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Most disorders that result in ataxia are found to have degeneration, or atrophy, of the cells in the part of the brain called the cerebellum. The cerebellum is located at the back of the head. Its function is to coordinate voluntary muscle movements and to maintain posture, balance, and equilibrium.
The spine can also be affected. The terms cerebellar degeneration and spinocerebellar degeneration may be used to refer to this type of damage to the nervous system.
The various abnormal genes that cause ataxia do have something in common: they make abnormal proteins that affect nerve cells, primarily in the cerebellum and in the spinal cord. They may also affect other parts of the brain.
The affected nerve cells begin to function poorly and ultimately degenerate. As the disease progresses, muscles become less and less responsive to the commands of the brain. This causes balance and coordination to become a greater problem.