Corticobasal Degeneration: A Review
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Abstract
Corticobasal degeneration (CBD) is a rare neurodegenerative tauopathy characterized by progressive motor and cognitive dysfunction. This comprehensive review examines the clinical manifestations, neuropathology, diagnostic challenges, and management strategies for CBD. The disease presents with asymmetric motor symptoms, including rigidity, dystonia, myoclonus, and apraxia, often accompanied by cognitive impairments affecting executive function, language, and behavior. Neuropathologically, CBD is defined by 4-repeat tau protein accumulation in neurons and glial cells, producing characteristic lesions including astrocytic plaques and ballooned neurons. Diagnosis remains challenging due to significant clinical overlap with other tauopathies, particularly progressive supranuclear palsy, frontotemporal dementia, and Alzheimer’s disease. Currently, no disease-modifying treatments exist; management focuses on symptomatic relief through multidisciplinary approaches, including physical therapy, occupational therapy, and speech-language pathology. This review synthesizes current understanding of CBD pathophysiology and highlights the urgent need for improved diagnostic biomarkers and targeted therapeutic interventions.
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Copyright (c) 2025 Membreno Lopez AS, et al.
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1. Sacristán H, Cvitanich F, Mentana N, Liotta C. the Essentials in Corticobasal Degeneration. EC Neurology 16.2; 2024. https://ecronicon.net/assets/ecne/pdf/ECNE-16-01158.pdf
2. Constantinides VC, Paraskevas GP, Paraskevas PG, Stefanis L, Kapaki E. Corticobasal degeneration and corticobasal syndrome: A review. Clinical Parkinsonism & Related Disorders. 2019;1:66-71. doi:https://doi.org/10.1016/j.prdoa.2019.08.005
3. Aiba I, Hayashi Y, Shimohata T, et al. Clinical course of pathologically confirmed corticobasal degeneration and corticobasal syndrome. Brain communications. 2023;5(6). doi:https://doi.org/10.1093/braincomms/fcad296
4. Ali F, Josephs KA. Corticobasal degeneration: key emerging issues. Journal of Neurology. 2017;265(2):439-445. doi:https://doi.org/10.1007/s00415-017-8644-3
5. Kouri N, Whitwell JL, Josephs KA, Rademakers R, Dickson DW. Corticobasal degeneration: a pathologically distinct 4R tauopathy. Nature Reviews Neurology. 2011;7(5):263-272. doi:https://doi.org/10.1038/nrneurol.2011.43
6. Mahapatra RK, Edwards MJ, Schott JM, Bhatia KP. Corticobasal Degeneration. The Lancet Neurology. 2004;3(12):736-743. doi:https://doi.org/10.1016/S1474-4422(04)00936-6
7. Rebeiz, J. G., Kolodny, E. H., & Richardson, E. P. (1968). Corticodentatonigral Degeneration With Neuronal Achromasia. Archives of Neurology, 18(1), 20–33. https://doi.org/10.1001/archneur.1968.00470310034003
8. Gibb, G., Luthert, P. J., & Marsden, C. D. (1989). CORTICOBASAL DEGENERATION. Brain, 112(5), 1171–1192. https://doi.org/10.1093/brain/112.5.1171
9. Armstrong, M. J., Litvan, I., & Lang, A. (2014). Diagnosis and Treatment of Corticobasal Degeneration. Current Treatment Options in Neurology, 16(3). https://doi.org/10.1007/s11940-013-0282-1
10. Bhatti, K. S., Sun, C. E., & Walker, I. (2025, February 5). Corticobasal Degeneration. Nih.gov; StatPearls Publishing. https://www.ncbi.nlm.nih.gov/books/NBK611986/
11. Amit Batla, & Deepti Vibha. (2017). Understanding Parkinsonism : the clinical perspective. Jaypee Brothers Medical Publishers (P) Ltd.
12. Burling, S. (2025). NIH Grant Boosts Effort to Identify PET Tracers for Multiple Neurodegenerative Diseases. Neurology Today, 25(4), 1, 19–23. https://doi.org/10.1097/01.wnt.0001108352.95173.e1
13. NIH to lead implementation of National Plan to End Parkinson’s Act. (2025, January 13). National Institutes of Health (NIH). https://www.nih.gov/news-events/news-releases/nih-lead-implementation-national-plan-end-parkinson-s-act
14. Jellinger KA. Pathomechanisms of cognitive and behavioral impairment in corticobasal degeneration. Journal of Neural Transmission. 2023;130(12):1509-1522. doi:https://doi.org/10.1007/s00702-023-02691-w
15.Armstrong RA. Visual signs and symptoms of corticobasal degeneration. Clinical and Experimental Optometry. 2016;99(6):498-506. doi:https://doi.org/10.1111/cxo.12429
16. Stover NP, Watts RL. Corticobasal Degeneration. Seminars in Neurology. 2001;21(01):049-058. doi:https://doi.org/10.1055/s-2001-13119
17. Lee SE, Rabinovici GD, Mayo M, et al. Clinicopathological correlations in corticobasal degeneration. Annals of Neurology. 2011;70(2):327-340. doi:https://doi.org/10.1002/ana.22424
18. Armstrong MJ, Litvan I, Lang AE, et al. Criteria for the diagnosis of corticobasal degeneration. Neurology. 2013;80(5):496-503. doi:https://doi.org/10.1212/wnl.0b013e31827f0fd1
19. Ling H, O’Sullivan SS, Holton JL, et al. Does corticobasal degeneration exist? A clinicopathological re-evaluation. Brain. 2010;133(7):2045-2057. doi:https://doi.org/10.1093/brain/awq123
20. Bhatti KS, Sun CE, Walker I. Corticobasal Degeneration. Nih.gov. Published February 5, 2025. https://www.ncbi.nlm.nih.gov/sites/books/NBK611986/#article-157387.s9
21. Alexander SK, Rittman T, Xuereb JH, Bak TH, Hodges JR, Rowe JB. Validation of the new consensus criteria for the diagnosis of corticobasal degeneration. Journal of Neurology, Neurosurgery & Psychiatry. 2014;85(8):925-929. doi:https://doi.org/10.1136/jnnp-2013-307035
22. Chunowski P, Madetko-Alster N, Alster P. Asymmetry in Atypical Parkinsonian Syndromes—A Review. Journal of Clinical Medicine. 2024;13(19):5798. doi:https://doi.org/10.3390/jcm13195798
23. Fekete R, Bainbridge M, Baizabal-Carvallo JF, et al. Exome sequencing in familial corticobasal degeneration. Parkinsonism Relat Disord. 2013;19(11):1049-1052. doi:10.1016/j.parkreldis.2013.06.016
24. Prado MB, Adiao KJ. Corticobasal syndrome etiologies in a young Filipino patient: A case report and literature review. Clin Neurol Neurosurg. 2021;210. doi:https://doi.org/10.1016/j.clineuro.2021.107002
25. NHS. Corticobasal degeneration – Treatment. NHS. September 27, 2022. https://www.nhs.uk/conditions/corticobasal-degeneration/treatment/
26. Kompoliti K, Goetz CG, Boeve BF, Maraganore DM, Ahlskog JE, Marsden CD, Litvan I. Clinical presentation and pharmacological therapy in corticobasal degeneration. Arch Neurol. 1998;55(7):957-961. doi:10.1001/archneur.55.7.957
27. Steffen, T. M., Boeve, B. F., Petersen, C. M., Dvorak, L., & Kantarci, K. (2014). Long-Term Exercise Training for an Individual With Mixed Corticobasal Degeneration and Progressive Supranuclear Palsy Features: 10-Year Case Report Follow-up. Physical Therapy, 94(2), 289–296. https://doi.org/10.2522/ptj.20130052
28. Silverstein, H. A., Hart, A. R., Bozorg, A., & Hackney, M. E. (2020). Improved Mobility, Cognition, and Disease Severity in Corticobasal Degeneration of an African American Man After 12 Weeks of Adapted Tango. American Journal of Physical Medicine & Rehabilitation, 99(2), e21–e27. https://doi.org/10.1097/phm.0000000000001165
29. PSP Association. A guide to PSP & CBD for occupational therapists. PSP Association. https://www.pspassociation.org.uk/wp-content/uploads/2024/03/OT-guide-2024-Final.pdf
30. Pagan FernandoL, Hebron ML, Wilmarth B, et al. Nilotinib Effects on Safety, Tolerability, and Potential Biomarkers in Parkinson Disease. JAMA Neurology. 2020;77(3):309. doi:https://doi.org/10.1001/jamaneurol.2019.4200
31. Pagan F, Hebron M, Valadez EH, et al. Nilotinib Effects in Parkinson’s disease and Dementia with Lewy bodies. Journal of Parkinson’s Disease. 2016;6(3):503-517. doi:https://doi.org/10.3233/jpd-160867
32. World Health Organization. Palliative care. WHO. 2020. https://www.who.int/news-room/fact-sheets/detail/palliative-care
33. Benefits for corticobasal degeneration. (2025). Disability Benefits Help; Disability Benefits Help. https://www.disability-benefits-help.org/compassionate-allowances/corticobasal-degeneration-and-social-security-disability
34. Bruns, M. B., & Josephs, K. A. (2013). Neuropsychiatry of corticobasal degeneration and progressive supranuclear palsy. International Review of Psychiatry, 25(2), 197–209. https://doi.org/10.3109/09540261.2013.766154
35. Arias, J. J., & Flicker, L. S. (2021). A Matter of Intent: A Social Obligation to Improve Criminal Procedures for Individuals with Dementia. Journal of Law, Medicine & Ethics, 48(2), 318–327. https://doi.org/10.1177/1073110520935345
36. Cornell Law School. Insanity defense. LII / Legal Information Institute. Published 2018. https://www.law.cornell.edu/wex/insanity_defense