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Degenerations, Frontotemporal Lobar

MedGen UID:
148228
Concept ID:
C0751072
Mental or Behavioral Dysfunction
Synonyms: Degeneration, Frontotemporal Lobar; Frontotemporal Lobar Degeneration; Frontotemporal Lobar Degenerations; FTLD; FTLDs; Lobar Degeneration, Frontotemporal; Lobar Degenerations, Frontotemporal

Definition

Heterogeneous group of neurodegenerative disorders characterized by frontal and temporal lobe atrophy associated with neuronal loss, gliosis, and dementia. Patients exhibit progressive changes in social, behavioral, and/or language function. Multiple subtypes or forms are recognized based on presence or absence of TAU PROTEIN inclusions. FTLD includes three clinical syndromes: FRONTOTEMPORAL DEMENTIA, semantic dementia, and PRIMARY PROGRESSIVE NONFLUENT APHASIA. [from MeSH]

Professional guidelines

PubMed

Loi SM, Cations M, Velakoulis D
Med J Aust 2023 Mar 6;218(4):182-189. Epub 2023 Feb 19 doi: 10.5694/mja2.51849. PMID: 36807325Free PMC Article
Magrath Guimet N, Zapata-Restrepo LM, Miller BL
J Neuropsychiatry Clin Neurosci 2022 Fall;34(4):316-327. Epub 2022 May 17 doi: 10.1176/appi.neuropsych.21060166. PMID: 35578801
Bott NT, Radke A, Stephens ML, Kramer JH
Neurodegener Dis Manag 2014;4(6):439-54. doi: 10.2217/nmt.14.34. PMID: 25531687Free PMC Article

Recent clinical studies

Etiology

Boeve BF, Boxer AL, Kumfor F, Pijnenburg Y, Rohrer JD
Lancet Neurol 2022 Mar;21(3):258-272. doi: 10.1016/S1474-4422(21)00341-0. PMID: 35182511
Meneses A, Koga S, O'Leary J, Dickson DW, Bu G, Zhao N
Mol Neurodegener 2021 Dec 20;16(1):84. doi: 10.1186/s13024-021-00503-x. PMID: 34930382Free PMC Article
Thijssen EH, La Joie R, Strom A, Fonseca C, Iaccarino L, Wolf A, Spina S, Allen IE, Cobigo Y, Heuer H, VandeVrede L, Proctor NK, Lago AL, Baker S, Sivasankaran R, Kieloch A, Kinhikar A, Yu L, Valentin MA, Jeromin A, Zetterberg H, Hansson O, Mattsson-Carlgren N, Graham D, Blennow K, Kramer JH, Grinberg LT, Seeley WW, Rosen H, Boeve BF, Miller BL, Teunissen CE, Rabinovici GD, Rojas JC, Dage JL, Boxer AL; Advancing Research and Treatment for Frontotemporal Lobar Degeneration investigators
Lancet Neurol 2021 Sep;20(9):739-752. doi: 10.1016/S1474-4422(21)00214-3. PMID: 34418401Free PMC Article
Elahi FM, Miller BL
Nat Rev Neurol 2017 Aug;13(8):457-476. Epub 2017 Jul 14 doi: 10.1038/nrneurol.2017.96. PMID: 28708131Free PMC Article
Bang J, Spina S, Miller BL
Lancet 2015 Oct 24;386(10004):1672-82. doi: 10.1016/S0140-6736(15)00461-4. PMID: 26595641Free PMC Article

Diagnosis

Grossman M, Seeley WW, Boxer AL, Hillis AE, Knopman DS, Ljubenov PA, Miller B, Piguet O, Rademakers R, Whitwell JL, Zetterberg H, van Swieten JC
Nat Rev Dis Primers 2023 Aug 10;9(1):40. doi: 10.1038/s41572-023-00447-0. PMID: 37563165
Boeve BF
Continuum (Minneap Minn) 2022 Jun 1;28(3):702-725. doi: 10.1212/CON.0000000000001105. PMID: 35678399Free PMC Article
Boeve BF, Boxer AL, Kumfor F, Pijnenburg Y, Rohrer JD
Lancet Neurol 2022 Mar;21(3):258-272. doi: 10.1016/S1474-4422(21)00341-0. PMID: 35182511
Elahi FM, Miller BL
Nat Rev Neurol 2017 Aug;13(8):457-476. Epub 2017 Jul 14 doi: 10.1038/nrneurol.2017.96. PMID: 28708131Free PMC Article
Bang J, Spina S, Miller BL
Lancet 2015 Oct 24;386(10004):1672-82. doi: 10.1016/S0140-6736(15)00461-4. PMID: 26595641Free PMC Article

Therapy

Ji D, Chen WZ, Zhang L, Zhang ZH, Chen LJ
J Neuroinflammation 2024 Jan 4;21(1):2. doi: 10.1186/s12974-023-02999-0. PMID: 38178103Free PMC Article
Kurnellas M, Mitra A, Schwabe T, Paul R, Arrant AE, Roberson ED, Ward M, Yeh F, Long H, Rosenthal A
J Transl Med 2023 Jun 15;21(1):387. doi: 10.1186/s12967-023-04251-y. PMID: 37322482Free PMC Article
Willemse SW, Harley P, van Eijk RPA, Demaegd KC, Zelina P, Pasterkamp RJ, van Damme P, Ingre C, van Rheenen W, Veldink JH, Kiernan MC, Al-Chalabi A, van den Berg LH, Fratta P, van Es MA
J Neurol Neurosurg Psychiatry 2023 Aug;94(8):649-656. Epub 2023 Feb 3 doi: 10.1136/jnnp-2022-330504. PMID: 36737245Free PMC Article
Perugi G, Vannucchi G, Bedani F, Favaretto E
Curr Psychiatry Rep 2017 Jan;19(1):7. doi: 10.1007/s11920-017-0758-x. PMID: 28144880
Walker AK, Spiller KJ, Ge G, Zheng A, Xu Y, Zhou M, Tripathy K, Kwong LK, Trojanowski JQ, Lee VM
Acta Neuropathol 2015 Nov;130(5):643-60. Epub 2015 Jul 22 doi: 10.1007/s00401-015-1460-x. PMID: 26197969Free PMC Article

Prognosis

Weintraub S
Continuum (Minneap Minn) 2022 Jun 1;28(3):781-799. doi: 10.1212/CON.0000000000001135. PMID: 35678402Free PMC Article
Boeve BF
Continuum (Minneap Minn) 2022 Jun 1;28(3):702-725. doi: 10.1212/CON.0000000000001105. PMID: 35678399Free PMC Article
Thijssen EH, La Joie R, Strom A, Fonseca C, Iaccarino L, Wolf A, Spina S, Allen IE, Cobigo Y, Heuer H, VandeVrede L, Proctor NK, Lago AL, Baker S, Sivasankaran R, Kieloch A, Kinhikar A, Yu L, Valentin MA, Jeromin A, Zetterberg H, Hansson O, Mattsson-Carlgren N, Graham D, Blennow K, Kramer JH, Grinberg LT, Seeley WW, Rosen H, Boeve BF, Miller BL, Teunissen CE, Rabinovici GD, Rojas JC, Dage JL, Boxer AL; Advancing Research and Treatment for Frontotemporal Lobar Degeneration investigators
Lancet Neurol 2021 Sep;20(9):739-752. doi: 10.1016/S1474-4422(21)00214-3. PMID: 34418401Free PMC Article
Sivasathiaseelan H, Marshall CR, Agustus JL, Benhamou E, Bond RL, van Leeuwen JEP, Hardy CJD, Rohrer JD, Warren JD
Semin Neurol 2019 Apr;39(2):251-263. Epub 2019 Mar 29 doi: 10.1055/s-0039-1683379. PMID: 30925617
Bott NT, Radke A, Stephens ML, Kramer JH
Neurodegener Dis Manag 2014;4(6):439-54. doi: 10.2217/nmt.14.34. PMID: 25531687Free PMC Article

Clinical prediction guides

Boeve BF
Continuum (Minneap Minn) 2022 Jun 1;28(3):702-725. doi: 10.1212/CON.0000000000001105. PMID: 35678399Free PMC Article
Thijssen EH, La Joie R, Strom A, Fonseca C, Iaccarino L, Wolf A, Spina S, Allen IE, Cobigo Y, Heuer H, VandeVrede L, Proctor NK, Lago AL, Baker S, Sivasankaran R, Kieloch A, Kinhikar A, Yu L, Valentin MA, Jeromin A, Zetterberg H, Hansson O, Mattsson-Carlgren N, Graham D, Blennow K, Kramer JH, Grinberg LT, Seeley WW, Rosen H, Boeve BF, Miller BL, Teunissen CE, Rabinovici GD, Rojas JC, Dage JL, Boxer AL; Advancing Research and Treatment for Frontotemporal Lobar Degeneration investigators
Lancet Neurol 2021 Sep;20(9):739-752. doi: 10.1016/S1474-4422(21)00214-3. PMID: 34418401Free PMC Article
Nelson PT, Dickson DW, Trojanowski JQ, Jack CR, Boyle PA, Arfanakis K, Rademakers R, Alafuzoff I, Attems J, Brayne C, Coyle-Gilchrist ITS, Chui HC, Fardo DW, Flanagan ME, Halliday G, Hokkanen SRK, Hunter S, Jicha GA, Katsumata Y, Kawas CH, Keene CD, Kovacs GG, Kukull WA, Levey AI, Makkinejad N, Montine TJ, Murayama S, Murray ME, Nag S, Rissman RA, Seeley WW, Sperling RA, White CL 3rd, Yu L, Schneider JA
Brain 2019 Jun 1;142(6):1503-1527. doi: 10.1093/brain/awz099. PMID: 31039256Free PMC Article
Elahi FM, Miller BL
Nat Rev Neurol 2017 Aug;13(8):457-476. Epub 2017 Jul 14 doi: 10.1038/nrneurol.2017.96. PMID: 28708131Free PMC Article
Swinnen B, Robberecht W
Nat Rev Neurol 2014 Nov;10(11):661-70. Epub 2014 Oct 14 doi: 10.1038/nrneurol.2014.184. PMID: 25311585

Recent systematic reviews

Guay-Gagnon M, Vat S, Forget MF, Tremblay-Gravel M, Ducharme S, Nguyen QD, Desmarais P
J Sleep Res 2022 Oct;31(5):e13589. Epub 2022 Apr 2 doi: 10.1111/jsr.13589. PMID: 35366021
Roman Meller M, Patel S, Duarte D, Kapczinski F, de Azevedo Cardoso T
Acta Psychiatr Scand 2021 Nov;144(5):433-447. Epub 2021 Aug 25 doi: 10.1111/acps.13362. PMID: 34390495
Arevalo-Rodriguez I, Smailagic N, Roqué-Figuls M, Ciapponi A, Sanchez-Perez E, Giannakou A, Pedraza OL, Bonfill Cosp X, Cullum S
Cochrane Database Syst Rev 2021 Jul 27;7(7):CD010783. doi: 10.1002/14651858.CD010783.pub3. PMID: 34313331Free PMC Article
Hogan DB, Jetté N, Fiest KM, Roberts JI, Pearson D, Smith EE, Roach P, Kirk A, Pringsheim T, Maxwell CJ
Can J Neurol Sci 2016 Apr;43 Suppl 1:S96-S109. doi: 10.1017/cjn.2016.25. PMID: 27307130
Arevalo-Rodriguez I, Smailagic N, Roqué I Figuls M, Ciapponi A, Sanchez-Perez E, Giannakou A, Pedraza OL, Bonfill Cosp X, Cullum S
Cochrane Database Syst Rev 2015 Mar 5;2015(3):CD010783. doi: 10.1002/14651858.CD010783.pub2. PMID: 25740785Free PMC Article

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