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Primary progressive aphasia(PPA)

MedGen UID:
79466
Concept ID:
C0282513
Mental or Behavioral Dysfunction
Synonyms: PPA; Primary progressive aphasia syndrome
SNOMED CT: Primary progressive apraxia of speech (723124007); Primary progressive aphasia (723124007)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
Concept ID:
C0600599
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019806
OMIM®: 138945; 607485
Orphanet: ORPHA95432

Definition

Primary progressive aphasia (PPA) is a neurodegenerative disorder, characterized by a primary dissolution of language, with relative sparing of other mental faculties for at least the first 2 years of illness. PPA is recognized as the language variant in the frontotemporal dementia (FTD; see this term) spectrum of disorders. PPA can be classified into 3 subtypes based on specific speech and language features: semantic dementia (SD), progressive non-fluent aphasia (PNFA) and logopenic progressive aphasia (lv-PPA) (see these terms). [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary progressive aphasia
Follow this link to review classifications for Primary progressive aphasia in Orphanet.

Professional guidelines

PubMed

Volkmer A, Rogalski E, Henry M, Taylor-Rubin C, Ruggero L, Khayum R, Kindell J, Gorno-Tempini ML, Warren JD, Rohrer JD
Pract Neurol 2020 Apr;20(2):154-161. Epub 2019 Jul 29 doi: 10.1136/practneurol-2018-001921. PMID: 31358572Free PMC Article
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
Armstrong MJ, Litvan I, Lang AE, Bak TH, Bhatia KP, Borroni B, Boxer AL, Dickson DW, Grossman M, Hallett M, Josephs KA, Kertesz A, Lee SE, Miller BL, Reich SG, Riley DE, Tolosa E, Tröster AI, Vidailhet M, Weiner WJ
Neurology 2013 Jan 29;80(5):496-503. doi: 10.1212/WNL.0b013e31827f0fd1. PMID: 23359374Free PMC Article

Recent clinical studies

Etiology

Polsinelli AJ, Apostolova LG
Continuum (Minneap Minn) 2022 Jun 1;28(3):676-701. doi: 10.1212/CON.0000000000001082. PMID: 35678398Free PMC Article
Puppala GK, Gorthi SP, Chandran V, Gundabolu G
Neurol India 2021 Sep-Oct;69(5):1144-1152. doi: 10.4103/0028-3886.329593. PMID: 34747778
Volkmer A, Rogalski E, Henry M, Taylor-Rubin C, Ruggero L, Khayum R, Kindell J, Gorno-Tempini ML, Warren JD, Rohrer JD
Pract Neurol 2020 Apr;20(2):154-161. Epub 2019 Jul 29 doi: 10.1136/practneurol-2018-001921. PMID: 31358572Free PMC Article
Marshall CR, Hardy CJD, Volkmer A, Russell LL, Bond RL, Fletcher PD, Clark CN, Mummery CJ, Schott JM, Rossor MN, Fox NC, Crutch SJ, Rohrer JD, Warren JD
J Neurol 2018 Jun;265(6):1474-1490. Epub 2018 Feb 1 doi: 10.1007/s00415-018-8762-6. PMID: 29392464Free PMC Article
Gorno-Tempini ML, Hillis AE, Weintraub S, Kertesz A, Mendez M, Cappa SF, Ogar JM, Rohrer JD, Black S, Boeve BF, Manes F, Dronkers NF, Vandenberghe R, Rascovsky K, Patterson K, Miller BL, Knopman DS, Hodges JR, Mesulam MM, Grossman M
Neurology 2011 Mar 15;76(11):1006-14. Epub 2011 Feb 16 doi: 10.1212/WNL.0b013e31821103e6. PMID: 21325651Free PMC Article

Diagnosis

Puppala GK, Gorthi SP, Chandran V, Gundabolu G
Neurol India 2021 Sep-Oct;69(5):1144-1152. doi: 10.4103/0028-3886.329593. PMID: 34747778
Marshall CR, Hardy CJD, Volkmer A, Russell LL, Bond RL, Fletcher PD, Clark CN, Mummery CJ, Schott JM, Rossor MN, Fox NC, Crutch SJ, Rohrer JD, Warren JD
J Neurol 2018 Jun;265(6):1474-1490. Epub 2018 Feb 1 doi: 10.1007/s00415-018-8762-6. PMID: 29392464Free PMC Article
Olney NT, Spina S, Miller BL
Neurol Clin 2017 May;35(2):339-374. doi: 10.1016/j.ncl.2017.01.008. PMID: 28410663Free PMC Article
Mendez MF
Neurol Clin 2017 May;35(2):263-281. doi: 10.1016/j.ncl.2017.01.005. PMID: 28410659Free PMC Article
Gorno-Tempini ML, Hillis AE, Weintraub S, Kertesz A, Mendez M, Cappa SF, Ogar JM, Rohrer JD, Black S, Boeve BF, Manes F, Dronkers NF, Vandenberghe R, Rascovsky K, Patterson K, Miller BL, Knopman DS, Hodges JR, Mesulam MM, Grossman M
Neurology 2011 Mar 15;76(11):1006-14. Epub 2011 Feb 16 doi: 10.1212/WNL.0b013e31821103e6. PMID: 21325651Free PMC Article

Therapy

Nassan M, Piras IS, Rogalski E, Geula C, Mesulam MM, Huentelman M
Neurology 2023 May 2;100(18):e1922-e1929. Epub 2023 Mar 8 doi: 10.1212/WNL.0000000000207136. PMID: 36889925Free PMC Article
Themistocleous C, Ficek B, Webster K, den Ouden DB, Hillis AE, Tsapkini K
J Alzheimers Dis 2021;79(3):1185-1194. doi: 10.3233/JAD-201101. PMID: 33427742Free PMC Article
Zhao Y, Ficek B, Webster K, Frangakis C, Caffo B, Hillis AE, Faria A, Tsapkini K
Neurorehabil Neural Repair 2021 Jan;35(1):44-57. doi: 10.1177/1545968320971741. PMID: 33317422Free PMC Article
Eikelboom WS, Janssen N, Jiskoot LC, van den Berg E, Roelofs A, Kessels RPC
Neurosci Biobehav Rev 2018 Sep;92:243-254. Epub 2018 Jun 18 doi: 10.1016/j.neubiorev.2018.06.015. PMID: 29928907
Cadório I, Lousada M, Martins P, Figueiredo D
Int J Lang Commun Disord 2017 Sep;52(5):543-560. Epub 2017 Jan 24 doi: 10.1111/1460-6984.12310. PMID: 28120406

Prognosis

Younes K, Borghesani V, Montembeault M, Spina S, Mandelli ML, Welch AE, Weis E, Callahan P, Elahi FM, Hua AY, Perry DC, Karydas A, Geschwind D, Huang E, Grinberg LT, Kramer JH, Boxer AL, Rabinovici GD, Rosen HJ, Seeley WW, Miller ZA, Miller BL, Sturm VE, Rankin KP, Gorno-Tempini ML
Brain 2022 Nov 21;145(11):4080-4096. doi: 10.1093/brain/awac217. PMID: 35731122Free PMC Article
Botha H, Josephs KA
Continuum (Minneap Minn) 2019 Feb;25(1):101-127. doi: 10.1212/CON.0000000000000699. PMID: 30707189Free PMC Article
Mendez MF
Neurol Clin 2017 May;35(2):263-281. doi: 10.1016/j.ncl.2017.01.005. PMID: 28410659Free PMC Article
Fried-Oken M, Mooney A, Peters B
NeuroRehabilitation 2015;37(1):69-87. doi: 10.3233/NRE-151241. PMID: 26409694Free PMC Article
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

Younes K, Borghesani V, Montembeault M, Spina S, Mandelli ML, Welch AE, Weis E, Callahan P, Elahi FM, Hua AY, Perry DC, Karydas A, Geschwind D, Huang E, Grinberg LT, Kramer JH, Boxer AL, Rabinovici GD, Rosen HJ, Seeley WW, Miller ZA, Miller BL, Sturm VE, Rankin KP, Gorno-Tempini ML
Brain 2022 Nov 21;145(11):4080-4096. doi: 10.1093/brain/awac217. PMID: 35731122Free 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
Rowe JB
Int Rev Neurobiol 2019;149:249-275. Epub 2019 Nov 21 doi: 10.1016/bs.irn.2019.10.012. PMID: 31779815
Finger EC
Continuum (Minneap Minn) 2016 Apr;22(2 Dementia):464-89. doi: 10.1212/CON.0000000000000300. PMID: 27042904Free PMC Article
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 systematic reviews

Conca F, Esposito V, Giusto G, Cappa SF, Catricalà E
Ageing Res Rev 2022 Dec;82:101760. Epub 2022 Oct 13 doi: 10.1016/j.arr.2022.101760. PMID: 36244629
Karantali E, Kazis D, Chatzikonstantinou S, Petridis F, Mavroudis I
Aging Clin Exp Res 2021 Apr;33(4):869-881. Epub 2020 Apr 18 doi: 10.1007/s40520-020-01554-8. PMID: 32306372
Stalpaert J, Cocquyt EM, Criel Y, Segers L, Miatton M, Van Langenhove T, van Mierlo P, De Letter M
Am J Speech Lang Pathol 2020 Nov 12;29(4):2206-2225. Epub 2020 Aug 17 doi: 10.1044/2020_AJSLP-20-00008. PMID: 32810414
Cadório I, Lousada M, Martins P, Figueiredo D
Int J Lang Commun Disord 2017 Sep;52(5):543-560. Epub 2017 Jan 24 doi: 10.1111/1460-6984.12310. PMID: 28120406
Villarejo-Galende A, Llamas-Velasco S, Gómez-Grande A, Puertas-Martín V, Contador I, Sarandeses P, González-Sánchez M, Trincado R, Pilkington P, Ruiz-Solis S, Pérez-Martínez DA, Herrero-San Martín A
J Neurol 2017 Jan;264(1):121-130. Epub 2016 Nov 4 doi: 10.1007/s00415-016-8324-8. PMID: 27815682

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