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Hereditary ataxia

Ataxia (from Greek ataxiā, meaning failure to put in order) is unsteady and clumsy motion of the limbs or trunk due to a failure of the gross coordination of muscle movements. more...

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Ataxia often occurs when parts of the nervous system that control movement are damaged. People with ataxia experience a failure of muscle control in their arms and legs, resulting in a lack of balance and coordination or a disturbance of gait. While the term ataxia is primarily used to describe this set of symptoms, it is sometimes also used to refer to a family of disorders. It is not, however, a specific diagnosis.

Most disorders that result in ataxia cause cells in the part of the brain called the cerebellum to degenerate, or atrophy. Sometimes the spine is also affected. The phrases cerebellar degeneration and spinocerebellar degeneration are used to describe changes that have taken place in a person’s nervous system; neither term constitutes a specific diagnosis. Cerebellar and spinocerebellar degeneration have many different causes. The age of onset of the resulting ataxia varies depending on the underlying cause of the degeneration.

Many ataxias are hereditary and are classified by chromosomal location and pattern of inheritance: autosomal dominant, in which the affected person inherits a normal gene from one parent and a faulty gene from the other parent; and autosomal recessive, in which both parents pass on a copy of the faulty gene. Among the more common inherited ataxias are Friedreich’s ataxia and Machado-Joseph disease. Sporadic ataxias can also occur in families with no prior history.

Ataxia can also be acquired. Conditions that can cause acquired ataxia include stroke, multiple sclerosis, tumors, lesions of the central nervous system or spinal cord, alcoholism, peripheral neuropathy, metabolic disorders, and vitamin deficiencies.

Dysdiadochokinesia is a sign of cerebellar ataxia.

Ataxia is also the name of a band featuring John Frusciante (of the Red Hot Chili Peppers), Joe Lally (of Fugazzi), and Josh Klinghoffer. Frusciante plays synthesizer, guitar, and vocals; Lally plays bass; Klinghoffer plays percussion.

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Ataxia
From Gale Encyclopedia of Childhood and Adolescence, 4/6/01

Ataxia, an extreme lack of coordination of the muscles, is a symptom of damage to the central nervous system. People with ataxia typically stand with feet planted far apart, and sway while standing, struggling to maintain balance. Jerky eye and head movements are also commonly observed with ataxia. Ataxia may result from a number of causes, including damage to or diseases of the spinal cord or the cerebellum, a part of the brain; tumor on the spinal cord or in the cerebellum; infection affecting the central nervous system; response to poisons and toxins or other environmental factor that causes the central nervous system to stop functioning normally or to degenerate; disease that causes degeneration of the central nervous system, such as multiple sclerosis; or hereditary factors. Hereditary ataxia conditions are rare, and include Friedreich's ataxia, which usually appears between the ages of five and twenty. Friedreich's ataxia is a disabling condition that often causes premature death.

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For Your Information

  • Gale Encyclopedia of Childhood & Adolescence. Gale Research, 1998.

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