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Genetic and Rare Diseases Information Center (GARD)

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Cerebellar degeneration


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Your Question

My mom has had cerebellar degeneration for several years, yet I still know very little about it. Can you provide me with some information?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is cerebellar degeneration?

Cerebellar degeneration is a disease process in which neurons in the cerebellum (the area of the brain that controls muscle coordination and balance) deteriorate and die.[1] Cerebellar degeneration does not constitute a specific diagnosis, but rather is used to describe the changes that occur in a person's nervous system.[2] Diseases that cause cerebellar degeneration can also involve areas of the brain that connect the cerebellum to the spinal cord, such as the medulla oblongata, the cerebral cortex, and the brain stem. Cerebellar degeneration has many different causes, but is most often the result of inherited genetic mutations.[1][2]
Last updated: 3/28/2011

What diseases have been associated with cerebellar degeneration?

Diseases that are specific to the brain, as well as diseases that occur in other parts of the body, can cause neurons to die in the cerebellum. Neurological diseases that feature cerebellar degeneration include:[1]

 

Other diseases that can cause cerebellar degeneration include:[1]

  • endocrine diseases that involve the thyroid or the pituitary gland
  • chronic alcohol abuse that leads to temporary or permanent cerebellar damage
  • paraneoplastic disorders in which tumors in other parts of the body produce substances that cause immune system cells to attack neurons in the cerebellum
Last updated: 10/2/2008

What are the signs and symptoms of cerebellar degeneration?

The most characteristic symptom of cerebellar degeneration is a wide-legged, unsteady, lurching walk, usually accompanied by a back and forth tremor in the trunk of the body. Other symptoms include slow, unsteady and jerky movement of the arms or legs, slowed and slurred speech, and nystagmus (rapid, small movements of the eyes).[1][3] Although cerebellar disorders usually strike adults in middle age, the age of symptomatic onset varies depending on the underlying cause of the degeneration.[2][3]

Studies have shown that many patients with movement disorders caused by damage to the cerebellum also have psychiatric symptoms. These studies suggest that patients with cerebellar diseases may benefit from screening and treatment of psychiatric symptoms.[3]

Last updated: 3/28/2011

How might cerebellar degeneration be treated?

There is currently no cure for the hereditary causes of cerebellar degeneration.[2] Treatment is usually supportive unless the cause is acquired and reversible.[4] In such cases, the underlying condition is treated first.[2] For example, paraneoplastic cerebellar degeneration may improve after successful treatment of the underlying cancer. For alcoholic/nutritional cerebellar degeneration, thiamine is given along with other B vitamins, usually relieving the condition if the patient stops drinking alcohol and resumes a normal diet.[5] A variety of drugs may be used to treat gait and swallowing disorders. Physical therapy can strengthen muscles, while special devices or appliances can assist in walking and other activities of daily life.[2]
Last updated: 3/28/2011

What is the prognosis for individuals with cerebellar degeneration?

The prognosis for individuals with cerebellar degeneration varies depending on the underlying cause.[2]
Last updated: 10/2/2008

References
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.