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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.
 
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

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
Cardarelli R, Kertesz A, Knebl JA
Am Fam Physician 2010 Dec 1;82(11):1372-7. PMID: 21121521
Mesulam MM
Ann Neurol 2001 Apr;49(4):425-32. PMID: 11310619

Recent clinical studies

Etiology

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
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
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
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
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

Diagnosis

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
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
Botha H, Josephs KA
Continuum (Minneap Minn) 2019 Feb;25(1):101-127. doi: 10.1212/CON.0000000000000699. PMID: 30707189Free PMC Article
Deleon J, Miller BL
Handb Clin Neurol 2018;148:409-430. doi: 10.1016/B978-0-444-64076-5.00027-2. PMID: 29478591
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

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
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
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
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
Arvanitakis Z
Neurologist 2010 Jan;16(1):16-22. doi: 10.1097/NRL.0b013e3181b1d5c6. PMID: 20065792Free PMC Article

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
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
Botha H, Josephs KA
Continuum (Minneap Minn) 2019 Feb;25(1):101-127. doi: 10.1212/CON.0000000000000699. PMID: 30707189Free PMC Article
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
Knibb JA, Kipps CM, Hodges JR
Curr Opin Neurol 2006 Dec;19(6):565-71. doi: 10.1097/01.wco.0000247606.57567.41. PMID: 17102695

Clinical prediction guides

Snowden JS
J Neuropsychol 2023 Jun;17(2):211-234. Epub 2022 Oct 31 doi: 10.1111/jnp.12297. PMID: 36315040
Borghesani V, DeLeon J, Gorno-Tempini ML
Handb Clin Neurol 2022;187:429-448. doi: 10.1016/B978-0-12-823493-8.00011-0. PMID: 35964986Free PMC Article
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
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
Snowden JS, Harris JM, Thompson JC, Kobylecki C, Jones M, Richardson AM, Neary D
Cortex 2018 Oct;107:188-203. Epub 2017 Aug 31 doi: 10.1016/j.cortex.2017.08.024. PMID: 28947063

Recent systematic reviews

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
Ulugut H, Dijkstra AA, Scarioni M; Netherlands Brain Bank, Barkhof F, Scheltens P, Rozemuller AJM, Pijnenburg YAL
Acta Neuropathol Commun 2021 Aug 3;9(1):131. doi: 10.1186/s40478-021-01229-z. PMID: 34344452Free PMC Article
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
Portugal Mda G, Marinho V, Laks J
Braz J Psychiatry 2011 Mar;33(1):81-90. doi: 10.1590/s1516-44462011000100016. PMID: 21537725
Gainotti G
Neuropsychologia 2007 Apr 9;45(8):1591-607. Epub 2007 Jan 7 doi: 10.1016/j.neuropsychologia.2006.12.013. PMID: 17275042

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