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Semantic dementia

MedGen UID:
83268
•Concept ID:
C0338462
•
Mental or Behavioral Dysfunction
Synonyms: Dementia, Semantic; Dementias, Semantic; Semantic Dementia; Semantic Dementias
SNOMED CT: Semantic dementia (230288001)
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)
 
HPO: HP:0030219
Monarch Initiative: MONDO:0010857
Orphanet: ORPHA100069

Definition

A progressive loss of the ability to remember the meaning of words, faces and objects. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Semantic dementia
Follow this link to review classifications for Semantic dementia in Orphanet.

Professional guidelines

PubMed

Primary Progressive Aphasia: Toward a Pathophysiological Synthesis.
Ruksenaite J, Volkmer A, Jiang J, Johnson JC, Marshall CR, Warren JD, Hardy CJ
Curr Neurol Neurosci Rep 2021 Feb 4;21(3):7. doi: 10.1007/s11910-021-01097-z. PMID: 33543347Free PMC Article
Frontotemporal lobar degeneration: epidemiology, pathology, diagnosis and management.
Seltman RE, Matthews BR
CNS Drugs 2012 Oct 1;26(10):841-70. doi: 10.2165/11640070-000000000-00000. PMID: 22950490
Primary progressive aphasia.
Mesulam MM
Ann Neurol 2001 Apr;49(4):425-32. PMID: 11310619

Recent clinical studies

Etiology

Right temporal degeneration and socioemotional semantics: semantic behavioural variant frontotemporal dementia.
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
Primary progressive aphasia: a clinical approach.
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
The frontotemporal dementias.
Waldö ML
Psychiatr Clin North Am 2015 Jun;38(2):193-209. Epub 2015 Mar 18 doi: 10.1016/j.psc.2015.02.001. PMID: 25998110
Frontotemporal dementia and its subtypes: a genome-wide association study.
Ferrari R, Hernandez DG, Nalls MA, Rohrer JD, Ramasamy A, Kwok JB, Dobson-Stone C, Brooks WS, Schofield PR, Halliday GM, Hodges JR, Piguet O, Bartley L, Thompson E, Haan E, Hernández I, Ruiz A, Boada M, Borroni B, Padovani A, Cruchaga C, Cairns NJ, Benussi L, Binetti G, Ghidoni R, Forloni G, Galimberti D, Fenoglio C, Serpente M, Scarpini E, Clarimón J, Lleó A, Blesa R, Waldö ML, Nilsson K, Nilsson C, Mackenzie IR, Hsiung GY, Mann DM, Grafman J, Morris CM, Attems J, Griffiths TD, McKeith IG, Thomas AJ, Pietrini P, Huey ED, Wassermann EM, Baborie A, Jaros E, Tierney MC, Pastor P, Razquin C, Ortega-Cubero S, Alonso E, Perneczky R, Diehl-Schmid J, Alexopoulos P, Kurz A, Rainero I, Rubino E, Pinessi L, Rogaeva E, St George-Hyslop P, Rossi G, Tagliavini F, Giaccone G, Rowe JB, Schlachetzki JC, Uphill J, Collinge J, Mead S, Danek A, Van Deerlin VM, Grossman M, Trojanowski JQ, van der Zee J, Deschamps W, Van Langenhove T, Cruts M, Van Broeckhoven C, Cappa SF, Le Ber I, Hannequin D, Golfier V, Vercelletto M, Brice A, Nacmias B, Sorbi S, Bagnoli S, Piaceri I, Nielsen JE, Hjermind LE, Riemenschneider M, Mayhaus M, Ibach B, Gasparoni G, Pichler S, Gu W, Rossor MN, Fox NC, Warren JD, Spillantini MG, Morris HR, Rizzu P, Heutink P, Snowden JS, Rollinson S, Richardson A, Gerhard A, Bruni AC, Maletta R, Frangipane F, Cupidi C, Bernardi L, Anfossi M, Gallo M, Conidi ME, Smirne N, Rademakers R, Baker M, Dickson DW, Graff-Radford NR, Petersen RC, Knopman D, Josephs KA, Boeve BF, Parisi JE, Seeley WW, Miller BL, Karydas AM, Rosen H, van Swieten JC, Dopper EG, Seelaar H, Pijnenburg YA, Scheltens P, Logroscino G, Capozzo R, Novelli V, Puca AA, Franceschi M, Postiglione A, Milan G, Sorrentino P, Kristiansen M, Chiang HH, Graff C, Pasquier F, Rollin A, Deramecourt V, Lebert F, Kapogiannis D, Ferrucci L, Pickering-Brown S, Singleton AB, Hardy J, Momeni P
Lancet Neurol 2014 Jul;13(7):686-99. doi: 10.1016/S1474-4422(14)70065-1. PMID: 24943344Free PMC Article
Primary progressive aphasia.
Mesulam MM
Ann Neurol 2001 Apr;49(4):425-32. PMID: 11310619

Diagnosis

Primary progressive aphasia: six questions in search of an answer.
Belder CRS, Marshall CR, Jiang J, Mazzeo S, Chokesuwattanaskul A, Rohrer JD, Volkmer A, Hardy CJD, Warren JD
J Neurol 2024 Feb;271(2):1028-1046. Epub 2023 Oct 31 doi: 10.1007/s00415-023-12030-4. PMID: 37906327Free PMC Article
Neuroimaging in aging and neurologic diseases.
Risacher SL, Saykin AJ
Handb Clin Neurol 2019;167:191-227. doi: 10.1016/B978-0-12-804766-8.00012-1. PMID: 31753134Free PMC Article
Primary Progressive Aphasias and Apraxia of Speech.
Botha H, Josephs KA
Continuum (Minneap Minn) 2019 Feb;25(1):101-127. doi: 10.1212/CON.0000000000000699. PMID: 30707189Free PMC Article
Frontotemporal dementia.
Deleon J, Miller BL
Handb Clin Neurol 2018;148:409-430. doi: 10.1016/B978-0-444-64076-5.00027-2. PMID: 29478591
Primary progressive aphasia: a clinical approach.
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

Therapy

Relationship between hearing impairment and dementia and cognitive function: a Mendelian randomization study.
Jiang D, Hou J, Nan H, Yue A, Chu M, Wang Y, Wang Y, Wu L
Alzheimers Res Ther 2024 Oct 9;16(1):215. doi: 10.1186/s13195-024-01586-6. PMID: 39385207Free PMC Article
The frontotemporal dementias.
Waldö ML
Psychiatr Clin North Am 2015 Jun;38(2):193-209. Epub 2015 Mar 18 doi: 10.1016/j.psc.2015.02.001. PMID: 25998110
Semantic dementia: a specific network-opathy.
Fletcher PD, Warren JD
J Mol Neurosci 2011 Nov;45(3):629-36. Epub 2011 Jun 29 doi: 10.1007/s12031-011-9586-3. PMID: 21710360Free PMC Article
Pharmacological treatment of frontotemporal lobar degeneration: systematic review.
Portugal Mda G, Marinho V, Laks J
Braz J Psychiatry 2011 Mar;33(1):81-90. doi: 10.1590/s1516-44462011000100016. PMID: 21537725
Frontotemporal dementia and pharmacologic interventions.
Kaye ED, Petrovic-Poljak A, Verhoeff NP, Freedman M
J Neuropsychiatry Clin Neurosci 2010 Winter;22(1):19-29. doi: 10.1176/jnp.2010.22.1.19. PMID: 20160206

Prognosis

Primary progressive aphasia: six questions in search of an answer.
Belder CRS, Marshall CR, Jiang J, Mazzeo S, Chokesuwattanaskul A, Rohrer JD, Volkmer A, Hardy CJD, Warren JD
J Neurol 2024 Feb;271(2):1028-1046. Epub 2023 Oct 31 doi: 10.1007/s00415-023-12030-4. PMID: 37906327Free PMC Article
Right temporal degeneration and socioemotional semantics: semantic behavioural variant frontotemporal dementia.
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
Physical understanding in neurodegenerative diseases.
Baumard J, Lesourd M, Guézouli L, Osiurak F
Cogn Neuropsychol 2021 Oct-Dec;38(7-8):490-514. Epub 2022 May 13 doi: 10.1080/02643294.2022.2071152. PMID: 35549825
Primary Progressive Aphasias and Apraxia of Speech.
Botha H, Josephs KA
Continuum (Minneap Minn) 2019 Feb;25(1):101-127. doi: 10.1212/CON.0000000000000699. PMID: 30707189Free PMC Article
An update on semantic dementia: genetics, imaging, and pathology.
Landin-Romero R, Tan R, Hodges JR, Kumfor F
Alzheimers Res Ther 2016 Dec 5;8(1):52. doi: 10.1186/s13195-016-0219-5. PMID: 27915998Free PMC Article

Clinical prediction guides

Relationship between hearing impairment and dementia and cognitive function: a Mendelian randomization study.
Jiang D, Hou J, Nan H, Yue A, Chu M, Wang Y, Wang Y, Wu L
Alzheimers Res Ther 2024 Oct 9;16(1):215. doi: 10.1186/s13195-024-01586-6. PMID: 39385207Free PMC Article
Primary progressive aphasia: six questions in search of an answer.
Belder CRS, Marshall CR, Jiang J, Mazzeo S, Chokesuwattanaskul A, Rohrer JD, Volkmer A, Hardy CJD, Warren JD
J Neurol 2024 Feb;271(2):1028-1046. Epub 2023 Oct 31 doi: 10.1007/s00415-023-12030-4. PMID: 37906327Free PMC Article
Changing perspectives on frontotemporal dementia: A review.
Snowden JS
J Neuropsychol 2023 Jun;17(2):211-234. Epub 2022 Oct 31 doi: 10.1111/jnp.12297. PMID: 36315040
Right temporal degeneration and socioemotional semantics: semantic behavioural variant frontotemporal dementia.
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
Physical understanding in neurodegenerative diseases.
Baumard J, Lesourd M, Guézouli L, Osiurak F
Cogn Neuropsychol 2021 Oct-Dec;38(7-8):490-514. Epub 2022 May 13 doi: 10.1080/02643294.2022.2071152. PMID: 35549825

Recent systematic reviews

FDG-PET in the diagnosis of primary progressive aphasia: a systematic review.
Mirbod M, Ayubcha C, Redden HWK, Teichner E, Subtirelu RC, Patel R, Raynor W, Werner T, Alavi A, Revheim ME
Ann Nucl Med 2024 Sep;38(9):673-687. Epub 2024 Jul 19 doi: 10.1007/s12149-024-01958-w. PMID: 39028529Free PMC Article
Behavioral Treatment for Speech and Language in Primary Progressive Aphasia and Primary Progressive Apraxia of Speech: A Systematic Review.
Wauters LD, Croot K, Dial HR, Duffy JR, Grasso SM, Kim E, Schaffer Mendez K, Ballard KJ, Clark HM, Kohley L, Murray LL, Rogalski EJ, Figeys M, Milman L, Henry ML
Neuropsychol Rev 2024 Sep;34(3):882-923. Epub 2023 Oct 4 doi: 10.1007/s11065-023-09607-1. PMID: 37792075Free PMC Article
Right Temporal Lobe Variant of Frontotemporal Dementia: Systematic Review.
Campos DF, Rocca AR, Caixeta LF
Alzheimer Dis Assoc Disord 2022 Jul-Sep 01;36(3):272-279. Epub 2022 Jul 21 doi: 10.1097/WAD.0000000000000511. PMID: 35867973
Atrophy in Alzheimer's Disease and Semantic Dementia: An ALE Meta-Analysis of Voxel-Based Morphometry Studies.
Chapleau M, Aldebert J, Montembeault M, Brambati SM
J Alzheimers Dis 2016 Oct 4;54(3):941-955. doi: 10.3233/JAD-160382. PMID: 27567843
Pharmacological treatment of frontotemporal lobar degeneration: systematic review.
Portugal Mda G, Marinho V, Laks J
Braz J Psychiatry 2011 Mar;33(1):81-90. doi: 10.1590/s1516-44462011000100016. PMID: 21537725

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