Correction of foot deformity by the Ilizarov method in a patient with Segawa disease

Hereditary progressive dystonia, or Segawa disease, is rare. Diagnosis depends on typical clinical features with normal laboratory findings. It responds well to levodopa treatment. This article contains a case report of a patient with Segawa disease with a fixed equinovarus foot. A 21-year-old woman was diagnosed with Segawa disease since she was 8 years old; she became wheelchair dependent at the age of 15. The dystonia responded well to levodopa, except for the fixed-foot deformity. The deformity was corrected successfully by the Ilizarov method. The patient returned to independent ambulation after surgery. Because similar types of foot deformities appeared in several progressive degenerative neurologic diseases, the treatable Segawa disease should be added to the list of differential diagnoses of progressive degenerative neurologic diseases with talipes equinovarus.