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Corticobasal Degeneration (CBD)

Author: Judy Zhu

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Introduction
Corticobasal degeneration, also known as corticobasal syndrome, is a rare form of dementia that causes death of neurons in the cerebral cortex and basal ganglia. This leads to problems with movement, vision, coordination, speaking and swallowing, and cognitive abilities. People with CBD may also experience serious complications, like pneumonia, blood clots in the lungs, or sepsis (a dangerous response to an infection). The disease worsens over 6-8 years, and patients may live 5-10 years after symptom onset [1], [2], [3].

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Causes
CBD is most commonly caused by the buildup of tau proteins in brain cells, including neurons and glia [1], [2]. Normally, tau proteins stabilize the internal skeleton of neurons, allowing nutrients and other essential substances to travel within the cell [4]. These proteins are usually broken down before they reach high levels. However, in CBD patients, tau proteins don’t break down properly and accumulate into harmful clumps [2].

 

Diagnosis 
Diagnosing CBD is extremely challenging due to its diverse symptoms affecting motor skills, behaviour, and cognition. Diagnosis involves assessment of symptoms, physical exams, and testing. For example, imaging tests may be needed to detect brain change. Since CBD can cause similar symptoms as Alzheimer’s disease (AD), blood or cerebrospinal fluid tests may be used to detect amyloid and tau proteins, which are markers for AD [5].

Treatments
Currently, there are no treatments to slow down CBD progression, and the disease often resists drug therapy. Available treatments are mostly based on features of other similar diseases due to the lack of research and its low prevalence [6]. According to the NHS, these treatments include:

 

  • Medication – to improve stiffness and muscle spasms, sleep and mood, pain or memory

  • Physiotherapy – to help with movement and balance difficulties

  • Speech and language therapy – to help with communication and swallowing problems

  • Occupational therapy – to improve the skills and abilities needed for daily activities at home

  • Palliative care and advanced care planning [2]

  • In addition to treating behavioural symptoms, addressing psychiatric symptoms, such as depression and anxiety, is also essential for improving the quality of life for CBD patients [6]. 

References:
[1] Corticobasal degeneration (corticobasal syndrome) - Symptoms and causes - Mayo Clinic. (2023, December 22). Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/corticobasal-degeneration/symptoms-causes/syc-20354767
[2] Website, N. (2023, August 22). Corticobasal degeneration. nhs.uk. https://www.nhs.uk/conditions/corticobasal-degeneration/#:~:text=Tau%20occurs%20naturally%20in%20the,family%20members%20is%20very%20low.
[3] Professional, C. C. M. (n.d.). Corticobasal degeneration. Cleveland Clinic. https://my.clevelandclinic.org/health/diseases/22522-corticobasal-degeneration
[4] Alzheimer’s Association. (2021). Tau. https://www.alz.org/media/documents/alzheimers-dementia-tau-ts.pdf
[5] Corticobasal degeneration (corticobasal syndrome) - Diagnosis and treatment - Mayo Clinic. (2023, December 22). https://www.mayoclinic.org/diseases-conditions/corticobasal-degeneration/diagnosis-treatment/drc-20475446
[6] Caixeta, L., Caixeta, V. M., Nogueira, Y. L., & Aversi-Ferreira, T. A. (2020). Pharmacological interventions in corticobasal degeneration: a review. Dementia & neuropsychologia, 14(3), 243–247. https://doi.org/10.1590/1980-57642020dn14-030006
[7] Kobylecki, C., & Mann, D. M. (2016). Presymptomatic anterior frontal involvement in corticobasal degeneration. Brain, 139(12), 3059–3062. https://doi.org/10.1093/brain/aww267

 

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