U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Posterior cortical atrophy

MedGen UID:
909667
Concept ID:
C4275079
Disease or Syndrome
Synonyms: Benson syndrome; Biparietal Alzheimer disease; biparietal Alzheimer disease; PCA; posterior cortical atrophy; Posterior cortical atrophy syndrome
SNOMED CT: Benson syndrome (715574002); Posterior cortical atrophy syndrome (715574002); Posterior cortical atrophy (715574002)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
Concept ID:
CN307042
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
 
Monarch Initiative: MONDO:0018899
Orphanet: ORPHA54247

Definition

A rare progressive neurodegenerative disorder with typical onset between 50 and 65 years of age. Manifestation is of progressive impairment of higher visual processing skills and other posterior cortical functions without any evidence of ocular abnormalities. Prevalence is unknown, largely due to the lack of awareness of the syndrome and the inaccurate terminology referring to it. Alzheimer''s disease is the most common underlying pathology, but cases attributable to Dementia with Lewy Bodies, corticobasal degeneration or prion disease have also been reported. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPosterior cortical atrophy

Professional guidelines

PubMed

Mendez MF
J Neuropsychiatry Clin Neurosci 2018 Fall;30(4):334-336. Epub 2018 Apr 24 doi: 10.1176/appi.neuropsych.17120356. PMID: 29685063Free PMC Article
Morhardt D, Weintraub S, Khayum B, Robinson J, Medina J, O'Hara M, Mesulam M, Rogalski EJ
Psychiatr Clin North Am 2015 Jun;38(2):333-52. Epub 2015 Mar 7 doi: 10.1016/j.psc.2015.01.005. PMID: 25998120Free PMC Article
Sitek EJ, Barczak A, Harciarek M
Psychiatr Clin North Am 2015 Jun;38(2):265-79. Epub 2015 Mar 7 doi: 10.1016/j.psc.2015.01.003. PMID: 25998115

Recent clinical studies

Etiology

Chapleau M, La Joie R, Yong K, Agosta F, Allen IE, Apostolova L, Best J, Boon BDC, Crutch S, Filippi M, Fumagalli GG, Galimberti D, Graff-Radford J, Grinberg LT, Irwin DJ, Josephs KA, Mendez MF, Mendez PC, Migliaccio R, Miller ZA, Montembeault M, Murray ME, Nemes S, Pelak V, Perani D, Phillips J, Pijnenburg Y, Rogalski E, Schott JM, Seeley W, Sullivan AC, Spina S, Tanner J, Walker J, Whitwell JL, Wolk DA, Ossenkoppele R, Rabinovici GD; PCA International Work Group
Lancet Neurol 2024 Feb;23(2):168-177. doi: 10.1016/S1474-4422(23)00414-3. PMID: 38267189
Polsinelli AJ, Apostolova LG
Continuum (Minneap Minn) 2022 Jun 1;28(3):676-701. doi: 10.1212/CON.0000000000001082. PMID: 35678398Free 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
Villain N, Dubois B
Semin Neurol 2019 Apr;39(2):213-226. Epub 2019 Mar 29 doi: 10.1055/s-0039-1681041. PMID: 30925614
Suárez-González A, Henley SM, Walton J, Crutch SJ
Psychiatr Clin North Am 2015 Jun;38(2):211-20. Epub 2015 Mar 12 doi: 10.1016/j.psc.2015.01.009. PMID: 25998111

Diagnosis

Polsinelli AJ, Apostolova LG
Continuum (Minneap Minn) 2022 Jun 1;28(3):676-701. doi: 10.1212/CON.0000000000001082. PMID: 35678398Free PMC Article
Holden SK, Bettcher BM, Pelak VS
Curr Opin Neurol 2020 Feb;33(1):68-73. doi: 10.1097/WCO.0000000000000767. PMID: 31688098Free 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
Villain N, Dubois B
Semin Neurol 2019 Apr;39(2):213-226. Epub 2019 Mar 29 doi: 10.1055/s-0039-1681041. PMID: 30925614
Schott JM, Crutch SJ
Continuum (Minneap Minn) 2019 Feb;25(1):52-75. doi: 10.1212/CON.0000000000000696. PMID: 30707187Free PMC Article

Therapy

Montembeault M, Migliaccio R
Curr Opin Neurol 2023 Aug 1;36(4):245-252. Epub 2023 Jun 22 doi: 10.1097/WCO.0000000000001182. PMID: 37365819
North C, Desai R, Saunders R, Suárez-González A, Bamiou D, Costafreda SG, de Haan G, Halls G, Heutink J, O'Nions E, Utoomprurkporn N, John A, Stott J
Cortex 2021 Oct;143:223-236. Epub 2021 Aug 6 doi: 10.1016/j.cortex.2021.07.011. PMID: 34464853
Suarez-Gonzalez A, Ocal D, Pavisic I, Peacock A, Naessens M, Ahmed S, Butler CR, Leff AP, Yong KXX, Crutch SJ
J Alzheimers Dis 2019;71(4):1285-1295. doi: 10.3233/JAD-190335. PMID: 31524161
Yong KX, Rajdev K, Shakespeare TJ, Leff AP, Crutch SJ
Neurology 2015 Jul 28;85(4):339-48. Epub 2015 Jul 2 doi: 10.1212/WNL.0000000000001782. PMID: 26138948Free PMC Article
Crutch SJ, Lehmann M, Warren JD, Rohrer JD
J Neurol Neurosurg Psychiatry 2013 Apr;84(4):460-6. Epub 2012 Nov 8 doi: 10.1136/jnnp-2012-303309. PMID: 23138762Free PMC Article

Prognosis

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
Holden SK, Bettcher BM, Pelak VS
Curr Opin Neurol 2020 Feb;33(1):68-73. doi: 10.1097/WCO.0000000000000767. PMID: 31688098Free PMC Article
Wong B, Lucente DE, MacLean J, Padmanabhan J, Quimby M, Brandt KD, Putcha D, Sherman J, Frosch MP, McGinnis S, Dickerson BC
Neurodegener Dis Manag 2019 Aug;9(4):217-239. Epub 2019 Aug 8 doi: 10.2217/nmt-2018-0052. PMID: 31392920Free PMC Article
Montembeault M, Lacomblez L, Habert MO, Kas A, Migliaccio R
Geriatr Psychol Neuropsychiatr Vieil 2018 Mar 1;16(1):57-66. doi: 10.1684/pnv.2017.0717. PMID: 29569567
Benson DF, Davis RJ, Snyder BD
Arch Neurol 1988 Jul;45(7):789-93. doi: 10.1001/archneur.1988.00520310107024. PMID: 3390033

Clinical prediction guides

Cipriano L, Oliva M, Puoti G, Signoriello E, Bonavita S, Coppola C
Rev Neurosci 2022 Dec 16;33(8):849-858. Epub 2022 Jun 6 doi: 10.1515/revneuro-2022-0003. PMID: 35659868
Shir D, Graff-Radford J, Machulda MM, Pham NTT, Jack CR Jr, Lowe VJ, Whitwell JL, Josephs KA
Eur J Neurol 2022 Jul;29(7):2138-2143. Epub 2022 Mar 30 doi: 10.1111/ene.15327. PMID: 35298068Free PMC Article
North C, Desai R, Saunders R, Suárez-González A, Bamiou D, Costafreda SG, de Haan G, Halls G, Heutink J, O'Nions E, Utoomprurkporn N, John A, Stott J
Cortex 2021 Oct;143:223-236. Epub 2021 Aug 6 doi: 10.1016/j.cortex.2021.07.011. PMID: 34464853
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
Tetzloff KA, Duffy JR, Strand EA, Machulda MM, Schwarz CG, Senjem ML, Jack CR, Josephs KA, Whitwell JL
Dement Geriatr Cogn Disord 2021;50(2):195-203. Epub 2021 Jul 16 doi: 10.1159/000516481. PMID: 34274933Free PMC Article

Recent systematic reviews

Cotta Ramusino M, Massa F, Festari C, Gandolfo F, Nicolosi V, Orini S, Nobili F, Frisoni GB, Morbelli S, Garibotto V; European Inter-Societal Consensus on the Biomarker-Based Diagnosis of Dementia
Eur J Nucl Med Mol Imaging 2024 Jun;51(7):1876-1890. Epub 2024 Feb 15 doi: 10.1007/s00259-024-06631-y. PMID: 38355740
Collins JD, Henley SMD, Suárez-González A
Int Psychogeriatr 2023 Sep;35(9):457-476. Epub 2020 Jul 20 doi: 10.1017/S1041610220001118. PMID: 32684177
Pelak VS, Mahmood A, Abe-Ridgway K
Curr Neurol Neurosci Rep 2022 Nov;22(11):803-812. Epub 2022 Oct 15 doi: 10.1007/s11910-022-01238-y. PMID: 36242715
Shea YF, Pan Y, Mak HK, Bao Y, Lee SC, Chiu PK, Chan HF
Psychogeriatrics 2021 May;21(3):396-406. Epub 2021 Feb 16 doi: 10.1111/psyg.12665. PMID: 33594793
Alves J, Soares JM, Sampaio A, Gonçalves OF
Brain Imaging Behav 2013 Sep;7(3):353-61. doi: 10.1007/s11682-013-9236-1. PMID: 23690254

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...