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Logopenic progressive aphasia

MedGen UID:
907332
•Concept ID:
C4274665
•
Mental or Behavioral Dysfunction
Synonyms: Logopenic primary progressive aphasia; logopenic progressive aphasia; Logopenic variant PPA; LPA
SNOMED CT: Logopenic progressive aphasia (716380002)
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.
See: This record
Non-Mendelian inheritance (Orphanet)
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0016644
Orphanet: ORPHA250831

Definition

A form of primary progressive aphasia with characteristics of impaired single-word retrieval and naming and impaired repetition with spared single-word comprehension and object knowledge. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLogopenic progressive aphasia

Professional guidelines

PubMed

Validating new diagnostic imaging criteria for primary progressive aphasia via anatomical likelihood estimation meta-analyses.
Bisenius S, Neumann J, Schroeter ML
Eur J Neurol 2016 Apr;23(4):704-12. Epub 2016 Feb 22 doi: 10.1111/ene.12902. PMID: 26901360
Is descriptive writing useful in the differential diagnosis of logopenic variant of primary progressive aphasia, Alzheimer's disease and mild cognitive impairment?
Sitek EJ, Barczak A, Kluj-Kozłowska K, Kozłowski M, Barcikowska M, Sławek J
Neurol Neurochir Pol 2015;49(4):239-44. Epub 2015 Jun 17 doi: 10.1016/j.pjnns.2015.06.001. PMID: 26188940
Fluorodeoxyglucose F18 positron emission tomography in progressive apraxia of speech and primary progressive aphasia variants.
Josephs KA, Duffy JR, Fossett TR, Strand EA, Claassen DO, Whitwell JL, Peller PJ
Arch Neurol 2010 May;67(5):596-605. doi: 10.1001/archneurol.2010.78. PMID: 20457960

Recent clinical studies

Etiology

Distinct brain iron profiles associated with logopenic progressive aphasia and posterior cortical atrophy.
Singh NA, Arani A, Graff-Radford J, Senjem ML, Martin PR, Machulda MM, Schwarz CG, Shu Y, Cogswell PM, Knopman DS, Petersen RC, Lowe VJ, Jack CR Jr, Josephs KA, Whitwell JL
Neuroimage Clin 2022;36:103161. Epub 2022 Aug 22 doi: 10.1016/j.nicl.2022.103161. PMID: 36029670Free PMC Article
Regional white matter hyperintensities in posterior cortical atrophy and logopenic progressive aphasia.
Pham NTT, Graff-Radford J, Machulda MM, Spychalla AJ, Schwarz CG, Senjem ML, Lowe VJ, Vemuri P, Kantarci K, Knopman DS, Petersen RC, Jack CR, Josephs KA, Whitwell JL
Neurobiol Aging 2022 Nov;119:46-55. Epub 2022 Jul 28 doi: 10.1016/j.neurobiolaging.2022.07.008. PMID: 35970009Free PMC Article
Clinical, Imaging, and Pathologic Characteristics of Patients With Right vs Left Hemisphere-Predominant Logopenic Progressive Aphasia.
Buciuc M, Duffy JR, Machulda MM, Graff-Radford J, Pham NTT, Martin PR, Senjem ML, Jack CR Jr, Ertekin-Taner N, Dickson DW, Lowe VJ, Whitwell JL, Josephs KA
Neurology 2021 Aug 3;97(5):e523-e534. Epub 2021 Jun 4 doi: 10.1212/WNL.0000000000012322. PMID: 34088877Free PMC Article
Lewy Body Disease is a Contributor to Logopenic Progressive Aphasia Phenotype.
Buciuc M, Whitwell JL, Kasanuki K, Graff-Radford J, Machulda MM, Duffy JR, Strand EA, Lowe VJ, Graff-Radford NR, Rush BK, Franczak MB, Flanagan ME, Baker MC, Rademakers R, Ross OA, Ghetti BF, Parisi JE, Raghunathan A, Reichard RR, Bigio EH, Dickson DW, Josephs KA
Ann Neurol 2021 Mar;89(3):520-533. Epub 2020 Dec 17 doi: 10.1002/ana.25979. PMID: 33274526Free PMC Article
Deciphering logopenic primary progressive aphasia: a clinical, imaging and biomarker investigation.
Teichmann M, Kas A, Boutet C, Ferrieux S, Nogues M, Samri D, Rogan C, Dormont D, Dubois B, Migliaccio R
Brain 2013 Nov;136(Pt 11):3474-88. Epub 2013 Oct 8 doi: 10.1093/brain/awt266. PMID: 24108322

Diagnosis

Diffusivity Changes in Posterior Cortical Atrophy and Logopenic Progressive Aphasia: A Longitudinal Diffusion Tensor Imaging Study.
Singh NA, Graff-Radford J, Machulda MM, Pham NTT, Schwarz CG, Reid RI, Lowe VJ, Petersen RC, Jack CR, Josephs KA, Whitwell JL
J Alzheimers Dis 2023;94(2):709-725. doi: 10.3233/JAD-221217. PMID: 37302032Free PMC Article
Lewy Body Disease is a Contributor to Logopenic Progressive Aphasia Phenotype.
Buciuc M, Whitwell JL, Kasanuki K, Graff-Radford J, Machulda MM, Duffy JR, Strand EA, Lowe VJ, Graff-Radford NR, Rush BK, Franczak MB, Flanagan ME, Baker MC, Rademakers R, Ross OA, Ghetti BF, Parisi JE, Raghunathan A, Reichard RR, Bigio EH, Dickson DW, Josephs KA
Ann Neurol 2021 Mar;89(3):520-533. Epub 2020 Dec 17 doi: 10.1002/ana.25979. PMID: 33274526Free PMC Article
Deciphering logopenic primary progressive aphasia: a clinical, imaging and biomarker investigation.
Teichmann M, Kas A, Boutet C, Ferrieux S, Nogues M, Samri D, Rogan C, Dormont D, Dubois B, Migliaccio R
Brain 2013 Nov;136(Pt 11):3474-88. Epub 2013 Oct 8 doi: 10.1093/brain/awt266. PMID: 24108322
Primary progressive aphasia: clinicopathological correlations.
Grossman M
Nat Rev Neurol 2010 Feb;6(2):88-97. doi: 10.1038/nrneurol.2009.216. PMID: 20139998Free PMC Article
An overview on Primary Progressive Aphasia and its variants.
Amici S, Gorno-Tempini ML, Ogar JM, Dronkers NF, Miller BL
Behav Neurol 2006;17(2):77-87. doi: 10.1155/2006/260734. PMID: 16873918Free PMC Article

Prognosis

Clinical and cortical trajectories in non-fluent primary progressive aphasia and Alzheimer's disease: A role for emotion processing.
Landin-Romero R, Kumfor F, Ys Lee A, Leyton C, Piguet O
Brain Res 2024 Apr 15;1829:148777. Epub 2024 Jan 28 doi: 10.1016/j.brainres.2024.148777. PMID: 38286395
Altered reward processing underpins emotional apathy in dementia.
Wong S, Wei G, Husain M, Hodges JR, Piguet O, Irish M, Kumfor F
Cogn Affect Behav Neurosci 2023 Apr;23(2):354-370. Epub 2022 Nov 23 doi: 10.3758/s13415-022-01048-2. PMID: 36417157Free PMC Article
Retained capacity for perceptual learning of degraded speech in primary progressive aphasia and Alzheimer's disease.
Hardy CJD, Marshall CR, Bond RL, Russell LL, Dick K, Ariti C, Thomas DL, Ross SJ, Agustus JL, Crutch SJ, Rohrer JD, Bamiou DE, Warren JD
Alzheimers Res Ther 2018 Jul 25;10(1):70. doi: 10.1186/s13195-018-0399-2. PMID: 30045755Free PMC Article
The midbrain-to-pons ratio distinguishes progressive supranuclear palsy from non-fluent primary progressive aphasias.
Silsby M, Tweedie-Cullen RY, Murray CR, Halliday GM, Hodges JR, Burrell JR
Eur J Neurol 2017 Jul;24(7):956-965. Epub 2017 May 16 doi: 10.1111/ene.13314. PMID: 28510312
Progranulin mutations as risk factors for Alzheimer disease.
Perry DC, Lehmann M, Yokoyama JS, Karydas A, Lee JJ, Coppola G, Grinberg LT, Geschwind D, Seeley WW, Miller BL, Rosen H, Rabinovici G
JAMA Neurol 2013 Jun;70(6):774-8. doi: 10.1001/2013.jamaneurol.393. PMID: 23609919Free PMC Article

Clinical prediction guides

Distinct brain iron profiles associated with logopenic progressive aphasia and posterior cortical atrophy.
Singh NA, Arani A, Graff-Radford J, Senjem ML, Martin PR, Machulda MM, Schwarz CG, Shu Y, Cogswell PM, Knopman DS, Petersen RC, Lowe VJ, Jack CR Jr, Josephs KA, Whitwell JL
Neuroimage Clin 2022;36:103161. Epub 2022 Aug 22 doi: 10.1016/j.nicl.2022.103161. PMID: 36029670Free PMC Article
Apathy and its impact on carer burden and psychological wellbeing in primary progressive aphasia.
Wong S, Irish M, Husain M, Hodges JR, Piguet O, Kumfor F
J Neurol Sci 2020 Sep 15;416:117007. Epub 2020 Jun 30 doi: 10.1016/j.jns.2020.117007. PMID: 32623141
Western Aphasia Battery-Revised Profiles in Primary Progressive Aphasia and Primary Progressive Apraxia of Speech.
Clark HM, Utianski RL, Duffy JR, Strand EA, Botha H, Josephs KA, Whitwell JL
Am J Speech Lang Pathol 2020 Feb 21;29(1S):498-510. Epub 2019 Oct 22 doi: 10.1044/2019_AJSLP-CAC48-18-0217. PMID: 31639312Free PMC Article
Logopenic progressive aphasia with neologisms: a case report.
Watanabe H, Ikeda M, Mori E
BMC Neurol 2019 Nov 25;19(1):299. doi: 10.1186/s12883-019-1524-y. PMID: 31766995Free PMC Article
Is the logopenic-variant of primary progressive aphasia a unitary disorder?
Leyton CE, Hodges JR, McLean CA, Kril JJ, Piguet O, Ballard KJ
Cortex 2015 Jun;67:122-33. Epub 2015 Apr 1 doi: 10.1016/j.cortex.2015.03.011. PMID: 25955499

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