Successful treatment of hereditary progressive dystonia--a case report

Hereditary progressive dystonia or Segawa disease is a very rare disease. Diagnosis depends on typical clinical features with remarkably good response to levodopa and normal laboratory findings. Here, we report on a unique case of Segawa disease with a fixed equinovarus foot. The patient was a twenty one year old female with the typical clinical manifestations since eight years of age who became wheel-chair dependent at the age of fifteen. The dystonia responded well to levodopa except for the foot deformities. The foot deformities were successfully corrected by use of the Ilizarov apparatus and she ambulated freely at follow up. Since several similar foot deformities appeared in the early stage of a progressive neurological degenerative disease, the treatable Segawa disease should be added to the differential diagnosis when facing a patient with pes equinovarus.