Ataxia Causes – Diseases and Conditions – Mayo Clinic

Posted: Published on October 26th, 2014

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Damage, degeneration or loss of nerve cells in the part of your brain that controls muscle coordination (cerebellum), results in ataxia. Your cerebellum comprises two pingpong-ball-sized portions of folded tissue situated at the base of your brain near your brainstem. The right side of your cerebellum controls coordination on the right side of your body; the left side of your cerebellum controls coordination on the left.

Diseases that damage the spinal cord and peripheral nerves that connect your cerebellum to your muscles also may cause ataxia. Ataxia causes include:

For some adults who develop sporadic ataxia, no specific cause can be found. This is known as sporadic degenerative ataxia, which can take a number of forms, including multiple system atrophy, a progressive, degenerative disorder.

Some types of ataxia and some conditions that cause ataxia are hereditary. If you have one of these conditions, you were born with a defect in a certain gene that makes abnormal proteins. The abnormal proteins hamper the function of nerve cells, primarily in your cerebellum and spinal cord, and cause them to degenerate. As the disease progresses, coordination problems worsen.

You can inherit a genetic ataxia from either a dominant gene from one parent (autosomal dominant disorder) or a recessive gene from each parent (autosomal recessive disorder). In the latter case, it's possible neither parent has the disorder (silent mutation), so there may be no obvious family history.

Different gene defects cause different types of ataxia, most of which are progressive. Each type causes poor coordination, but each has specific signs and symptoms.

These include:

Episodic ataxia. There are seven recognized types of ataxia that are episodic rather than progressive EA1 through EA7. EA1 and EA2 are the most common. EA1 involves brief ataxic episodes that may last seconds or minutes. The episodes are triggered by stress, being startled or sudden movement, and often are associated with muscle twitching.

EA2 involves longer episodes, usually lasting from 30 minutes to six hours, that also are triggered by stress. With this type of ataxia, you may experience dizziness (vertigo), fatigue and muscle weakness during your episodes. In some cases of episodic ataxia, symptoms resolve in later life.

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Ataxia Causes - Diseases and Conditions - Mayo Clinic

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