Corticobasal Degeneration
What is Corticobasal Degeneration?
Corticobasal Degeneration (CBD) is a progressive rare neurodegenerative disease that involves the degeneration of brain cells and certain parts of the brain. Due to a loss of nerve cells, specific lobes in the brain begin to atrophy (waste away). While the progression and severity varies based on the part of the brain that is affected, most affected individuals experience progressive stiffness, lack of coordination, clumsiness, difficulty swallowing, and muscle spasms. Other than motor abnormalities, some cognitive impairments accompany CBD. These include trouble with speech/language and loss of thinking/memory. CBD generally presents its symptoms between the ages of 50 to 70, and men and women are both equally affected by the condition. CBD affects around 5 in every 100,000 people. However, many cases of CBD are misdiagnosed as other neurodegenerative conditions since they have many of the same symptoms. Only through neuropathologic examinations can doctors diagnose CBD accurately. While the cause of CBD is largely unknown, recent studies suggest that the development of CBD can be attributed to a buildup of the tau protein in the brain. The function of tau protein in the brain is primarily to aid with synaptic plasticity, axonal transport, and neurite outgrowth (the growing of projections as developing neurons grow). Although there is no specific cure for CBD currently, a multidisciplinary treatment plan can alleviate affected individuals’ symptoms.
Synonyms
- Cortical Basal Ganglionic Degeneration
Corticobasal Degeneration (CBD) is a progressive rare neurodegenerative disease that involves the degeneration of brain cells and certain parts of the brain. Due to a loss of nerve cells, specific lobes in the brain begin to atrophy (waste away). While the progression and severity varies based on the part of the brain that is affected, most affected individuals experience progressive stiffness, lack of coordination, clumsiness, difficulty swallowing, and muscle spasms. Other than motor abnormalities, some cognitive impairments accompany CBD. These include trouble with speech/language and loss of thinking/memory. CBD generally presents its symptoms between the ages of 50 to 70, and men and women are both equally affected by the condition. CBD affects around 5 in every 100,000 people. However, many cases of CBD are misdiagnosed as other neurodegenerative conditions since they have many of the same symptoms. Only through neuropathologic examinations can doctors diagnose CBD accurately. While the cause of CBD is largely unknown, recent studies suggest that the development of CBD can be attributed to a buildup of the tau protein in the brain. The function of tau protein in the brain is primarily to aid with synaptic plasticity, axonal transport, and neurite outgrowth (the growing of projections as developing neurons grow). Although there is no specific cure for CBD currently, a multidisciplinary treatment plan can alleviate affected individuals’ symptoms.
Many cases of CBD go either undiagnosed or misdiagnosed, causing discussion about prevalence to be a general estimation. It is known that it affects both men and women equally, but there have been slightly more cases in women. CBD affects around 5 in every 100,000 people. Generally, symptoms associated with CBD present between the ages of 50 to 70.
| Name | Abbreviation |
|---|---|
| Cortical Basal Ganglionic Degeneration | CBGD |
While the exact cause of CBD is unknown, researchers have some ideas of factors that may contribute to the development of the condition. Most of these factors are related to aging since they involve both genetic and environmental factors. Research suggests that a possible cause of CBD could be a buildup in a protein called tau. While tau is essential for normal brain function and plays a vital role in aiding nerve cells, a buildup of tau causes some problems in the nervous system (particularly in the brain). The abnormal accumulation of tau causes the deterioration of brain cells. The reason for this buildup of tau in the brain as CBD progresses is unknown. In fact, tau has been found to play a role in other neurodegenerative brain disorders such as Frontotemporal Dementia and Alzheimer’s Disease. Therefore, this group of conditions have been referred to as “tauopathies.”
The symptoms of CBD progress over many years (usually around 6 to 8 years). While symptoms for CBD can vary depending on the person, generally, affected individuals experience progressive stiffness, referred to as progressive asymmetric rigidity. In addition, people with CBD may have trouble with coordination and balance. In a few cases, affected individuals may not be able to control their muscle movement or they may be unaware of muscle movement. When this occurs, it typically starts on one side of the body and spreads to the other side. Also, affected individuals may experience muscle spasms and jerks (called myoclonus). Other symptoms of CBD include difficulty swallowing and abnormal postures/orientation of limbs.
Before movement abnormalities present themselves, cognitive and behavioral abnormalities may be noticed. For example, affected individuals may have trouble with speech/language and may develop a form of progressive aphasia. In addition, loss of thinking and memory frequently accompany the difficulties with speech.
CBD can make affected individuals more susceptible to developing serious complications such as pneumonia and sepsis (life-threatening and extreme response to an infection that could lead to organ failure).
CBD is typically diagnosed after physicians notice progressive neurologic symptoms without a stroke occuring or tumor present. Generally, it is difficult to distinguish CBD from other neurodegenerative diseases since it involves the same parts of the nervous system and presents itself in similar ways. However, there are some tests that medical professionals can conduct during a detailed neurological exam in order to diagnose an individual with CBD.
Tests to confirm CBD diagnosis include:
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Magnetic Resonance Imaging (MRI) → helps produce a detailed image of brain tissue through the use of radio waves and magnetic fields, allowing doctors to eliminate other neurodegenerative conditions that have similar symptoms as CBD
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Computerized Tomography Scanning (CT scan) → uses X-rays to display cross-sectional images of the brain
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Neuropathologic examinations → doctors take small biopsies from spinal cord and brain and examine it in order to accurately diagnose CBD
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Based on the quantity and the location of the accumulated tau protein in the brain, doctors can rule out other similar conditions
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Doctor may advise other tests in order to eliminate the possibility of other conditions that present themselves in similar ways
Although there is no specific cure for CBD, most treatment available is targeted at alleviating symptoms caused by CBD. Individuals affected by CBD can be prescribed drugs such as Levodopa, which is generally used as a treatment for Parkinson’s disease, that helps with the stiffness and slowness that they face. Other drugs include medications to treat sudden muscle jerks/spasms (myoclonus). Botox has been used to help treat muscle contractures (fixed tightening of muscles) and pain associated with CBD. Therapy including physical and speech therapy can also be useful in ensuring that the patient can carry out everyday tasks and maintain mobility. However, affected individuals are likely to require a cane or support for walking and some may be bed-ridden.
The rate of CBD progression varies across individuals and is difficult to predict. Survival ranges, but on average, individuals live up to 7 years after the presentation of their first symptoms. Since CBD can often be accompanied by other life-threatening illnesses, these related illnesses may shorten lives. The most common cause of death is Aspiration Pneumonia. However, despite the fact that there is no cure, if given a multidisciplinary treatment plan, affected individuals can alleviate some of their symptoms and lead an expanded lifestyle.
Last updated 12 Mar 2021, 04:02 AM
12 Mar 2021, 04:02 AM
Hi everyone,
The Corticobasal Degeneration community details have been updated. We added more information about the cause, prevalence, symptoms, diagnosis, and treatment. Hopefully, you find it helpful.
Thanks!
Last updated 13 Sep 2016, 11:27 PM
13 Sep 2016, 11:27 PM
Hi , never been a member of a forum before so here goes,,,my wife Janice (55) is now in a hospital bed at home and in the latter stages of Corticobasal degeneration...6 weeks ago we were going for runs in the car and for dinner etc,although I had to physically feed her etc..then she got constipated after discharging more than ample faeces ...along with her 6 monthly fall ...she ended up in hospital to clear the constipation and on getting X rayed there was no damage to her after the fall...she now is totally helpless at home getting liquidised food and incontinent ....all in the space of 6 weeks...carers 4 times a day ...my main worry at the moment is that she maybe in pain as for about 6-7 hours every day she looks like she's possessed by a demon and dizapam etc only relief it briefly...she's aggressive and tries to hit out then out of the blue she calms down and becomes placid and loving ...I'd like to ask has anyone on here ever experienced the likes of this with their loved one...thanks for reading this Garry
Last updated 6 Nov 2012, 03:18 AM
6 Nov 2012, 03:18 AM
i have had cbdg diagnosed 1 year ago, i have had physical therapy, it does not work, unless you and the patient are100^ committed. i am not that committed someone suggested i start on longvida, fish oil, vit d, c, b, ans o2q10. if you really want the dose let me know
17 Dec 2010, 12:56 PM
Hello everyone My father-in-law was diagnosed with CBD in July after a few years of experiencing symptoms that puzzled all of his doctors. What stage are your loved ones in and how fast has this disease progressed for them? One of his arm's started jerking a few years ago, followed by loss of peripheral vision. A year and a half ago he had to stop driving because he could not keep the car in the proper lane. Now he can't read because he can't track the lines, he can't put his clothes on properly, he must use a walker because his sense of balance is very bad, and he has trouble using everyday items. He says he knows what he should do with them but he can't make his hands do what they should do. He is also cold almost all of the time. Does anyone have any suggestions or practical tips? Have you tried occupational therapy? Thanks, Rosiemama
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Created by RareshareTeam | Last updated 12 Mar 2021, 04:02 AM
Progression of CBD
Created by Rosiemama | Last updated 6 Nov 2012, 03:18 AM
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