There are conditions associated with Friedreich's Ataxia that do not result from the degeneration of nerves. Heart disease, sometimes in forms severe enough to be fatal, is one of the more common and threatening of these conditions. Abnormalities in heartbeat rhythm and diminished strength of the heart muscle have been noted in a large percentage of Friedreich's Ataxia patients, with palpitations and dyspnea (shortness of breath) the most common found symptoms.
Diabetes mellitus, characterised by abnormally high blood and urinary sugar levels, is another condition that may attend Friedreich's Ataxia. Most Friedreich's Ataxia patients who becomes diabetic will do so late in the course of the disease. The nature of the relationship between Friedreich's Ataxia and diabetes has not yet been determined.

In most cases, the disease is slowly progressive. Scientists have described the progression of Friedreich's Ataxia in terms of two stages: ambulatory, when the patient is able to walk, and nonambulatory, when the patient requires a wheelchair.

The early ambulatory period is characterised by a decrease in or absence of muscle reflexes in addition to an unsteady gait. Elevated heels and flexed toes produce a foot deformity referred to as Friedreich's foot. Mild scoliosis (curvature of the spine) also develops during the period. A diminished sense of touch in arms and legs may occur. In the late ambulatory period, there is great difficulty in walking and a tendency to trip or fall. Use of a cane or other orthopedic aid is usually required. The hands and arms become affected, making writing and other manipulatory tasks difficult. Breakdowns in vocal articulation, irregularities in pitch and loudness, and other changes in voice quality such as speech muscle control. Scoliosis, if severe may cause respiratory problems. There may also be a mild horizontal oscillation of the eyeball, as eye muscles weaken.

The nonambulatory stage begins when the loss of muscle control in the legs has advanced to such a point as to require the constant use of a wheelchair. This usually happens in the third decade of life. The ataxia in the arms, head, and neck continue to worsen during the non ambulatory stage and it becomes increasingly difficult to sit upright. There is little if any movement in lower limbs by the late period of this stage, and upper limbs become very ataxic.

Although there is no known cure for Friedreich's Ataxia, many of the problems imposed by the disease can be treated. Orthopedic intervention, which may include surgery, can alleviate scoliosis, and orthopedic appliances and physical therapy can help prolong ambulation. Heart problems can also be successfully treated for many years with medication. Insulin therapy is effective in controlling diabetes mellitus.

Genetic counseling is also available to discuss pregnancy options.

Scientists are conducting research into the biochemistry, neurobiology, and biophysics of central and peripheral nerves, heart and skeletal muscle, and the interactions between muscle and nerve to uncover the pathological process of Friedreich's Ataxia. In addition, in-depth studies of diabetes and heart disease are being conducted to determine their relationship with this neurological disorder.

Many investigators have also been focussing their efforts on the possibility that an abnormal enzyme initiates the disease process. One MDA-sponsored scientist has identified a deficiency of mitochondrial malic enzyme in Friedreich's Ataxia patients as a possible cause of the disease. This enzyme is involved in the breakdown of carbohydrates and proteins necessary for the production of cellular energy. In healthy people, mitochondrial malic enzyme activity is highest in the nervous system and heart. Although further studies are needed to substantiate current findings, mitochondrial malic enzyme deficiency could explain the major involvement of the nervous system and heart in Friedreich's Ataxia.