are Ataxias and Cerebellar or Spinocerebellar Degeneration?
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
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, alcoholism, peripheral neuropathy,
metabolic disorders, and vitamin deficiencies.
there any treatment?
is no cure for the hereditary ataxias. If the ataxia is caused by another
condition, that underlying condition is treated first. For example, ataxia
caused by a metabolic disorder may be treated with medications and a controlled
diet. Vitamin deficiency is treated with vitamin therapy. 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.
is the prognosis?
prognosis for individuals with ataxia and cerebellar/spinocerebellar
degeneration varies depending on its underlying cause.
research is being done?
NINDS supports and conducts a broad range of basic and clinical research on
cerebellar and spinocerebellar degeneration, including work aimed at finding
the cause(s) of ataxias and ways to treat, cure, and, ultimately, prevent them.
Scientists are optimistic that understanding the genetics of these disorders
may lead to breakthroughs in treatment.
NINDS health-related material is
provided for information purposes only and does not necessarily represent
endorsement by or an official position of the National Institute of
Neurological Disorders and Stroke or any other Federal agency. Advice on the
treatment or care of an individual patient should be obtained through
consultation with a physician who has examined that patient or is familiar with
that patient's medical history.
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