Dopa Responsive Dystonia with Marked Diurnal Fluctuation (Sagawa Disease) Forty-Five Years of Follow–Up

Abstract

Objective: To describe the clinical course and very long personal follow-up of a patient with Segawa Disease Methods: The medical records of the patient since the diagnosis at the age of 9 years until her last follow-up at the age of 55 years were obtained and summarized Results: The long-term follow-up showed an excellent long-lasting response to L-DOPA therapy and also revealed that non-motor psychiatric features are an integral part of the disease rather than an emotional side effect of chronic motor disability. Conclusion: Long term follow-up of Segawa syndrome can provide additional information on prognosis and quality of life in old age, which are not known yet.