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Corticobasal syndrome

MedGen UID:
1801322
Concept ID:
C5575119
Disease or Syndrome
Synonym: corticobasal syndrome
SNOMED CT: Corticobasal syndrome (1230018005)
 
Monarch Initiative: MONDO:0018696
Orphanet: ORPHA454887

Definition

A rare neurologic disease with characteristics of multifaceted motor system dysfunctions and cognitive defects such as asymmetric rigidity, bradykinesia, limb apraxia and visuospatial dysfunction. The disease shows a wide clinical variability between patients with many developing a relatively pure motor syndrome, and others displaying a combination of motor and cognitive deficits. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Corticobasal syndrome

Professional guidelines

PubMed

VandeVrede L, Ljubenkov PA, Rojas JC, Welch AE, Boxer AL
Neurotherapeutics 2020 Oct;17(4):1563-1581. doi: 10.1007/s13311-020-00888-5. PMID: 32676851Free 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
Mahlknecht P, Hotter A, Hussl A, Esterhammer R, Schocke M, Seppi K
Neurodegener Dis 2010;7(5):300-18. Epub 2010 Jul 9 doi: 10.1159/000314495. PMID: 20616565

Recent clinical studies

Etiology

Pantelyat A
Continuum (Minneap Minn) 2022 Oct 1;28(5):1364-1378. doi: 10.1212/CON.0000000000001158. PMID: 36222770
Polsinelli AJ, Apostolova LG
Continuum (Minneap Minn) 2022 Jun 1;28(3):676-701. doi: 10.1212/CON.0000000000001082. PMID: 35678398Free PMC Article
Jabbari E, Holland N, Chelban V, Jones PS, Lamb R, Rawlinson C, Guo T, Costantini AA, Tan MMX, Heslegrave AJ, Roncaroli F, Klein JC, Ansorge O, Allinson KSJ, Jaunmuktane Z, Holton JL, Revesz T, Warner TT, Lees AJ, Zetterberg H, Russell LL, Bocchetta M, Rohrer JD, Williams NM, Grosset DG, Burn DJ, Pavese N, Gerhard A, Kobylecki C, Leigh PN, Church A, Hu MTM, Woodside J, Houlden H, Rowe JB, Morris HR
JAMA Neurol 2020 Mar 1;77(3):377-387. doi: 10.1001/jamaneurol.2019.4347. PMID: 31860007Free 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
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

Diagnosis

Utianski RL, Josephs KA
Curr Neurol Neurosci Rep 2023 Jul;23(7):353-359. Epub 2023 Jun 3 doi: 10.1007/s11910-023-01275-1. PMID: 37269450Free PMC Article
Pantelyat A
Continuum (Minneap Minn) 2022 Oct 1;28(5):1364-1378. doi: 10.1212/CON.0000000000001158. PMID: 36222770
Polsinelli AJ, Apostolova LG
Continuum (Minneap Minn) 2022 Jun 1;28(3):676-701. doi: 10.1212/CON.0000000000001082. PMID: 35678398Free 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
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

Therapy

Lyons S, Trépel D, Lynch T, Walsh R, O'Dowd S
J Neurol 2023 Sep;270(9):4451-4465. Epub 2023 Jun 8 doi: 10.1007/s00415-023-11791-2. PMID: 37289323Free PMC Article
Street D, Jabbari E, Costantini A, Jones PS, Holland N, Rittman T, Jensen MT, Chelban V, Goh YY, Guo T, Heslegrave AJ, Roncaroli F, Klein JC, Ansorge O, Allinson KSJ, Jaunmuktane Z, Revesz T, Warner TT, Lees AJ, Zetterberg H, Russell LL, Bocchetta M, Rohrer JD, Burn DJ, Pavese N, Gerhard A, Kobylecki C, Leigh PN, Church A, Hu MTM, Houlden H, Morris H, Rowe JB
Brain 2023 Aug 1;146(8):3232-3242. doi: 10.1093/brain/awad105. PMID: 36975168Free PMC Article
VandeVrede L, Ljubenkov PA, Rojas JC, Welch AE, Boxer AL
Neurotherapeutics 2020 Oct;17(4):1563-1581. doi: 10.1007/s13311-020-00888-5. PMID: 32676851Free PMC Article
Murley AG, Rowe JB
Brain 2018 May 1;141(5):1263-1285. doi: 10.1093/brain/awx327. PMID: 29373632Free PMC Article
Graff-Radford J, Boeve BF, Drubach DA, Knopman DS, Ahlskog JE, Golden EC, Drubach DI, Petersen RC, Josephs KA
Parkinsonism Relat Disord 2012 Dec;18(10):1097-9. Epub 2012 Jun 20 doi: 10.1016/j.parkreldis.2012.05.025. PMID: 22721974Free PMC Article

Prognosis

Pantelyat A
Continuum (Minneap Minn) 2022 Oct 1;28(5):1364-1378. doi: 10.1212/CON.0000000000001158. PMID: 36222770
Karikari TK, Pascoal TA, Ashton NJ, Janelidze S, Benedet AL, Rodriguez JL, Chamoun M, Savard M, Kang MS, Therriault J, Schöll M, Massarweh G, Soucy JP, Höglund K, Brinkmalm G, Mattsson N, Palmqvist S, Gauthier S, Stomrud E, Zetterberg H, Hansson O, Rosa-Neto P, Blennow K
Lancet Neurol 2020 May;19(5):422-433. doi: 10.1016/S1474-4422(20)30071-5. PMID: 32333900
Jiang C, Hopfner F, Katsikoudi A, Hein R, Catli C, Evetts S, Huang Y, Wang H, Ryder JW, Kuhlenbaeumer G, Deuschl G, Padovani A, Berg D, Borroni B, Hu MT, Davis JJ, Tofaris GK
J Neurol Neurosurg Psychiatry 2020 Jul;91(7):720-729. Epub 2020 Apr 9 doi: 10.1136/jnnp-2019-322588. PMID: 32273329Free PMC Article
Keener AM, Bordelon YM
Semin Neurol 2016 Aug;36(4):330-4. Epub 2016 Sep 19 doi: 10.1055/s-0036-1585097. PMID: 27643900
Grijalvo-Perez AM, Litvan I
Semin Neurol 2014 Apr;34(2):160-73. Epub 2014 Jun 25 doi: 10.1055/s-0034-1381734. PMID: 24963675

Clinical prediction guides

Shir D, Pham NTT, Botha H, Koga S, Kouri N, Ali F, Knopman DS, Petersen RC, Boeve BF, Kremers WK, Nguyen AT, Murray ME, Reichard RR, Dickson DW, Graff-Radford N, Josephs KA, Whitwell J, Graff-Radford J
Neurology 2023 Jul 18;101(3):e289-e299. Epub 2023 Jun 2 doi: 10.1212/WNL.0000000000207397. PMID: 37268436Free 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
Karikari TK, Pascoal TA, Ashton NJ, Janelidze S, Benedet AL, Rodriguez JL, Chamoun M, Savard M, Kang MS, Therriault J, Schöll M, Massarweh G, Soucy JP, Höglund K, Brinkmalm G, Mattsson N, Palmqvist S, Gauthier S, Stomrud E, Zetterberg H, Hansson O, Rosa-Neto P, Blennow K
Lancet Neurol 2020 May;19(5):422-433. doi: 10.1016/S1474-4422(20)30071-5. PMID: 32333900
Jiang C, Hopfner F, Katsikoudi A, Hein R, Catli C, Evetts S, Huang Y, Wang H, Ryder JW, Kuhlenbaeumer G, Deuschl G, Padovani A, Berg D, Borroni B, Hu MT, Davis JJ, Tofaris GK
J Neurol Neurosurg Psychiatry 2020 Jul;91(7):720-729. Epub 2020 Apr 9 doi: 10.1136/jnnp-2019-322588. PMID: 32273329Free PMC Article
Finger EC
Continuum (Minneap Minn) 2016 Apr;22(2 Dementia):464-89. doi: 10.1212/CON.0000000000000300. PMID: 27042904Free PMC Article

Recent systematic reviews

Taha HB, Bogoniewski A
J Neurol 2024 Apr;271(4):1680-1706. Epub 2023 Dec 16 doi: 10.1007/s00415-023-12093-3. PMID: 38103086Free PMC Article
Taha HB, Ati SS
CNS Neurosci Ther 2023 Dec;29(12):3741-3755. Epub 2023 Jul 7 doi: 10.1111/cns.14341. PMID: 37416941Free PMC Article
Lyons S, Trépel D, Lynch T, Walsh R, O'Dowd S
J Neurol 2023 Sep;270(9):4451-4465. Epub 2023 Jun 8 doi: 10.1007/s00415-023-11791-2. PMID: 37289323Free PMC Article
Arienti F, Lazzeri G, Vizziello M, Monfrini E, Bresolin N, Saetti MC, Picillo M, Franco G, Di Fonzo A
Cells 2021 Jan 15;10(1) doi: 10.3390/cells10010171. PMID: 33467748Free PMC Article
Niccolini F, Politis M
Eur J Nucl Med Mol Imaging 2016 Nov;43(12):2244-2254. Epub 2016 Jul 28 doi: 10.1007/s00259-016-3464-8. PMID: 27470326Free PMC Article

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