Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Frontotemporal dementia: Treatment

Suzee E Lee, MD
Bruce L Miller, MD
Section Editor
Steven T DeKosky, MD, FAAN, FACP, FANA
Deputy Editor
April F Eichler, MD, MPH


Frontotemporal dementia (FTD) is a neuropathologically and clinically heterogeneous disorder characterized by focal degeneration of the frontal and/or temporal lobes [1]. Age of onset is typically in the late 50s or early 60s. The primary initial clinical manifestations include changes in personality and social behavior or language, progressing over time to a more global dementia affecting cognitive domains as well. A subset of patients may also exhibit symptoms of extrapyramidal or motor neuron involvement at some point in the disease process.

There are no currently FDA-approved disease modifying treatments for the treatment of frontotemporal dementia (FTD). Since the underlying pathology of FTD most often consists of abnormal tau or TDP-43 protein aggregates, treatments geared toward beta-amyloid reduction that appear promising in Alzheimer disease (AD) are not expected to be effective for FTD.

Symptomatic relief can be provided by a variety of agents; these medications should be considered in conjunction with non-pharmacologic interventions, such as thoughtful evaluation of the patient's living environment.

This topic discusses treatment issues specific to patients with FTD. Epidemiology, pathology, clinical features and diagnosis of FTD, and the management of other forms of dementia are discussed separately. (See "Frontotemporal dementia: Epidemiology, pathology, and pathogenesis" and "Frontotemporal dementia: Clinical features and diagnosis" and "Treatment of dementia" and "Treatment and prevention of vascular dementia" and "Prognosis and treatment of dementia with Lewy bodies" and "Management of neuropsychiatric symptoms of dementia".)


Pharmacologic treatment in FTD is symptomatic and aimed at alleviating neurobehavioral symptoms. However, despite the fact that they are commonly used, there is limited evidence of the efficacy of pharmacologic treatments in FTD [2].

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Sep 21, 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Clinical and neuropathological criteria for frontotemporal dementia. The Lund and Manchester Groups. J Neurol Neurosurg Psychiatry 1994; 57:416.
  2. Bei Hu, Ross L, Neuhaus J, et al. Off-label medication use in frontotemporal dementia. Am J Alzheimers Dis Other Demen 2010; 25:128.
  3. Huey ED, Putnam KT, Grafman J. A systematic review of neurotransmitter deficits and treatments in frontotemporal dementia. Neurology 2006; 66:17.
  4. Francis PT, Holmes C, Webster MT, et al. Preliminary neurochemical findings in non-Alzheimer dementia due to lobar atrophy. Dementia 1993; 4:172.
  5. Swartz JR, Miller BL, Lesser IM, Darby AL. Frontotemporal dementia: treatment response to serotonin selective reuptake inhibitors. J Clin Psychiatry 1997; 58:212.
  6. Lanctôt KL, Herrmann N, Ganjavi H, et al. Serotonin-1A receptors in frontotemporal dementia compared with controls. Psychiatry Res 2007; 156:247.
  7. Procter AW, Qurne M, Francis PT. Neurochemical features of frontotemporal dementia. Dement Geriatr Cogn Disord 1999; 10 Suppl 1:80.
  8. Perry RJ, Miller BL. Behavior and treatment in frontotemporal dementia. Neurology 2001; 56:S46.
  9. Sparks DL, Markesbery WR. Altered serotonergic and cholinergic synaptic markers in Pick's disease. Arch Neurol 1991; 48:796.
  10. Rinne JO, Laine M, Kaasinen V, et al. Striatal dopamine transporter and extrapyramidal symptoms in frontotemporal dementia. Neurology 2002; 58:1489.
  11. Frisoni GB, Pizzolato G, Bianchetti A, et al. Single photon emission computed tomography with [99Tc]-HM-PAO and [123I]-IBZM in Alzheimer's disease and dementia of frontal type: preliminary results. Acta Neurol Scand 1994; 89:199.
  12. Engelborghs S, Vloeberghs E, Le Bastard N, et al. The dopaminergic neurotransmitter system is associated with aggression and agitation in frontotemporal dementia. Neurochem Int 2008; 52:1052.
  13. Mendez MF. Frontotemporal dementia: therapeutic interventions. Front Neurol Neurosci 2009; 24:168.
  14. Chow TW, Mendez MF. Goals in symptomatic pharmacologic management of frontotemporal lobar degeneration. Am J Alzheimers Dis Other Demen 2002; 17:267.
  15. Moretti R, Torre P, Antonello RM, et al. Frontotemporal dementia: paroxetine as a possible treatment of behavior symptoms. A randomized, controlled, open 14-month study. Eur Neurol 2003; 49:13.
  16. Mendez MF, Shapira JS, Miller BL. Stereotypical movements and frontotemporal dementia. Mov Disord 2005; 20:742.
  17. Ikeda M, Shigenobu K, Fukuhara R, et al. Efficacy of fluvoxamine as a treatment for behavioral symptoms in frontotemporal lobar degeneration patients. Dement Geriatr Cogn Disord 2004; 17:117.
  18. Manes FF, Torralva T, Roca M, et al. Frontotemporal dementia presenting as pathological gambling. Nat Rev Neurol 2010; 6:347.
  19. Anderson IM, Scott K, Harborne G. Serotonin and depression in frontal lobe dementia. Am J Psychiatry 1995; 152:645.
  20. Anneser JM, Jox RJ, Borasio GD. Inappropriate sexual behaviour in a case of ALS and FTD: successful treatment with sertraline. Amyotroph Lateral Scler 2007; 8:189.
  21. Mendez MF. Pathological stealing in dementia: poor response to SSRI medications. J Clin Psychiatry 2011; 72:418.
  22. Deakin JB, Rahman S, Nestor PJ, et al. Paroxetine does not improve symptoms and impairs cognition in frontotemporal dementia: a double-blind randomized controlled trial. Psychopharmacology (Berl) 2004; 172:400.
  23. Lebert F, Stekke W, Hasenbroekx C, Pasquier F. Frontotemporal dementia: a randomised, controlled trial with trazodone. Dement Geriatr Cogn Disord 2004; 17:355.
  24. Moretti R, Torre P, Antonello RM, et al. Olanzapine as a treatment of neuropsychiatric disorders of Alzheimer's disease and other dementias: a 24-month follow-up of 68 patients. Am J Alzheimers Dis Other Demen 2003; 18:205.
  25. Fellgiebel A, Müller MJ, Hiemke C, et al. Clinical improvement in a case of frontotemporal dementia under aripiprazole treatment corresponds to partial recovery of disturbed frontal glucose metabolism. World J Biol Psychiatry 2007; 8:123.
  26. Kerssens CJ, Pijnenburg YA. Vulnerability to neuroleptic side effects in frontotemporal dementia. Eur J Neurol 2008; 15:111.
  27. Pijnenburg YA, Sampson EL, Harvey RJ, et al. Vulnerability to neuroleptic side effects in frontotemporal lobar degeneration. Int J Geriatr Psychiatry 2003; 18:67.
  28. Czarnecki K, Kumar N, Josephs KA. Parkinsonism and tardive antecollis in frontotemporal dementia--increased sensitivity to newer antipsychotics? Eur J Neurol 2008; 15:199.
  29. Komossa K, Rummel-Kluge C, Schmid F, et al. Quetiapine versus other atypical antipsychotics for schizophrenia. Cochrane Database Syst Rev 2010; :CD006625.
  30. Goforth HW, Konopka L, Primeau M, et al. Quantitative electroencephalography in frontotemporal dementia with methylphenidate response: a case study. Clin EEG Neurosci 2004; 35:108.
  31. Huey ED, Garcia C, Wassermann EM, et al. Stimulant treatment of frontotemporal dementia in 8 patients. J Clin Psychiatry 2008; 69:1981.
  32. Rahman S, Robbins TW, Hodges JR, et al. Methylphenidate ('Ritalin') can ameliorate abnormal risk-taking behavior in the frontal variant of frontotemporal dementia. Neuropsychopharmacology 2006; 31:651.
  33. Kertesz A, Morlog D, Light M, et al. Galantamine in frontotemporal dementia and primary progressive aphasia. Dement Geriatr Cogn Disord 2008; 25:178.
  34. Mendez MF, Shapira JS, McMurtray A, Licht E. Preliminary findings: behavioral worsening on donepezil in patients with frontotemporal dementia. Am J Geriatr Psychiatry 2007; 15:84.
  35. Moretti R, Torre P, Antonello RM, et al. Rivastigmine in frontotemporal dementia: an open-label study. Drugs Aging 2004; 21:931.
  36. Vossel KA, Miller BL. New approaches to the treatment of frontotemporal lobar degeneration. Curr Opin Neurol 2008; 21:708.
  37. Swanberg MM. Memantine for behavioral disturbances in frontotemporal dementia: a case series. Alzheimer Dis Assoc Disord 2007; 21:164.
  38. Diehl-Schmid J, Förstl H, Perneczky R, et al. A 6-month, open-label study of memantine in patients with frontotemporal dementia. Int J Geriatr Psychiatry 2008; 23:754.
  39. Chow TW, Graff-Guerrero A, Verhoeff NP, et al. Open-label study of the short-term effects of memantine on FDG-PET in frontotemporal dementia. Neuropsychiatr Dis Treat 2011; 7:415.
  40. Boxer AL, Lipton AM, Womack K, et al. An open-label study of memantine treatment in 3 subtypes of frontotemporal lobar degeneration. Alzheimer Dis Assoc Disord 2009; 23:211.
  41. Vercelletto M, Boutoleau-Bretonnière C, Volteau C, et al. Memantine in behavioral variant frontotemporal dementia: negative results. J Alzheimers Dis 2011; 23:749.
  42. 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; 12:149.
  43. Cruz M, Marinho V, Fontenelle LF, et al. Topiramate may modulate alcohol abuse but not other compulsive behaviors in frontotemporal dementia: case report. Cogn Behav Neurol 2008; 21:104.
  44. Gálvez-Andres A, Blasco-Fontecilla H, González-Parra S, et al. Secondary bipolar disorder and Diogenes syndrome in frontotemporal dementia: behavioral improvement with quetiapine and sodium valproate. J Clin Psychopharmacol 2007; 27:722.
  45. Moretti R, Torre P, Antonello RM, et al. Effects of selegiline on fronto-temporal dementia: a neuropsychological evaluation. Int J Geriatr Psychiatry 2002; 17:391.
  46. Adler G, Teufel M, Drach LM. Pharmacological treatment of frontotemporal dementia: treatment response to the MAO-A inhibitor moclobemide. Int J Geriatr Psychiatry 2003; 18:653.
  47. Litvan I, Grimes DA, Lang AE, et al. Clinical features differentiating patients with postmortem confirmed progressive supranuclear palsy and corticobasal degeneration. J Neurol 1999; 246 Suppl 2:II1.
  48. van Balken I, Litvan I. Current and future treatments in progressive supranuclear palsy. Curr Treat Options Neurol 2006; 8:211.
  49. Merrilees JJ, Miller BL. Long-term care of patients with frontotemporal dementia. J Am Med Dir Assoc 2003; 4:S162.
  50. Piguet O, Hornberger M, Mioshi E, Hodges JR. Behavioural-variant frontotemporal dementia: diagnosis, clinical staging, and management. Lancet Neurol 2011; 10:162.
  51. 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; 74:1316.
  52. Robinson KM. Rehabilitation applications in caring for patients with Pick's disease and frontotemporal dementias. Neurology 2001; 56:S56.
  53. Talerico KA, Evans LK. Responding to safety issues in frontotemporal dementias. Neurology 2001; 56:S52.
  54. Cenik B, Sephton CF, Dewey CM, et al. Suberoylanilide hydroxamic acid (vorinostat) up-regulates progranulin transcription: rational therapeutic approach to frontotemporal dementia. J Biol Chem 2011; 286:16101.
  55. Roberson ED, Hesse JH, Rose KD, et al. Frontotemporal dementia progresses to death faster than Alzheimer disease. Neurology 2005; 65:719.
  56. Binetti G, Locascio JJ, Corkin S, et al. Differences between Pick disease and Alzheimer disease in clinical appearance and rate of cognitive decline. Arch Neurol 2000; 57:225.
  57. Rascovsky K, Salmon DP, Lipton AM, et al. Rate of progression differs in frontotemporal dementia and Alzheimer disease. Neurology 2005; 65:397.
  58. 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; 126:2016.
  59. Hodges JR, Davies R, Xuereb J, et al. Survival in frontotemporal dementia. Neurology 2003; 61:349.
  60. Knibb JA, Xuereb JH, Patterson K, Hodges JR. Clinical and pathological characterization of progressive aphasia. Ann Neurol 2006; 59:156.
  61. Le Ber I, Guedj E, Gabelle A, et al. Demographic, neurological and behavioural characteristics and brain perfusion SPECT in frontal variant of frontotemporal dementia. Brain 2006; 129:3051.
  62. Neary D, Snowden J, Mann D. Frontotemporal dementia. Lancet Neurol 2005; 4:771.
  63. Garcin B, Lillo P, Hornberger M, et al. Determinants of survival in behavioral variant frontotemporal dementia. Neurology 2009; 73:1656.
  64. Coyle-Gilchrist IT, Dick KM, Patterson K, et al. Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes. Neurology 2016; 86:1736.