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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.
 
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

Lashkarivand A, Eide PK
Neurology 2022 May 10;98(19):798-805. Epub 2022 Mar 25 doi: 10.1212/WNL.0000000000200511. PMID: 35338080
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
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

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
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
Van den Stock J, De Winter FL, de Gelder B, Rangarajan JR, Cypers G, Maes F, Sunaert S, Goffin K, Vandenberghe R, Vandenbulcke M
Neuropsychologia 2015 Aug;75:496-504. Epub 2015 Jul 8 doi: 10.1016/j.neuropsychologia.2015.06.035. PMID: 26162615
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
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

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
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
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
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
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

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
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
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
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
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

Sokołowski A, Brown JA, Roy ARK, Cryns N, Scheffler A, Hardy EG, Datta S, Seeley WW, Sturm VE, Miller BL, Rosen HJ, Perry DC
Cortex 2024 Dec;181:47-58. Epub 2024 Oct 22 doi: 10.1016/j.cortex.2024.09.011. PMID: 39488010
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
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
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
Ibañez A, Manes F
Neurology 2012 Apr 24;78(17):1354-62. doi: 10.1212/WNL.0b013e3182518375. PMID: 22529204Free PMC Article

Clinical prediction guides

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
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
Van den Stock J, De Winter FL, de Gelder B, Rangarajan JR, Cypers G, Maes F, Sunaert S, Goffin K, Vandenberghe R, Vandenbulcke M
Neuropsychologia 2015 Aug;75:496-504. Epub 2015 Jul 8 doi: 10.1016/j.neuropsychologia.2015.06.035. PMID: 26162615
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
Ibañez A, Manes F
Neurology 2012 Apr 24;78(17):1354-62. doi: 10.1212/WNL.0b013e3182518375. PMID: 22529204Free PMC Article

Recent systematic reviews

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
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
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
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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