The effectiveness of long-term dietary therapy in the treatment of adult Refsum disease.

Objective: To evaluate the long-term effectiveness of dietary therapy with regular dietetic reinforcement for adult Refsum disease.

Methods: Retrospective case note analysis of records of plasma phytanic acid and hospital admission of 13 patients with adult Refsum disease who attended the specialist centre and repeatedly received dietary instruction for a minimum of 10 years.

Results: Patients undergoing review had attended for 11-28 years totalling 237 years.