Which neurological disorder is characterized by cortical nerve cell processes?

Constantinides VC, Paraskevas GP, Paraskevas PG, Stefanis L, Kapaki E. Corticobasal degeneration and corticobasal syndrome: A review. Clin Park Relat Disord. 2019;1:66-71. Published 2019 Aug 30. doi:10.1016/j.prdoa.2019.08.005

Inhaltsverzeichnis Show

Products & Services
Complications
Associated Procedures
Products & Services
Corticobasal degeneration (corticobasal syndrome)
Advertisement

Ling H, Macerollo A. Is it useful to classify PSP and CBD as different disorders? Yes. Mov Disord Clin Pract. 2018;5(2):145-148. Published 2018 Mar 6. doi:10.1002/mdc3.12581

Kouri N, Carlomagno Y, Baker M, et al. Novel mutation in MAPT exon 13 (p.N410H) causes corticobasal degeneration. Acta Neuropathol. 2014;127(2):271-282. doi:10.1007/s00401-013-1193-7

Lee SE, Rabinovici GD, Mayo MC, et al. Clinicopathological correlations in corticobasal degeneration. Ann Neurol. 2011;70:327-340.
http://www.ncbi.nlm.nih.gov/pubmed/21823158

Hassan A, Whitwell JL, Josephs KA. The corticobasal syndrome-Alzheimer’s disease conundrum. Expert Rev Neurother. 2011;11:1569-1578. http://www.ncbi.nlm.nih.gov/pubmed/22014136

Canu E, Boccardi M, Ghidoni R, et al. H1 haplotype of the MAPT gene is associated with lower regional gray matter volume in healthy carriers. Eur J Hum Genet. 2009;17(3):287-294. doi:10.1038/ejhg.2008.185

Ludolph AC, Kassubek J, Landwehrmeye BG, et al. Tauopathies with parkinsonism: clinical spectrum, neuropathologic basis, biological markers, and treatment options. Eur J Neurol. 2009;16:297-309. http://www.ncbi.nlm.nih.gov/pubmed/19364361

Oshima K, Dickson DW. Cortical Alzheimer type pathology does not influence tau pathology in progressive supranuclear palsy. Int J Clin Exp Pathol. 2009;2(4):399-406.

Webb A, Miller B, Bonasera S, et al. Role of the tau gene region chromosome inversion in progressive supranuclear palsy, corticobasal degeneration, and related disorders. Arch Neurol. 2008;65:1473-1478. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2680206/

Geda YE, Boeve BF, Negash S, et al. Neuropsychiatric features in 36 pathologically confirmed cases of corticobasal degeneration. J Neuropsychiatry Clin Neurosci. 2007;19:77-80. http://www.ncbi.nlm.nih.gov/pubmed/17308231

Pittman AM, Fung HC, de Silva R. Untangling the tau gene association with neurodegenerative disorders. Hum Mol Genet. 2006;15:R188-195. http://www.ncbi.nlm.nih.gov/pubmed/16987883

Goedert M, Jakes R. Mutations causing neurodegenerative tauopathies. Biochim Biophys Acta. 2005;1739:240-250. http://www.ncbi.nlm.nih.gov/pubmed/15615642

Boeve BF, Lang AE, Litvan I. Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia. Ann Neurol. 2003;54:S15-S19. http://www.ncbi.nlm.nih.gov/pubmed/12833363

Dickson DW, Bergeron C, Chin SS, et al. Office of Rare Diseases neuropathologic criteria for corticobasal degeneration. J Neuropathol Exp Neurol. 2002;61:935-946. http://www.ncbi.nlm.nih.gov/pubmed/12430710

Lee VM, Goedert M, Trojanowski JQ. Neurodegenerative tauopathies. Annu Rev Neurosci. 2001;24:121-159. http://www.ncbi.nlm.nih.gov/pubmed/11520930

INTERNET
Pantelyat A. Cortical Basal Syndrome and Corticobasal Degeneration. Medscape. Updated: Dec 04, 2019. https://emedicine.medscape.com/article/1150039-overview
Accessed May 17. 2022.

Corticobasal degeneration (corticobasal syndrome). Mayo Clinic. Feb 22, 2022. Corticobasal degeneration (corticobasal syndrome) – Diagnosis and treatment – Mayo Clinic. Accessed May 17, 2022.

Corticobasal Syndrome. Corticobasal Syndrome | Memory and Aging Center (ucsf.edu). Accessed May 17, 2022.

Corticobasal degeneration, also called corticobasal syndrome, is a rare condition in which areas of your brain shrink and your nerve cells degenerate and die over time. The disease affects the area of the brain that processes information and brain structures that control movement. This degeneration results in growing difficulty in movement on one or both sides of your body.

The condition may cause you to have poor coordination, stiffness, difficulty thinking, trouble with speech or language, or other problems.

Products & Services

Book: Mayo Clinic Family Health Book, 5th Edition
Newsletter: Mayo Clinic Health Letter — Digital Edition

Show more products from Mayo Clinic

Symptoms

Signs and symptoms of corticobasal degeneration (corticobasal syndrome) include:

Difficulty moving on one or both sides of the body, which gets worse over time
Poor coordination
Trouble with balance
Stiffness
Abnormal postures of the hands or feet, such as a hand forming a clenched fist
Muscle jerks
Difficulty swallowing
Abnormal eye movements
Trouble with thinking and language skills
Speech problems, such as slow and halting speech
Difficulty swallowing

Corticobasal degeneration progresses over six to eight years. Eventually, people with corticobasal degeneration lose the ability to walk.

Request an Appointment at Mayo Clinic

Causes

Corticobasal degeneration (corticobasal syndrome) can be caused by several underlying pathologies. Most commonly, corticobasal degeneration is characterized by a buildup of tau in brain cells, which may lead to their deterioration and the symptoms of corticobasal degeneration. Half of the people who have signs and symptoms of corticobasal degeneration have corticobasal degeneration. The second most common cause of corticobasal degeneration is atypical Alzheimer's disease.

Other causes include progressive supranuclear palsy, Pick's disease and Creutzfeldt-Jakob disease.

Complications

The symptoms of corticobasal degeneration (corticobasal syndrome) progress to serious complications, such as pneumonia, blood clots in the lungs, or sepsis, a life-threatening response to an infection. Corticobasal degeneration complications ultimately lead to death.

By Mayo Clinic Staff

Corticobasal degeneration (corticobasal syndrome) care at Mayo Clinic

Request an Appointment at Mayo Clinic

Diagnosis & treatment

Feb. 22, 2022

Share on: FacebookTwitter

Show references

Factor SA, et al. Corticobasal degeneration. https://www.uptodate.com/contents/search. Accessed Oct. 22, 2019.
Braswell Pickering EA. Allscripts EPSi. Mayo Clinic. July 1, 2021.
Corticobasal degeneration information page. National Institute of Neurological Disorders and Stroke. https://www.ninds.nih.gov/Disorders/All-Disorders/Corticobasal-Degeneration-Information-Page. Accessed Oct. 22, 2019.
Daroff RB, et al. Alzheimer's disease and other dementias. In: Bradley's Neurology in Clinical Practice. 7th ed. Saunders Elsevier; 2016. https://www.clinicalkey.com. Accessed Oct. 22, 2019.
Budson AE, et al. Corticobasal degeneration. In: Memory Loss, Alzheimer's Disease, and Dementia. 2nd ed. Elsevier; 2016. https://www.clinicalkey.com. Accessed Oct. 22, 2019.
Nardone R, et al. Pathophysiology of corticobasal degeneration: Insights from neurophysiological studies. Journal of Clinical Neuroscience. 2019; doi:10.1016/j.jocn.2018.10.027.
Di Stasio F, et al. Corticobasal syndrome: Neuroimaging and neurophysiological advances. European Journal of Neurology. 2019; doi:10.1111/ene.13928.
Ali F, et al. Corticobasal degeneration: Key emerging issues. Journal of Neurology. 2018; doi:10.1007/s00415-017-8644-3.
Greene P, et al. Progressive supranuclear palsy, corticobasal degeneration and multiple system atrophy. Continuum: Lifelong Learning in Neurology. 2019; doi:10.1212/CON.0000000000000751.
Corticobasal degeneration. Genetic and Rare Diseases Information Center. https://rarediseases.info.nih.gov/diseases/46/corticobasal-degeneration. Accessed Oct. 22, 2019.
Alzheimer's disease research centers. National Institute on Aging. https://www.nia.nih.gov/health/alzheimers-disease-research-centers. Accessed Oct. 22, 2019.
Graff-Radford J (expert opinion). Mayo Clinic. Nov. 4, 2021.