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Definition

Friedreich's ataxia is a rare disease passed down through families (inherited) that affects the muscles and heart.

Symptoms

Symptoms are caused by the wearing away of structures in areas of the brain and spinal cord that control coordination, muscle movement, and some sensory functions. Symptoms generally begin in childhood before puberty, and may include:

* Abnormal speech
* Changes in vision, particularly color vision
* Decrease in ability to feel vibrations in lower limbs
* Foot problems, such as hammer toe and high arches
* Hearing loss -- occurs in about 10% of patients
* Jerky eye movements
* Loss of coordination and balance, which leads to frequent falls
* Muscle weakness
* No reflexes in the legs
* Unsteady gait and uncoordinated movements (ataxia) -- gets worse with time

Muscle problems lead to changes in the spine, which may result in scoliosis or kyphoscoliosis.

Heart disease usually develops and may lead to heart failure. Death may result from heart failure or dysrhythmias that do not respond to treatment. Diabetes may develop in later stages of the disease.