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Corticobasal degeneration is a disease that is often misdiagnosed as dementia or Alzheimer's. Read on to find out more about this rare and puzzling brain disorder.

Corticobasal degeneration is a progressive brain disorder characterized by nerve cell loss and shrinking in many areas of the brain. These portions of the brain can include the basal ganglia and cerebral cortex. The disease starts gradually, and usually begins at or around the age of 60. It can also be called “corticobasal ganglionic degeneration” and it is part of the “pick complex” of neurodegenerative diseases because it shares similarities with frontotemporal dementia.

CBD's History And Background

Corticobasal degeneration was first identified in 1968 by Rebeiz and his research associates. Initially, the researchers referred to this neurodegenerative disease as “corticodentatonigral degeneration with neuronal achromasia.” Although the precise cause of CBD is not currently known, the disease causes serious damage to the basal ganglia. Much of the recent advancements in the understanding of the disease comes within the past decade, due to it being formally recognized by medical science.

Corticobasal Degeneration: What Are The Signs And Symptoms?

People who are suffering from corticobasal degeneration will usually have a movement disorder or cognitive deficits. As the disease gets worse, most will have both types of symptoms and often with a delay of onset for about two to three years. The movement issues associated with the disease can mimic Parkinson’s disease (PD). However, unlike Parkinson’s disease, people with CBD will typically not improve with medications and many of the symptoms are not found in those with Parkinson’s. A key feature of movement issues in corticobasal degeneration is muscle rigidity, though tremors are not as common. Most often the symptoms will start in one hand, leg or arm.

Many people with CBD will complain of subtle changes in feeling or have an inability to make the affected limb or limbs follow a command. This deficit is called apraxia and it may be confused with weakness or clumsiness. There could also be trouble with doing specific tasks such as opening a door or brushing one’s hair. 

When CBD affects an individual’s leg, it could result in having problems with complex movements such as dancing or the person may trip and fall down frequently. An affected limb can also suffer from involuntary contraction, twisting or it can become stiff. 

Uncontrolled jumping of the limbs when it is gently tapped is called myoclonus. Lastly, individuals will often complain the affected limb doesn’t feel like a part of their body, it’s a phenomena called “alien limb.” Sometimes it will raise up on its own, with the person having no control. 

Cognitive Disabilities

Those with CBD who present with cognitive difficulties are often misdiagnosed with some form of dementia or Alzheimer’s disease. It’s only after developing movement issues that most people will receive a proper diagnosis. Occasionally, a diagnosis of corticobasal degeneration isn’t made until after a person’s brain is examined during an autopsy and it has ballooned neurons, neuronal inclusions and other abnormalities from deposits of the tau protein.

Another issue associated with this disease is language difficulties. This is most commonly witnessed as having difficulty with the expression of language, such as finding the “right” word or naming something correctly. Solving mathematical equations, reading and writing can also be impaired. It’s very common to see personality changes, repetitive or compulsive behaviors, and inappropriate behaviors in those with CBD.

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