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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Cerebellar degeneration


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Overview



What is cerebellar degeneration?

What are the signs and symptoms of cerebellar degeneration?


How might cerebellar degeneration be treated?



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 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

References
  1. NINDS Cerebellar Degeneration Information Page. National Institute of Neurological Disorders and Stroke (NINDS). June 27, 2008; http://www.ninds.nih.gov/disorders/cerebellar_degeneration/cerebellar_degeneration.htm. Accessed 10/2/2008.
  2. NINDS Ataxias and Cerebellar or Spinocerebellar Degeneration Information Page. National Institute of Neurological Disorders and Stroke (NINDS). June 3, 2008; http://www.ninds.nih.gov/disorders/ataxia/ataxia.htm. Accessed 10/2/2008.
  3. Study Finds Psychiatric Disorders are Common in People with Cerebellar Degeneration. National Institute of Neurological Disorders and Stroke (NINDS). March 8, 2005; http://www.ninds.nih.gov/news_and_events/news_articles/news_article_cerebellar.htm. Accessed 10/2/2008.
  4. Cerebellar Disorders. The Merck Manuals Online Medical Library. August 2007; http://www.merck.com/mmpe/sec16/ch221/ch221j.html. Accessed 10/2/2008.
  5. Cerebellar Degeneration, Subacute. NORD. 2007; http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Cerebellar%20Degeneration%2C%20Subacute. Accessed 3/28/2011.