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Behavioral variant of frontotemporal dementia

MedGen UID:
860225
•Concept ID:
C4011788
•
Disease or Syndrome
Synonym: Frontotemporal dementia, behavioral variant
SNOMED CT: Behavioral variant of frontotemporal dementia (716994006)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
•Concept ID:
C0443147
•
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
Autosomal dominant inheritance (Orphanet)
 
Monarch Initiative: MONDO:0017160
Orphanet: ORPHA275864

Definition

A form of frontotemporal dementia characterized by progressive behavioral impairment and a decline in executive function with frontal lobe-predominant atrophy. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBehavioral variant of frontotemporal dementia

Professional guidelines

PubMed

Brain Sagging Dementia-Diagnosis, Treatment, and Outcome: A Review.
Lashkarivand A, Eide PK
Neurology 2022 May 10;98(19):798-805. Epub 2022 Mar 25 doi: 10.1212/WNL.0000000000200511. PMID: 35338080
Tau-Centric Targets and Drugs in Clinical Development for the Treatment of Alzheimer's Disease.
Panza F, Solfrizzi V, Seripa D, Imbimbo BP, Lozupone M, Santamato A, Zecca C, Barulli MR, Bellomo A, Pilotto A, Daniele A, Greco A, Logroscino G
Biomed Res Int 2016;2016:3245935. Epub 2016 Jun 26 doi: 10.1155/2016/3245935. PMID: 27429978Free PMC Article
Diagnosis and management of behavioral variant frontotemporal dementia.
Pressman PS, Miller BL
Biol Psychiatry 2014 Apr 1;75(7):574-81. Epub 2013 Nov 13 doi: 10.1016/j.biopsych.2013.11.006. PMID: 24315411Free PMC Article

Recent clinical studies

Etiology

Incidence of Syndromes Associated With Frontotemporal Lobar Degeneration in 9 European Countries.
Logroscino G, Piccininni M, Graff C, Hardiman O, Ludolph AC, Moreno F, Otto M, Remes AM, Rowe JB, Seelaar H, Solje E, Stefanova E, Traykov L, Jelic V, Rydell MT, Pender N, Anderl-Straub S, Barandiaran M, Gabilondo A, Krüger J, Murley AG, Rittman T, van der Ende EL, van Swieten JC, Hartikainen P, Stojmenovic GM, Mehrabian S, Benussi L, Alberici A, Dell'Abate MT, Zecca C, Borroni B; FRONTIERS group
JAMA Neurol 2023 Mar 1;80(3):279-286. doi: 10.1001/jamaneurol.2022.5128. PMID: 36716024Free PMC Article
In vivo exploration of synaptic projections in frontotemporal dementia.
Salmon E, Bahri MA, Plenevaux A, Becker G, Seret A, Delhaye E, Degueldre C, Balteau E, Lemaire C, Luxen A, Bastin C
Sci Rep 2021 Aug 9;11(1):16092. doi: 10.1038/s41598-021-95499-1. PMID: 34373529Free PMC Article
Recognition of musical emotions in the behavioral variant of frontotemporal dementia.
Orjuela-Rojas JM, Montañés P, Rodríguez ILL, González-Marín NR
Rev Colomb Psiquiatr (Engl Ed) 2021 Apr-Jun;50(2):74-81. doi: 10.1016/j.rcpeng.2020.01.003. PMID: 34099256
Empathy and contextual social cognition.
Melloni M, Lopez V, Ibanez A
Cogn Affect Behav Neurosci 2014 Mar;14(1):407-25. doi: 10.3758/s13415-013-0205-3. PMID: 23955101
Frontotemporal lobar degeneration: diversity of FTLD lesions.
Seilhean D, Bielle F, Plu I, Duyckaerts C
Rev Neurol (Paris) 2013 Oct;169(10):786-92. Epub 2013 Sep 12 doi: 10.1016/j.neurol.2013.07.015. PMID: 24035575

Diagnosis

Behavioral Variant of Frontotemporal Dementia and Homicide in a Historical Case.
Zago S, Scarpazza C, Difonzo T, Arighi A, Hajhajate D, Torrente Y, Sartori G
J Am Acad Psychiatry Law 2021 Jun;49(2):219-227. Epub 2021 Mar 17 doi: 10.29158/JAAPL.200081-20. PMID: 33731422
Differential Diagnosis of Behavioral Variant and Semantic Variant of Frontotemporal Dementia Using Visual Rating Scales.
Manouvelou S, Koutoulidis V, Tsougos I, Tolia M, Kyrgias G, Anyfantakis G, Moulopoulos LA, Gouliamos A, Papageorgiou S
Curr Med Imaging 2020;16(4):444-451. doi: 10.2174/1573405615666190225154834. PMID: 32410545
Behavioral and neural impairments of frontotemporal dementia: Potential implications for criminal responsibility and sentencing.
Berryessa CM
Int J Law Psychiatry 2016 May-Jun;46:1-6. Epub 2016 Mar 31 doi: 10.1016/j.ijlp.2016.02.020. PMID: 27039661
The neuropsychiatric examination of the young-onset dementias.
Ducharme S, Dickerson BC
Psychiatr Clin North Am 2015 Jun;38(2):249-64. Epub 2015 Mar 12 doi: 10.1016/j.psc.2015.01.002. PMID: 25998114
The behavioral variant of frontotemporal dementia: linking neuropathology to social cognition.
Cerami C, Cappa SF
Neurol Sci 2013 Aug;34(8):1267-74. Epub 2013 Feb 3 doi: 10.1007/s10072-013-1317-9. PMID: 23377232

Therapy

Incidence of Syndromes Associated With Frontotemporal Lobar Degeneration in 9 European Countries.
Logroscino G, Piccininni M, Graff C, Hardiman O, Ludolph AC, Moreno F, Otto M, Remes AM, Rowe JB, Seelaar H, Solje E, Stefanova E, Traykov L, Jelic V, Rydell MT, Pender N, Anderl-Straub S, Barandiaran M, Gabilondo A, Krüger J, Murley AG, Rittman T, van der Ende EL, van Swieten JC, Hartikainen P, Stojmenovic GM, Mehrabian S, Benussi L, Alberici A, Dell'Abate MT, Zecca C, Borroni B; FRONTIERS group
JAMA Neurol 2023 Mar 1;80(3):279-286. doi: 10.1001/jamaneurol.2022.5128. PMID: 36716024Free PMC Article
Recognition of musical emotions in the behavioral variant of frontotemporal dementia.
Orjuela-Rojas JM, Montañés P, Rodríguez ILL, González-Marín NR
Rev Colomb Psiquiatr (Engl Ed) 2021 Apr-Jun;50(2):74-81. doi: 10.1016/j.rcpeng.2020.01.003. PMID: 34099256
Clinical and MRI Predictors of Conversion From Mild Behavioural Impairment to Dementia.
Orso B, Mattei C, Arnaldi D, Massa F, Serafini G, Plantone D, Doglione E, Grafman J, Nobili F, Pardini M
Am J Geriatr Psychiatry 2020 Jul;28(7):755-763. Epub 2019 Dec 17 doi: 10.1016/j.jagp.2019.12.007. PMID: 31928846
Tau-directed approaches for the treatment of Alzheimer's disease: focus on leuco-methylthioninium.
Seripa D, Solfrizzi V, Imbimbo BP, Daniele A, Santamato A, Lozupone M, Zuliani G, Greco A, Logroscino G, Panza F
Expert Rev Neurother 2016;16(3):259-77. doi: 10.1586/14737175.2016.1140039. PMID: 26822031
Prospective evaluation of behavioral scales in the behavioral variant of frontotemporal dementia.
Boutoleau-Bretonnière C, Lebouvier T, Volteau C, Jaulin P, Lacomblez L, Damier P, Thomas-Anterion C, Vercelletto M
Dement Geriatr Cogn Disord 2012;34(2):75-82. Epub 2012 Aug 23 doi: 10.1159/000341784. PMID: 22922703

Prognosis

Incidence of Syndromes Associated With Frontotemporal Lobar Degeneration in 9 European Countries.
Logroscino G, Piccininni M, Graff C, Hardiman O, Ludolph AC, Moreno F, Otto M, Remes AM, Rowe JB, Seelaar H, Solje E, Stefanova E, Traykov L, Jelic V, Rydell MT, Pender N, Anderl-Straub S, Barandiaran M, Gabilondo A, Krüger J, Murley AG, Rittman T, van der Ende EL, van Swieten JC, Hartikainen P, Stojmenovic GM, Mehrabian S, Benussi L, Alberici A, Dell'Abate MT, Zecca C, Borroni B; FRONTIERS group
JAMA Neurol 2023 Mar 1;80(3):279-286. doi: 10.1001/jamaneurol.2022.5128. PMID: 36716024Free PMC Article
Behavioral variant of frontotemporal dementia: Fundamental clinical issues associated with prediction of pathological bases.
Miki T, Yokota O, Ishizu H, Kuroda S, Oshima E, Terada S, Yamada N
Neuropathology 2016 Aug;36(4):388-404. Epub 2016 Mar 11 doi: 10.1111/neup.12290. PMID: 26969837
Metacognition in the behavioral variant of frontotemporal dementia and Alzheimer's disease.
Rosen HJ, Alcantar O, Zakrzewski J, Shimamura AP, Neuhaus J, Miller BL
Neuropsychology 2014 May;28(3):436-47. Epub 2014 Feb 17 doi: 10.1037/neu0000012. PMID: 24548124Free PMC Article
Empathy and contextual social cognition.
Melloni M, Lopez V, Ibanez A
Cogn Affect Behav Neurosci 2014 Mar;14(1):407-25. doi: 10.3758/s13415-013-0205-3. PMID: 23955101
Contextual social cognition and the behavioral variant of frontotemporal dementia.
Ibañez A, Manes F
Neurology 2012 Apr 24;78(17):1354-62. doi: 10.1212/WNL.0b013e3182518375. PMID: 22529204Free PMC Article

Clinical prediction guides

In vivo exploration of synaptic projections in frontotemporal dementia.
Salmon E, Bahri MA, Plenevaux A, Becker G, Seret A, Delhaye E, Degueldre C, Balteau E, Lemaire C, Luxen A, Bastin C
Sci Rep 2021 Aug 9;11(1):16092. doi: 10.1038/s41598-021-95499-1. PMID: 34373529Free PMC Article
Recognition of musical emotions in the behavioral variant of frontotemporal dementia.
Orjuela-Rojas JM, Montañés P, Rodríguez ILL, González-Marín NR
Rev Colomb Psiquiatr (Engl Ed) 2021 Apr-Jun;50(2):74-81. doi: 10.1016/j.rcpeng.2020.01.003. PMID: 34099256
Comparing moral judgments of patients with frontotemporal dementia and frontal stroke.
Baez S, Couto B, Torralva T, Sposato LA, Huepe D, Montañes P, Reyes P, Matallana D, Vigliecca NS, Slachevsky A, Manes F, Ibanez A
JAMA Neurol 2014 Sep;71(9):1172-6. doi: 10.1001/jamaneurol.2014.347. PMID: 25047907
Empathy and contextual social cognition.
Melloni M, Lopez V, Ibanez A
Cogn Affect Behav Neurosci 2014 Mar;14(1):407-25. doi: 10.3758/s13415-013-0205-3. PMID: 23955101
Contextual social cognition and the behavioral variant of frontotemporal dementia.
Ibañez A, Manes F
Neurology 2012 Apr 24;78(17):1354-62. doi: 10.1212/WNL.0b013e3182518375. PMID: 22529204Free PMC Article

Recent systematic reviews

Evaluation of Discriminative Detection Abilities of Social Cognition Measures for the Diagnosis of the Behavioral Variant of Frontotemporal Dementia: a Systematic Review.
Dodich A, Crespi C, Santi GC, Cappa SF, Cerami C
Neuropsychol Rev 2021 Jun;31(2):251-266. Epub 2020 Oct 10 doi: 10.1007/s11065-020-09457-1. PMID: 33040199
The relationship between theory of mind and cognition in Alzheimer's disease: A systematic review.
Lucena AT, Bhalla RK, Belfort Almeida Dos Santos TT, Dourado MCN
J Clin Exp Neuropsychol 2020 Apr;42(3):223-239. Epub 2020 Jan 5 doi: 10.1080/13803395.2019.1710112. PMID: 31902277
Behavioral variant frontotemporal dementia in patients with previous severe mental illness: a systematic and critical review.
Gambogi LB, Guimarães HC, De Souza LC, Caramelli P
Arq Neuropsiquiatr 2019;77(9):654-668. Epub 2019 Sep 23 doi: 10.1590/0004-282X20190107. PMID: 31553396
Phenocopy syndrome of behavioral variant frontotemporal dementia: a systematic review.
Valente ES, Caramelli P, Gambogi LB, Mariano LI, Guimarães HC, Teixeira AL, de Souza LC
Alzheimers Res Ther 2019 Apr 1;11(1):30. doi: 10.1186/s13195-019-0483-2. PMID: 30935398Free PMC Article

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