Cerebellar Degeneration

What is cerebellar degeneration?

Cerebellar degeneration is deterioration (damage or death) of the nerve cells in your cerebellum. Your cerebellum is in the back part of your brain. It controls many important functions. These include coordination in balance, standing, walking, speech and muscle movements.

Cerebellar degeneration can be the result of:

• Alcohol use disorder or nutritional deficiencies.
• Neurodegenerative disorders.
• Paraneoplastic syndromes (immune responses to cancerous tumors).

Who’s at risk for cerebellar degeneration?

Risk factors for cerebellar degeneration vary according to the cause. For example, paraneoplastic cerebellar degeneration tends to be more common in women over 50. But overall, men tend to have more problems with balance and coordination as they get older.

Alcohol or nutrition-related cerebellar degeneration usually happens around middle age. It’s common in people who have a history of alcohol use disorder.

Cerebellar degeneration can also occur in children who have certain inherited disorders.

How common is cerebellar degeneration?

Less than 1% of people with cancer develop paraneoplastic cerebellar degeneration. But alcohol-related cerebellar degeneration is more common.

What causes cerebellar degeneration?

There are a variety of conditions that can cause cerebellar degeneration, including:

• Alcohol use disroder: Alcohol use disorder can lead to a thiamine (vitamin B1) deficiency. This disrupts your brain function and damages cells. People with alcohol use disorder may not get enough thiamine in their diets. Or, their bodies may not be able to absorb thiamine.
• Neurodegenerative disorders: Some disorders cause nerve degeneration in your spinal cord and brain, including your cerebellum. These diseases include ataxia, multiple system atrophy, Creutzfeldt-Jakob disease and multiple sclerosis (MS). Neurodegenerative disorders may be inherited (passed through abnormal genes from parents to children). Or, you may develop the disease during your lifetime.
• Paraneoplastic syndromes: Some people have an autoimmune disorder that causes their bodies to attack healthy brain cells in response to a tumor. Paraneoplastic syndromes are more common in people with certain cancers. These include breast cancer, ovarian cancer, uterine cancer, adult Hodgkin’s lymphoma and small cell lung cancer.

What are the symptoms of cerebellar degeneration?

Symptoms of cerebellar degeneration may include:

• Balance problems, vertigo or dizziness.
• Decreased muscle tone (hypotonia).
• Eye problems, including double vision and involuntary eye movements (nystagmus).
• Poor muscle coordination in your arms or legs (ataxia).
• Problems with motor skills, such as holding your head up or using your limbs.
• Tremors or shaking when performing muscle movements.
• Unsteady or uncoordinated walk with a wide gait.

Additional symptoms that are more common in people with paraneoplastic cerebellar degeneration include:

• Dementia (problems with memory, reasoning and thinking).
• Slow and slurred speech (dysarthria).

People with cerebellar degeneration may also have conditions like:

• Attention-deficit/hyperactivity disorder (ADHD).
• Autism spectrum disorder.
• Schizophrenia.

How is cerebellar degeneration diagnosed?

If your healthcare provider suspects you have cerebellar degeneration, they’ll perform a careful evaluation of your symptoms. They’ll also review your personal and family medical history. Be sure to explain how and to what extent your symptoms are interfering with your daily life.

To confirm a diagnosis, your provider will likely suggest an imaging exam. You might have magnetic resonance imaging (MRI) or a CT (computed tomography) scan. These exams create highly detailed pictures of your brain and its tissues. If you have cerebellar degeneration, an imaging exam may show that your cerebellum has grown smaller or deteriorated. These tests can also detect other brain conditions, such as a brain tumor or evidence of stroke.

Your healthcare provider may test your cerebrospinal fluid (the liquid around your brain and spinal cord). They’ll take a sample with a needle during a spinal tap (lumbar puncture). Substances in the fluid may indicate an autoimmune disorder such as paraneoplastic syndromes.

In some cases, a blood test can detect certain antibodies. These are proteins your immune system produces to fight illnesses. People with cancer and paraneoplastic syndromes may have high levels of antibodies in their blood.

If your healthcare provider thinks you have hereditary cerebellar degeneration, they may do genetic testing.

How is cerebellar degeneration treated?

There isn’t a cure for cerebellar degeneration. Treatment usually depends on the underlying cause of your brain dysfunction. However, medications can sometimes help manage certain symptoms, including tremors or problems with walking and dizziness.

Treatment for paraneoplastic cerebellar degeneration may include:

• Chemotherapy.
• Radiation therapy.
• Immunosuppressants.
• Immunotherapy.
• Different combinations of chemotherapy, radiation therapy and surgery to remove a tumor.

Treatment for alcohol-related cerebellar degeneration may include:

• Not drinking alcohol.
• Dietary changes. This may mean eating more whole grains, lean meat and fish. You might also need foods fortified with thiamin and other B vitamins.
• Thiamine supplements.

People with any form of cerebellar degeneration may need physical, occupational or speech therapy. These therapies can help you improve:

• Motor skills so you can perform daily activities.
• Muscle strength and coordination.
• Swallowing, speaking and language skills.

Can cerebellar degeneration be prevented?

Not drinking alcohol can greatly reduce your risk of alcohol-related cerebellar degeneration. But there aren’t any proven prevention strategies for other forms of the disease.

What’s the prognosis (outlook) for people with cerebellar degeneration?

The prognosis (outlook) for cerebellar degeneration depends on the underlying cause of the disease. Cancer treatment, abstaining from alcohol or adjusting your diet may slow or stop the degeneration. But for other neurodegenerative conditions, the disease will likely worsen over time at various speeds.

People with progressive forms of the disease may develop severe loss of coordination. They might need a wheelchair or an assistive device to move around and prevent falls. They may also lose the ability to live independently if they can’t perform daily tasks or activities. Some become bed-bound.

Children with cerebellar degeneration may have developmental delays.

When should I contact my healthcare provider about cerebellar degeneration?

If you notice any problems with your balance, speech, eyesight or ability to perform everyday activities, contact your healthcare provider right away. Sometimes, people assume these issues are part of the aging process. But they could be signs of a more serious issue.

What questions should I ask my healthcare provider about cerebellar degeneration?

If you or a loved one is living with cerebellar degeneration, you may want to ask your healthcare provider the following questions:

• Are there any lifestyle changes I can make to better cope with the disease?
• How can I find support?
• Should I start physical, occupational or speech therapy?
• What’s the underlying cause of the disease?
• Will the disease progress? If so, how quickly?