Corticobasal degeneration (corticobasal syndrome)

Overview

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.

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.

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.

Diagnosis

A diagnosis of corticobasal degeneration (corticobasal syndrome) is made based on your signs and symptoms. However, your signs and symptoms could be due to another degenerative disease such as progressive supranuclear palsy, Alzheimer's disease, Pick's disease and Creutzfeldt-Jakob disease. You may need an imaging test, such as a CT scan or MRI, to rule out these other conditions. Sometimes, these tests are performed every few months to look for abnormal changes in the brain.

Researchers are looking at whether positron emission tomography (PET) and single-photon emission computerized tomography (SPECT) scans can uncover changes in the brain that are consistent with corticobasal degeneration. However, more research needs to be done in this area.

Treatment

There are no treatments that help slow the progression of corticobasal degeneration (corticobasal syndrome). Your doctor may recommend medications to try to manage your symptoms.

Getting occupational and physical therapy may help you manage the disabilities caused by corticobasal degeneration. Walking devices may help with mobility and prevent falls. Speech therapy can help with communication and swallowing problems. A dietitian may be helpful in ensuring the proper nutrition and reducing the risk of inhaling food into the lungs (aspiration).

Preparing for an appointment

You may start by seeing your primary care provider. Or, you may be referred immediately to a specialist, such as a neurologist.

Here's some information to help you get ready for your appointment.

What you can do

When you make the appointment, ask if there's anything you need to do in advance, such as fasting before having a specific test. Make a list of:

  • Your symptoms, including any that seem unrelated to the reason for your appointment
  • Key personal information, including major stresses, recent life changes and family medical history
  • All medications, vitamins or other supplements you take, including the doses
  • Questions to ask your doctor

Take a family member or friend along, if possible, to help you remember the information you're given.

For corticobasal degeneration, some basic questions to ask your doctor include:

  • What's likely causing my symptoms?
  • Other than the most likely cause, what are other possible causes for my symptoms?
  • What tests do I need?
  • Is my condition likely temporary or chronic?
  • What's the best course of action?
  • What are the alternatives to the primary approach you're suggesting?
  • I have these other health conditions. How can I best manage them together?
  • Are there restrictions I need to follow?
  • Should I see a specialist?
  • Are there brochures or other printed material I can have? What websites do you recommend?

Don't hesitate to ask other questions.

What to expect from your doctor

Your doctor is likely to ask you several questions, such as:

  • When did your symptoms begin?
  • Have your symptoms been continuous or occasional?
  • How severe are your symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?

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