Cerebellar degeneration

• Article
• 2021-03-05

Cerebellar degeneration is a process by which neurons (nerve cells) in the cerebellum - the area of the brain that controls coordination and balance - deteriorate and die. Diseases that cause cerebellar degeneration can also involve other parts of the central nervous system, including the spinal cord, medulla oblongata, cerebral cortex, and brain stem. Cerebellar degeneration can result from hereditary genetic mutations that alter the normal production of specific proteins necessary for neurons to survive. In some cases, the disease is aqauired (not hereditary or genetic).

The most characteristic symptom of cerebellar degeneration is a wide, unstable, meandering walk, often accompanied by a back and forth tremor in the trunk of the body. Other symptoms may include slow, unstable and jerky movements of the arms or legs, slow and slurred speech, and nystagmus - rapid, small movements of the eyes.

Associated Diseases: Diseases specific to the brain, as well as diseases that occur in other parts of the body, can cause neurons in the cerebellum to die. Neurological conditions with cerebellar degeneration include:

• ischemic or hemorrhagic stroke, when there is no blood flow or oxygen to the cerebellum
• cerebellar cortical atrophy, multi-system atrophy and olivopontocerebellar degeneration, progressive degenerative conditions in which cerebellar degeneration is an important feature
• Friedreich's ataxia and other spinocerebellar ataxias, which are caused by inherited genetic mutations that result in persistent loss of neurons in the cerebellum, brainstem, and spinal cord
• transmissible spongiform encephalopathies (such as Creutzfeldt-Jakob disease) in which abnormal proteins cause inflammation in the brain, including the cerebellum
• multiple sclerosis, in which damage to the insulating membrane (myelin) that wraps around and protects nerve cells, can involve the cerebellum

  Acquired diseases that can cause cerebellar degeneration include:

• chronic alcohol abuse that leads to temporary or permanent cerebellar damage

• paraneoplastic conditions, in which a malignancy (cancer) in other parts of the body produces substances that cause cells of the immune system to attack neurons in the cerebellum

Therapy

There is no cure for hereditary forms of cerebellar degeneration. Treatment is usually supportive and is based on the person's symptoms. For example, medications may be prescribed to reduce gait abnormalities. Physical therapy can strengthen muscles. Other conditions that can contribute to the cerebellar degeneration may have treatment options that relieve symptoms.

Prognosis

The long-term outlook for people with cerebellar degeneration varies depending on the underlying cause.

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