Fig. 5.

Clinical course of patient 1 during diet therapy.

Changes in the urinary levels of valine metabolites and symptoms are observed during the diet therapy in patient 1. Sixteen months following the first diet therapy 1, the patient's condition improves such that he has visual fixation and follows objects and dystonia disappears. Following the third diet therapy 1, apnea disappears However, spasticity remains; visual fixation is gradually lost, regardless of diet. Metabolic analysis presents improved levels of the toxic internal metabolites of valine. In contrast, diet therapy 2 does not improve the symptoms and the level of metabolites increases.