Incidence, Natural History, and Treatment of Scoliosis in Friedreichʼs Ataxia

We carried out a retrospective study of 42 patients with documented Friedreich's ataxia to answer four questions: what is the incidence and natural history of scoliosis in Friedreich's ataxia, and what are the results of operative and nonoperative treatment? We concluded that the incidence of scoliosis in true Friedreich's ataxia is extremely high. The curves tend to progress with the severity of the disease and often tend to progress after skeletal maturity. Bracing and electrospinal instrumentation have no role in treatment. Patients with progressive curves should have early surgical stabilization.