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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

Pini L, Pizzini FB, Boscolo-Galazzo I, Ferrari C, Galluzzi S, Cotelli M, Gobbi E, Cattaneo A, Cotelli MS, Geroldi C, Zanetti O, Corbetta M, van den Heuvel M, Frisoni GB, Manenti R, Pievani M
Neurobiol Aging 2022 Mar;111:24-34. Epub 2021 Nov 20 doi: 10.1016/j.neurobiolaging.2021.11.005. PMID: 34942516
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
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
Caruso G, Perri R, Fadda L, Caltagirone C, Carlesimo GA
J Alzheimers Dis 2020;77(2):655-666. doi: 10.3233/JAD-200126. PMID: 32741812
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

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
Laforce R Jr
Clin Neurol Neurosurg 2013 Dec;115(12):2405-10. doi: 10.1016/j.clineuro.2013.09.031. PMID: 24446563
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
Ibañez A, Manes F
Neurology 2012 Apr 24;78(17):1354-62. doi: 10.1212/WNL.0b013e3182518375. PMID: 22529204Free PMC Article

Therapy

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
Pardini M, Serrati C, Guida S, Mattei C, Abate L, Massucco D, Sassos D, Amore M, Krueger F, Cocito L, Emberti Gialloreti L
Neurodegener Dis 2015;15(1):58-62. Epub 2015 Jan 15 doi: 10.1159/000369811. PMID: 25592742
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

Rus T, Perovnik M, Vo A, Nguyen N, Tang C, Jamšek J, Šurlan Popović K, Grimmer T, Yakushev I, Diehl-Schmid J, Eidelberg D, Trošt M
Hum Brain Mapp 2023 Feb 15;44(3):1079-1093. Epub 2022 Nov 5 doi: 10.1002/hbm.26140. PMID: 36334269Free PMC Article
Bergeron D, Sellami L, Poulin S, Verret L, Bouchard RW, Laforce R Jr
Dement Geriatr Cogn Disord 2020;49(5):518-525. Epub 2020 Nov 18 doi: 10.1159/000511210. PMID: 33207355
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

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
Teichmann M, Daigmorte C, Funkiewiez A, Sanches C, Camus M, Mauras T, Le Ber I, Dubois B, Levy R, Azuar C
J Alzheimers Dis 2019;69(3):887-896. doi: 10.3233/JAD-180991. PMID: 31127763
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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