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Corticobasal degeneration disorder

MedGen UID:
95979
•Concept ID:
C0393570
•
Disease or Syndrome
Synonyms: Cortical-Basal Ganglionic degeneration; Cortico-Basal Ganglionic Degeneration (CBGD); Corticobasal degeneration
SNOMED CT: Cortical basal ganglionic degeneration (18842008); Corticobasal degeneration (18842008); Corticodentatonigral degeneration with neuronal achromasia (18842008)
 
Monarch Initiative: MONDO:0022308

Definition

A progressive neurodegenerative condition affecting the cerebral cortex and basal ganglia. The disorder is characterized by varying degrees of cognitive and motor impairment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCorticobasal degeneration disorder
Follow this link to review classifications for Corticobasal degeneration disorder in Orphanet.

Professional guidelines

PubMed

(18)F-FDG PET in Parkinsonism: Differential Diagnosis and Evaluation of Cognitive Impairment.
Meyer PT, Frings L, Rücker G, Hellwig S
J Nucl Med 2017 Dec;58(12):1888-1898. Epub 2017 Sep 14 doi: 10.2967/jnumed.116.186403. PMID: 28912150
The Differential Diagnosis and Treatment of Atypical Parkinsonism.
Levin J, Kurz A, Arzberger T, Giese A, Höglinger GU
Dtsch Arztebl Int 2016 Feb 5;113(5):61-9. doi: 10.3238/arztebl.2016.0061. PMID: 26900156Free PMC Article
Criteria for the diagnosis of corticobasal degeneration.
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

Recent clinical studies

Etiology

The therapeutic landscape of tauopathies: challenges and prospects.
Cummings JL, Gonzalez MI, Pritchard MC, May PC, Toledo-Sherman LM, Harris GA
Alzheimers Res Ther 2023 Oct 6;15(1):168. doi: 10.1186/s13195-023-01321-7. PMID: 37803386Free PMC Article
The tauopathies.
Devi G
Handb Clin Neurol 2023;196:251-265. doi: 10.1016/B978-0-323-98817-9.00015-6. PMID: 37620072
Progressive Supranuclear Palsy and Corticobasal Syndrome.
Pantelyat A
Continuum (Minneap Minn) 2022 Oct 1;28(5):1364-1378. doi: 10.1212/CON.0000000000001158. PMID: 36222770
(18)F-FDG PET in Parkinsonism: Differential Diagnosis and Evaluation of Cognitive Impairment.
Meyer PT, Frings L, Rücker G, Hellwig S
J Nucl Med 2017 Dec;58(12):1888-1898. Epub 2017 Sep 14 doi: 10.2967/jnumed.116.186403. PMID: 28912150
Criteria for the diagnosis of corticobasal degeneration.
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

The tauopathies.
Devi G
Handb Clin Neurol 2023;196:251-265. doi: 10.1016/B978-0-323-98817-9.00015-6. PMID: 37620072
Progressive Supranuclear Palsy and Corticobasal Syndrome.
Pantelyat A
Continuum (Minneap Minn) 2022 Oct 1;28(5):1364-1378. doi: 10.1212/CON.0000000000001158. PMID: 36222770
Corticobasal degeneration.
Saranza GM, Whitwell JL, Kovacs GG, Lang AE
Int Rev Neurobiol 2019;149:87-136. Epub 2019 Nov 21 doi: 10.1016/bs.irn.2019.10.014. PMID: 31779825
(18)F-FDG PET in Parkinsonism: Differential Diagnosis and Evaluation of Cognitive Impairment.
Meyer PT, Frings L, Rücker G, Hellwig S
J Nucl Med 2017 Dec;58(12):1888-1898. Epub 2017 Sep 14 doi: 10.2967/jnumed.116.186403. PMID: 28912150
Criteria for the diagnosis of corticobasal degeneration.
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

Therapy

Evolving concepts in progressive supranuclear palsy and other 4-repeat tauopathies.
Stamelou M, Respondek G, Giagkou N, Whitwell JL, Kovacs GG, Höglinger GU
Nat Rev Neurol 2021 Oct;17(10):601-620. Epub 2021 Aug 23 doi: 10.1038/s41582-021-00541-5. PMID: 34426686
Outcome measurement in functional neurological disorder: a systematic review and recommendations.
Pick S, Anderson DG, Asadi-Pooya AA, Aybek S, Baslet G, Bloem BR, Bradley-Westguard A, Brown RJ, Carson AJ, Chalder T, Damianova M, David AS, Edwards MJ, Epstein SA, Espay AJ, Garcin B, Goldstein LH, Hallett M, Jankovic J, Joyce EM, Kanaan RA, Keynejad RC, Kozlowska K, LaFaver K, LaFrance WC Jr, Lang AE, Lehn A, Lidstone S, Maurer CW, Mildon B, Morgante F, Myers L, Nicholson C, Nielsen G, Perez DL, Popkirov S, Reuber M, Rommelfanger KS, Schwingenshuh P, Serranova T, Shotbolt P, Stebbins GT, Stone J, Tijssen MA, Tinazzi M, Nicholson TR
J Neurol Neurosurg Psychiatry 2020 Jun;91(6):638-649. Epub 2020 Feb 28 doi: 10.1136/jnnp-2019-322180. PMID: 32111637Free PMC Article
Tau protein in neurodegenerative diseases - a review.
Pîrşcoveanu DFV, Pirici I, Tudorică V, Bălşeanu TA, Albu VC, Bondari S, Bumbea AM, Pîrşcoveanu M
Rom J Morphol Embryol 2017;58(4):1141-1150. PMID: 29556602
(18)F-FDG PET in Parkinsonism: Differential Diagnosis and Evaluation of Cognitive Impairment.
Meyer PT, Frings L, Rücker G, Hellwig S
J Nucl Med 2017 Dec;58(12):1888-1898. Epub 2017 Sep 14 doi: 10.2967/jnumed.116.186403. PMID: 28912150
Parkinsonian syndromes.
Williams DR, Litvan I
Continuum (Minneap Minn) 2013 Oct;19(5 Movement Disorders):1189-212. doi: 10.1212/01.CON.0000436152.24038.e0. PMID: 24092286Free PMC Article

Prognosis

The tauopathies.
Devi G
Handb Clin Neurol 2023;196:251-265. doi: 10.1016/B978-0-323-98817-9.00015-6. PMID: 37620072
Progressive Supranuclear Palsy and Corticobasal Syndrome.
Pantelyat A
Continuum (Minneap Minn) 2022 Oct 1;28(5):1364-1378. doi: 10.1212/CON.0000000000001158. PMID: 36222770
Amyloid β, Tau, and α-Synuclein aggregates in the pathogenesis, prognosis, and therapeutics for neurodegenerative diseases.
Sengupta U, Kayed R
Prog Neurobiol 2022 Jul;214:102270. Epub 2022 Apr 18 doi: 10.1016/j.pneurobio.2022.102270. PMID: 35447272
Update on posterior cortical atrophy.
Holden SK, Bettcher BM, Pelak VS
Curr Opin Neurol 2020 Feb;33(1):68-73. doi: 10.1097/WCO.0000000000000767. PMID: 31688098Free PMC Article
(18)F-FDG PET in Parkinsonism: Differential Diagnosis and Evaluation of Cognitive Impairment.
Meyer PT, Frings L, Rücker G, Hellwig S
J Nucl Med 2017 Dec;58(12):1888-1898. Epub 2017 Sep 14 doi: 10.2967/jnumed.116.186403. PMID: 28912150

Clinical prediction guides

Amyloid β, Tau, and α-Synuclein aggregates in the pathogenesis, prognosis, and therapeutics for neurodegenerative diseases.
Sengupta U, Kayed R
Prog Neurobiol 2022 Jul;214:102270. Epub 2022 Apr 18 doi: 10.1016/j.pneurobio.2022.102270. PMID: 35447272
Structure-based classification of tauopathies.
Shi Y, Zhang W, Yang Y, Murzin AG, Falcon B, Kotecha A, van Beers M, Tarutani A, Kametani F, Garringer HJ, Vidal R, Hallinan GI, Lashley T, Saito Y, Murayama S, Yoshida M, Tanaka H, Kakita A, Ikeuchi T, Robinson AC, Mann DMA, Kovacs GG, Revesz T, Ghetti B, Hasegawa M, Goedert M, Scheres SHW
Nature 2021 Oct;598(7880):359-363. Epub 2021 Sep 29 doi: 10.1038/s41586-021-03911-7. PMID: 34588692Free PMC Article
Plasma phosphorylated tau 217 and phosphorylated tau 181 as biomarkers in Alzheimer's disease and frontotemporal lobar degeneration: a retrospective diagnostic performance study.
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
Tau aggregates are RNA-protein assemblies that mislocalize multiple nuclear speckle components.
Lester E, Ooi FK, Bakkar N, Ayers J, Woerman AL, Wheeler J, Bowser R, Carlson GA, Prusiner SB, Parker R
Neuron 2021 May 19;109(10):1675-1691.e9. Epub 2021 Apr 12 doi: 10.1016/j.neuron.2021.03.026. PMID: 33848474Free PMC Article
Magnetic resonance imaging in dementia.
Raposo Rodríguez L, Tovar Salazar DJ, Fernández García N, Pastor Hernández L, Fernández Guinea Ó
Radiologia (Engl Ed) 2018 Nov-Dec;60(6):476-484. Epub 2018 Jun 11 doi: 10.1016/j.rx.2018.04.003. PMID: 29903629

Recent systematic reviews

The prevalence and incidence of progressive supranuclear palsy and corticobasal syndrome: a systematic review and meta-analysis.
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
Retina thickness in atypical parkinsonism: a systematic review and meta-analysis.
Ma X, Wang Y, Wang N, Zhang R
J Neurol 2022 Mar;269(3):1272-1281. Epub 2021 Jul 10 doi: 10.1007/s00415-021-10703-6. PMID: 34245345
Genotype-Phenotype Relations for the Atypical Parkinsonism Genes: MDSGene Systematic Review.
Wittke C, Petkovic S, Dobricic V, Schaake S; MDS‐endorsed PSP Study Group, Respondek G, Weissbach A, Madoev H, Trinh J, Vollstedt EJ, Kuhnke N, Lohmann K, Dulovic Mahlow M, Marras C, König IR, Stamelou M, Bonifati V, Lill CM, Kasten M, Huppertz HJ, Höglinger G, Klein C
Mov Disord 2021 Jul;36(7):1499-1510. Epub 2021 Mar 19 doi: 10.1002/mds.28517. PMID: 34396589Free PMC Article
Outcome measurement in functional neurological disorder: a systematic review and recommendations.
Pick S, Anderson DG, Asadi-Pooya AA, Aybek S, Baslet G, Bloem BR, Bradley-Westguard A, Brown RJ, Carson AJ, Chalder T, Damianova M, David AS, Edwards MJ, Epstein SA, Espay AJ, Garcin B, Goldstein LH, Hallett M, Jankovic J, Joyce EM, Kanaan RA, Keynejad RC, Kozlowska K, LaFaver K, LaFrance WC Jr, Lang AE, Lehn A, Lidstone S, Maurer CW, Mildon B, Morgante F, Myers L, Nicholson C, Nielsen G, Perez DL, Popkirov S, Reuber M, Rommelfanger KS, Schwingenshuh P, Serranova T, Shotbolt P, Stebbins GT, Stone J, Tijssen MA, Tinazzi M, Nicholson TR
J Neurol Neurosurg Psychiatry 2020 Jun;91(6):638-649. Epub 2020 Feb 28 doi: 10.1136/jnnp-2019-322180. PMID: 32111637Free PMC Article
Characterization of Movement Disorder Phenomenology in Genetically Proven, Familial Frontotemporal Lobar Degeneration: A Systematic Review and Meta-Analysis.
Gasca-Salas C, Masellis M, Khoo E, Shah BB, Fisman D, Lang AE, Kleiner-Fisman G
PLoS One 2016;11(4):e0153852. Epub 2016 Apr 21 doi: 10.1371/journal.pone.0153852. PMID: 27100392Free PMC Article

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