin Neurosci. 2019 Spring;31(2):132-6.[Abstract][Full Text]
136. Mendez MF, Lauterbach EC, Sampson SM, et al. An evidence-based review of the psychopathology of frontotemporal dementia: a report of the ANPA Committee on Research. J Neuropsychiatry Clin Neurosci. 2008 Spring;20(2):130-49.[Abstract]
137. Landqvist Waldö M, Frizell Santillo A, Passant U, et al. Cerebrospinal fluid neurofilament light chain protein levels in subtypes of frontotemporal dementia. BMC Neurol. 2013 May 29;13:54.[Abstract][Full Text]
138. Mosconi L, Tsui WH, Herholz K, et al. Multicenter standardized 18F-FDG PET diagnosis of mild cognitive impairment, Alzheimer's disease, and other dementias. J Nucl Med. 2008 Mar;49(3):390-8.[Abstract][Full Text]
139. McKeith I, O'Brien J, Walker Z, et al. Sensitivity and specificity of dopamine transporter imaging with 123I-FP-CIT SPECT in dementia with Lewy bodies: a phase III, multicentre study. Lancet Neurol. 2007 Apr;6(4):305-13.[Abstract]
140. World Health Organization. Clinical descriptions and diagnostic requirements for ICD-11 mental, behavioural and neurodevelopmental disorders (CDDR). Mar 2024 [internet publication].[Full Text]
141. Goldman JS, Farmer JM, Van Deerlin VM, et al. Frontotemporal dementia: genetics and genetic counseling dilemmas. Neurologist. 2004 Sep;10(5):227-34.[Abstract]
142. Meeter LH, Dopper EG, Jiskoot LC, et al. Neurofilament light chain: a biomarker for genetic frontotemporal dementia. Ann Clin Transl Neurol. 2016 Aug;3(8):623-36.[Abstract][Full Text]
143. Cajanus A, Katisko K, Kontkanen A, et al. Serum neurofilament light chain in FTLD: association with C9orf72, clinical phenotype, and prognosis. Ann Clin Transl Neurol. 2020 Jun;7(6):903-10.[Abstract][Full Text]
144. van der Ende EL, Meeter LH, Poos JM, et al. Serum neurofilament light chain in genetic frontotemporal dementia: a longitudinal, multicentre cohort study. Lancet Neurol. 2019 Dec;18(12):1103-11.[Abstract]
145. Wilke C, Reich S, van Swieten JC, et al. Stratifying the presymptomatic phase of genetic frontotemporal dementia by serum NfL and pNfH: a longitudinal multicentre study. Ann Neurol. 2022 Jan;91(1):33-47.[Abstract][Full Text]
146. American College of Radiology. ACR-ACNM-ASNR-SNMMI practice parameter for brain PET-CT imaging in dementia. 2020 [internet publication].[Full Text]
147. American Academy of Neurology. Diagnosis of dementia. May 2001 [internet publication].[Full Text]
148. Nguyen J, Ko I, Martinez-Sosa S, et al. Ward based management of behavioural and psychological symptoms of dementia. BMJ. 2021 Aug 4;374:n1779.[Abstract]
149. Bogaerts JMK, Gussekloo J, de Jong-Schmit BEM, et al. Effects of the discontinuation of antihypertensive treatment on neuropsychiatric symptoms and quality of life in nursing home residents with dementia (DANTON): a multicentre, open-label, blinded-outcome, randomised controlled trial. Age Ageing. 2024 Jul 2;53(7):afae133.[Abstract][Full Text]
150. Young JJ, Lavakumar M, Tampi D, et al. Frontotemporal dementia: latest evidence and clinical implications. Ther Adv Psychopharmacol. 2018 Jan;8(1):33-48.[Abstract][Full Text]
151. American Psychiatric Association. Guideline watch: practice guideline for the treatment of patients with Alzheimer's disease and other dementias. Oct 2014 [internet publication].[Full Text]
152. Reuben DB, Kremen S, Maust DT. Dementia prevention and treatment: a narrative review. JAMA Intern Med. 2024 May 1;184(5):563-72.[Abstract][Full Text]
153. Ayeno HD, Kassie GM, Atee M, et al. Factors influencing the implementation of non-pharmacological interventions for behavioural and psychological symptoms of dementia in residential aged-care homes: a systematic review and qualitative evidence synthesis: a systematic review. Campbell Syst Rev. 2025 Jun;21(2):e70029.[Abstract][Full Text]
154. Khanassov V, Vedel I. Family physician-case manager collaboration and needs of patients with dementia and their caregivers: a systematic mixed studies review. Ann Fam Med. 2016 Mar;14(2):166-77.[Abstract][Full Text]
155. Soril LJ, Leggett LE, Lorenzetti DL, et al. Effective use of the built environment to manage behavioural and psychological symptoms of dementia: a systematic review. PLoS One. 2014 Dec 17;9(12):e115425.[Abstract][Full Text]
156. Mulkey MA, Everhart DE, Hardin SR. Fronto-temporal dementia: a case study and strategies and support for caregivers. Br J Community Nurs. 2019 Nov 2;24(11):544-9.[Abstract]
157. O'Brien R, Beeke S, Pilnick A, et al. When people living with dementia say 'no': negotiating refusal in the acute hospital setting. Soc Sci Med. 2020 Oct;263:113188.[Abstract][Full Text]
158. Machiels M, Metzelthin SF, Hamers JP, et al. Interventions to improve communication between people with dementia and nursing staff during daily nursing care: a systematic review. Int J Nurs Stud. 2017 Jan;66:37-46.[Abstract]
159. Bott NT, Radke A, Stephens ML, et al. Frontotemporal dementia: diagnosis, deficits and management. Neurodegener Dis Manag. 2014;4(6):439-54.[Abstract]
160. Cooper C, Vickerstaff V, Barber J, et al. A psychosocial goal-setting and manualised support intervention for independence in dementia (NIDUS-Family) versus goal setting and routine care: a single-masked, phase 3, superiority, randomised controlled trial. Lancet Healthy Longev. 2024 Feb;5(2):e141-51.[Abstract][Full Text]
161. Abraha I, Rimland JM, Trotta FM, et al. Systematic review of systematic reviews of non-pharmacological interventions to treat behavioural disturbances in older patients with dementia. The SENATOR-OnTop series. BMJ Open. 2017 Mar 16;7(3):e012759.[Abstract][Full Text]
162. Neylan KD, Miller BL. New approaches to the treatment of frontotemporal dementia. Neurotherapeutics. 2023 Jul;20(4):1055-65.[Abstract][Full Text]
163. Brodaty H, Arasaratnam C. Meta-analysis of nonpharmacological interventions for neuropsychiatric symptoms of dementia. Am J Psychiatry. 2012 Sep;169(9):946-53.[Abstract][Full Text]
164. Dyer SM, Harrison SL, Laver K, et al. An overview of systematic reviews of pharmacological and non-pharmacological interventions for the treatment of behavioral and psychological symptoms of dementia. Int Psychogeriatr. 2018 Mar;30(3):295-309.[Abstract][Full Text]
165. Watt JA, Goodarzi Z, Veroniki AA, et al. Comparative efficacy of interventions for reducing symptoms of depression in people with dementia: systematic review and network meta-analysis. BMJ. 2021 Mar 24;372:n532.[Abstract][Full Text]
166. Lühnen J, Richter T, Calo S, et al. Psychosocial interventions for reducing antipsychotic medication in care home residents. Cochrane Database Syst Rev. 2023 Aug 31;8(8):CD008634.[Abstract][Full Text]
167. Carrion C, Folkvord F, Anastasiadou D, et al. Cognitive therapy for dementia patients: a systematic review. Dement Geriatr Cogn Disord. 2018;46(1-2):1-26.[Abstract][Full Text]
168. Kudlicka A, Martyr A, Bahar-Fuchs A, et al. Cognitive rehabilitation for people with mild to moderate dementia. Cochrane Database Syst Rev. 2023 Jun 29;6(6):CD013388.[Abstract][Full Text]
169. van der Steen JT, van der Wouden JC, Methley AM, et al. Music-based therapeutic interventions for people with dementia. Cochrane Database Syst Rev. 2025 Mar 7;3(3):CD003477.[Abstract]
170. Langhammer B, Sagbakken M, Kvaal K, et al. Music therapy and physical activity to ease anxiety, restlessness, irritability, and aggression in individuals with dementia with signs of frontotemporal lobe degeneration. J Psychosoc Nurs Ment Health Serv. 2019 May 1;57(5):29-37.[Abstract]
171. Bell G, Baou CE, Saunders R, et al. Effectiveness of primary care psychological therapy services for the treatment of depression and anxiety in people living with dementia: evidence from national healthcare records in England. EClinicalMedicine. 2022 Oct;52:101692.[Abstract][Full Text]
172. Orgeta V, Leung P, Del-Pino-Casado R, et al. Psychological treatments for depression and anxiety in dementia and mild cognitive impairment. Cochrane Database Syst Rev. 2022 Apr 25;4(4):CD009125.[Abstract][Full Text]
173. Watt JA, Goodarzi Z, Veroniki AA, et al. Comparative efficacy of interventions for aggressive and agitated behaviors in dementia: a systematic review and network meta-analysis. Ann Intern Med. 2019 Nov 5;171(9):633-42.[Abstract]
174. Leng M, Zhao Y, Wang Z. Comparative efficacy of non-pharmacological interventions on agitation in people with dementia: a systematic review and Bayesian network meta-analysis. Int J Nurs Stud. 2020 Feb;102:103489.[Abstract]
175. McCarter SJ, St Louis EK, Boeve BF. Sleep disturbances in frontotemporal dementia. Curr Neurol Neurosci Rep. 2016 Sep;16(9):85.[Abstract]
176. Wilfling D, Calo S, Dichter MN, et al. Non-pharmacological interventions for sleep disturbances in people with dementia. Cochrane Database Syst Rev. 2023 Jan 3;1(1):CD011881.[Abstract][Full Text]
177. Migliaccio R, Tanguy D, Bouzigues A, et al. Cognitive and behavioural inhibition deficits in neurodegenerative dementias. Cortex. 2020 Oct;131:265-83.[Abstract][Full Text]
178. Tsoi KKF, Chan JYC, Ng YM, et al. Receptive music therapy is more effective than interactive music therapy to relieve behavioral and psychological symptoms of dementia: a systematic review and meta-analysis. J Am Med Dir Assoc. 2018 Jul;19(7):568-76.e3.[Abstract]
179. Shinagawa S, Nakajima S, Plitman E, et al. Non-pharmacological management for patients with frontotemporal dementia: a systematic review. J Alzheimers Dis. 2015;45(1):283-93.[Abstract]
180. Buoli M, Serati M, Caldiroli A, et al. Pharmacological management of psychiatric symptoms in frontotemporal dementia: a systematic review. J Geriatr Psychiatry Neurol. 2017 May;30(3):162-9.[Abstract]
181. Trieu C, Gossink F, Stek ML, et al. Effectiveness of pharmacological interventions for symptoms of behavioral variant frontotemporal dementia: a systematic review. Cogn Behav Neurol. 2020 Mar;33(1):1-15.[Abstract]
182. Reus VI, Fochtmann LJ, Eyler AE, et al. The American Psychiatric Association practice guideline on the use of antipsychotics to treat agitation or psychosis in patients with dementia. Am J Psychiatry. 2016 May 1;173(5):543-6.[Abstract][Full Text]
183. Magierski R, Sobow T, Schwertner E, et al. Pharmacotherapy of behavioral and psychological symptoms of dementia: state of the art and future progress. Front Pharmacol. 2020 Jul 31;11:1168.[Abstract][Full Text]
184. Ralph SJ, Espinet AJ. Increased all-cause mortality by antipsychotic drugs: updated review and meta-analysis in dementia and general mental health care. J Alzheimers Dis Rep. 2018 Feb 2;2(1):1-26.[Abstract][Full Text]
185. Mok PLH, Carr MJ, Guthrie B, et al. Multiple adverse outcomes associated with antipsychotic use in people with dementia: population based matched cohort study. BMJ. 2024 Apr 17;385:e076268.[Abstract][Full Text]
186. Wang GH, Man KKC, Chang WH, et al. Use of antipsychotic drugs and cholinesterase inhibitors and risk of falls and fractures: self-controlled case series. BMJ. 2021 Sep 9;374:n1925.[Abstract][Full Text]
187. Yunusa I, Alsumali A, Garba AE, et al. Assessment of reported comparative effectiveness and safety of atypical antipsychotics in the treatment of behavioral and psychological symptoms of dementia: a network meta-analysis. JAMA Netw Open. 2019 Mar 1;2(3):e190828.[Abstract][Full Text]
188. Huang YY, Teng T, Giovane CD, et al. Pharmacological treatment of neuropsychiatric symptoms of dementia: a network meta-analysis. Age Ageing. 2023 Jun 1;52(6):afad091.[Abstract][Full Text]
189. Le C, Finger E. Pharmacotherapy for neuropsychiatric symptoms in frontotemporal dementia. CNS Drugs. 2021 Oct;35(10):1081-96.[Abstract]
190. McCleery J, Sharpley AL. Pharmacotherapies for sleep disturbances in dementia. Cochrane Database Syst Rev. 2020 Nov 15;(11):CD009178.[Abstract][Full Text]
191. Richardson K, Savva GM, Boyd PJ, et al. Non-benzodiazepine hypnotic use for sleep disturbance in people aged over 55 years living with dementia: a series of cohort studies. Health Technol Assess. 2021 Jan;25(1):1-202.[Abstract][Full Text]
192. Herzig SJ, Rothberg MB, Moss CR, et al. Risk of in-hospital falls among medications commonly used for insomnia in hospitalized patients. Sleep. 2021 Sep 13;44(9):zsab064.[Abstract][Full Text]
193. Drayton SJ, Davies K, Steinberg M, et al. Amantadine for executive dysfunction syndrome in patients with dementia. Psychosomatics. 2004 May-Jun;45(3):205-9.[Abstract]
194. Boxer AL, Knopman DS, Kaufer DI, et al. Memantine in patients with frontotemporal lobar degeneration: a multicentre, randomised, double-blind, placebo-controlled trial. Lancet Neurol. 2013 Feb;12(2):149-56.[Abstract][Full Text]
195. McShane R, Westby MJ, Roberts E, et al. Memantine for dementia. Cochrane Database Syst Rev. 2019 Mar 20;3(3):CD003154.[Abstract][Full Text]
196. Galvez-Andres A, Blasco-Fontecilla H, Gonzalez-Parra S, et al. Secondary bipolar disorder and Diogenes syndrome in frontotemporal dementia: behavioral improvement with quetiapine and sodium valproate. J Clin Psychopharmacol. 2007 Dec;27(6):722-3.[Abstract]
197. Chow TW, Mendez MF. Goals in symptomatic pharmacologic management of frontotemporal lobar degeneration. Am J Alzheimers Dis Other Demen. 2002 Sep-Oct;17(5):267-72.[Abstract][Full Text]
198. Tsai RM, Boxer AL. Treatment of frontotemporal dementia. Curr Treat Options Neurol. 2014 Nov;16(11):319.[Abstract][Full Text]
199. Hakimi M, Maurer CW. Pseudobulbar affect in parkinsonian disorders: a review. J Mov Disord. 2019 Jan;12(1):14-21.[Abstract][Full Text]
200. El-Sohl AA, Niederman MS, Drinka P. Nursing home-acquired pneumonia: a review of risk factors and therapeutic approaches. Curr Med Res Opin. 2010 Dec;26(12):2707-14.[Abstract]
201. Taylor LP, Besbris JM, Graf WD, et al. Clinical guidance in neuropalliative care: an AAN position statement. Neurology. 2022 Mar 8;98(10):409-16.[Abstract][Full Text]
202. Chiong W, Tsou AY, Simmons Z, et al. Ethical considerations in dementia diagnosis and care: AAN position statement. Neurology. 2021 Jul 13;97(2):80-9.[Abstract][Full Text]
203. Walsh SC, Murphy E, Devane D, et al. Palliative care interventions in advanced dementia. Cochrane Database Syst Rev. 2021 Sep 28;9(9):CD011513.[Abstract][Full Text]
204. Davies N, Barrado-Martín Y, Vickerstaff V, et al. Enteral tube feeding for people with severe dementia. Cochrane Database Syst Rev. 2021 Aug 13;(8):CD013503.[Abstract][Full Text]
205. Stall NM, Quinn KL, van der Steen JT, et al. Enteral tube feeding in people with advanced dementia. BMJ. 2025 May 15;389:e075326.[Abstract]
206. Boxer AL, Gold M, Huey E, et al. Frontotemporal degeneration, the next therapeutic frontier: molecules and animal models for frontotemporal drug development. Alzheimers Dement. 2013 Mar;9(2):176-88.[Abstract][Full Text]
207. Seripa D, Solfrizzi V, Imbimbo BP, et al. Tau-directed approaches for the treatment of Alzheimer's disease: focus on leuco-methylthioninium. Expert Rev Neurother. 2016;16(3):259-77.[Abstract]
208. Min SW, Chen X, Tracy TE, et al. Critical role of acetylation in tau-mediated neurodegeneration and cognitive deficits. Nat Med. 2015 Oct;21(10):1154-62.[Abstract][Full Text]
209. Liu W, Zhao L, Blackman B, et al. Vectored intracerebral immunization with the anti-tau monoclonal antibody PHF1 markedly reduces tau pathology in mutant tau transgenic mice. J Neurosci. 2016 Dec 7;36(49):12425-35.[Abstract][Full Text]
210. Gozes I, Ivashko-Pachima Y. ADNP: in search for molecular mechanisms and innovative therapeutic strategies for frontotemporal degeneration. Front Aging Neurosci. 2015 Oct 29;7:205.[Abstract][Full Text]
211. Robbins M. Therapies for Tau-associated neurodegenerative disorders: targeting molecules, synapses, and cells. Neural Regen Res. 2023 Dec;18(12):2633-7.[Abstract][Full Text]
212. Alberici A, Archetti S, Pilotto A, et al. Results from a pilot study on amiodarone administration in monogenic frontotemporal dementia with granulin mutation. Neurol Sci. 2014 Aug;35(8):1215-9.[Abstract]
213. Lee YB, Miranda CJ, Allison J, et al. Pre‐clinical development of AVB‐101, an AAV gene therapy treatment for frontotemporal dementia with progranulin mutations. Alzheimers Dement. 2025 Jan 3;20(Suppl 1):e086546.[Full Text]
214. Lagier-Tourenne C, Baughn M, Rigo F, et al. Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration. Proc Natl Acad Sci U S A. 2013 Nov 19;110(47):E4530-9.[Abstract][Full Text]
215. Prins ND, de Haan W, Gardner A, et al. Phase 2A learnings incorporated into RewinD-LB, a phase 2B clinical trial of Neflamapimod in dementia with lewy bodies. J Prev Alzheimers Dis. 2024;11(3):549-57.[Abstract][Full Text]
216. Supasitthumrong T, Bolea-Alamanac BM, Asmer S, et al. Gabapentin and pregabalin to treat aggressivity in dementia: a systematic review and illustrative case report. Br J Clin Pharmacol. 2019 Apr;85(4):690-703.[Abstract][Full Text]
217. Callegari I, Mattei C, Benassi F, et al. Agomelatine improves apathy in frontotemporal dementia. Neurodegener Dis. 2016;16(5-6):352-6.[Abstract]
218. Coleman KKL, Berry S, Cummings J, et al. Intranasal oxytocin for apathy in people with frontotemporal dementia (FOXY): a multicentre, randomised, double-blind, placebo-controlled, adaptive, crossover, phase 2a/2b superiority trial. Lancet Neurol. 2025 Feb;24(2):128-39.[Abstract]
219. Lebert F, Stekke W, Hasenbroekx C, et al. Frontotemporal dementia: a randomised, controlled trial with trazodone. Dement Geriatr Cogn Disord. 2004;17(4):355-9.[Abstract]
220. Nisar M, Abubaker ZJ, Nizam MA, et al. Behavioral and cognitive response to selective serotonin reuptake inhibitors in frontotemporal lobar degeneration: a systematic review and meta-analysis. Clin Neuropharmacol. 2021 Sep-Oct 01;44(5):175-83.[Abstract]
221. Boxer AL, Boeve BF. Frontotemporal dementia treatment: current symptomatic therapies and implications of recent genetic, biochemical, and neuroimaging studies. Alzheimer Dis Assoc Disord. 2007 Oct-Dec;21(4):S79-87.[Abstract]
222. Ferrucci R, Mrakic-Sposta S, Gardini S, et al. Behavioral and neurophysiological effects of transcranial direct current stimulation (tDCS) in fronto-temporal dementia. Front Behav Neurosci. 2018;12:235.[Abstract][Full Text]
223. Iverson DJ, Gronseth GS, Reger MA, et al. Practice parameter update: evaluation and management of driving risk in dementia: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2010 Apr 20;74(16):1316-24.[Abstract][Full Text]
224. Thurman DJ, Stevens JA, Rao JK, et al. Practice parameter: assessing patients in a neurology practice for risk of falls (an evidence-based review): report of the quality standards subcommittee of the American Academy of Neurology. Neurology. 2008 Feb 5;70(6):473-9.[Abstract][Full Text]
225. American Psychiatric Association. Guideline watch (October 2014): practice guideline for the treatment of patients with Alzheimer's disease and other dementias. Oct 2014 [internet publication].[Full Text]
226. Anderl-Straub S, Lausser L, Lombardi J, et al. Predicting disease progression in behavioral variant frontotemporal dementia. Alzheimers Dement (Amst). 2021;13(1):e12262.[Abstract][Full Text]
227. Day S, Roberts S, Launder NH, et al. Age of symptom onset and longitudinal course of sporadic alzheimer's disease, frontotemporal dementia, and vascular dementia: a systematic review and meta-analysis. J Alzheimers Dis. 2022;85(4):1819-33.[Abstract]
228. Malpetti M, Cope TE, Street D, et al. Microglial activation in the frontal cortex predicts cognitive decline in frontotemporal dementia. Brain. 2023 Aug 1;146(8):3221-31.[Abstract][Full Text]
229. Xie SX, Forman MS, Farmer J, et al. Factors associated with survival probability in autopsy-proven frontotemporal lobar degeneration. J Neurol Neurosurg Psychiatry. 2008 Feb;79(2):126-9.[Abstract]
230. Pasquier F, Richard F, Lebert F. Natural history of frontotemporal dementia: comparison with Alzheimer's disease. Dement Geriatr Cogn Disord. 2004;17(4):253-7.[Abstract]
231. Borroni B, Grassi M, Agosti C, et al. Survival in frontotemporal lobar degeneration and related disorders: latent class predictors and brain functional correlates. Rejuvenation Res. 2009 Feb;12(1):33-44.[Abstract]
232. Kang SJ, Cha KR, Seo SW, et al. Survival in frontotemporal lobar degeneration in a Korean population. Alzheimer Dis Assoc Disord. 2010 Oct-Dec;24(4):339-42.[Abstract]
233. Kansal K, Mareddy M, Sloane KL, et al. Survival in frontotemporal dementia phenotypes: a meta-analysis. Dement Geriatr Cogn Disord. 2016;41(1-2):109-22.[Abstract]
234. Nunnemann S, Last D, Schuster T, et al. Survival in a German population with frontotemporal lobar degeneration. Neuroepidemiology. 2011;37(3-4):160-5.[Abstract]
235. Rascovsky K, Salmon DP, Lipton AM, et al. Rate of progression differs in frontotemporal dementia and Alzheimer disease. Neurology. 2005 Aug 9;65(3):397-403.[Abstract]
236. Glasmacher SA, Wong C, Pearson IE, et al. Survival and prognostic factors in C9orf72 repeat expansion carriers: a systematic review and meta-analysis. JAMA Neurol. 2020 Mar 1;77(3):367-76.[Abstract][Full Text]
237. Reus LM, Willemse SW, de Boer SCM, et al. UNC13A polymorphism influences survival in patients with frontotemporal dementia. Ann Neurol. 2025 Jun;97(6):1062-6.[Abstract][Full Text]
238. Jung NY, Park KH, Seo SW, et al. Survival in Korean patients with frontotemporal dementia syndrome: association with behavioral features and parkinsonism. J Clin Med. 2022 Apr 18;11(8):2260.[Abstract][Full Text]
239. Arena JE, Weigand SD, Whitwell JL, et al. Progressive supranuclear palsy: progression and survival. J Neurol. 2016 Feb;263(2):380-9.[Abstract]
240. Nath U, Ben-Shlomo Y, Thomson RG, et al. Clinical features and natural history of progressive supranuclear palsy: a clinical cohort study. Neurology. 2003 Mar 25;60(6):910-6.[Abstract]
241. Ahmed RM, Iodice V, Daveson N, et al. Autonomic dysregulation in frontotemporal dementia. J Neurol Neurosurg Psychiatry. 2015 Sep;86(9):1048-9.[Abstract]
242. Fletcher PD, Downey LE, Golden HL, et al. Pain and temperature processing in dementia: a clinical and neuroanatomical analysis. Brain. 2015 Nov;138(pt 11):3360-72.[Abstract][Full Text]
243. Manietta C, Labonté V, Thiesemann R, et al. Algorithm-based pain management for people with dementia in nursing homes. Cochrane Database Syst Rev. 2022 Apr 1;4(4):CD013339.[Abstract][Full Text]
244. Riedijk SR, de Vugt ME, Duivenvoorden HJ, et al. Caregiver burden, health-related quality of life and coping in dementia caregivers: a comparison of frontotemporal dementia and Alzheimer's disease. Dement Geriatr Cogn Disord. 2006;22(5-6):405-12.[Abstract]
245. Toepper M, Falkenstein M. Driving fitness in different forms of dementia: an update. J Am Geriatr Soc. 2019 Oct;67(10):2186-92.[Abstract]
246. Alothman D, Card T, Lewis S, et al. Risk of suicide after dementia diagnosis. JAMA Neurol. 2022 Oct 3;79(11):1148-54.[Abstract][Full Text]
