Friedreich’s Ataxia

Friedreich’s ataxia is an inherited disease, with the patient usually affected in childhood. The disease generally sets in between the child’s sixth and tenth years. Onset is gradual, with foot deformity and ataxia (an inability to appreciate the normal sensation of space). Awkwardness of walking is the typical symptom, with a tendency to stumble and fall easily. This may even date from infancy. The walk becomes clumsy and irregular, short steps are taken and the trunk tends to sway unsteadily from side to side. When walking each foot is raised in a poorly coordinated manner. Gradually this tendency spreads to the upper limbs.

As the condition advances, irregular movements of the head and neck follow, with the upper part of the body moving with jerky, nodding motions. Irregular eye movements occur, also difficulties in speech and word correlation. There may be wasting of the muscles of the hands and feet however, sensation appreciation is often little affected.

The course is a slow and irregular one, but the average duration of the disease is more than 30 years. Concurrent infections do not help, and may finally cause the patient’s death, rather than the disease itself.

There is no known treatment of any benefit. Training of the limbs by physiotherapy may assist, and orthopedic shoes can assist the deformities of the feet.