Method for monitoring and preventing clinically significant ketone levels in patients receiving dichloroacetate co-administered with a carbohydrate restricted diet

A method for treating pyruvate dehydrogenase complex deficiency (PDCD) with dichloroacetate (DCA) in a PDCD patient on a ketogenic diet having a ratio of calories from fat:(carbohydrates+proteins) that is at least 3:1, the method comprising:

• measuring a pre-administration plasma beta-hydroxybutyrate (BHB) level of the patient on the ketogenic diet before initiating DCA treatment,
• assessing a GSTZ1 haplotype of the patient to determine whether the patient is an EGT carrier or an EGT non-carrier,
• after measuring the pre-administration plasma BHB level of the patient, orally administering to the patient the DCA in the form of a solution, wherein the DCA is dosed at 25 mg/kg/day when the patient is an EGT carrier, or the DCA is dosed at 12.5 mg/kg/day when the patient is an EGT non-carrier,
• measuring a post-administration plasma BHB level of the patient after the orally administering the DCA to the patient,
• after measuring the post-administration plasma BHB level of the patient, administering to the patient a modified diet having a ratio of calories from fat:(carbohydrates+proteins) that ranges from 2.5:1 to 0.7:1,
• and repeating the orally administering to the patient the DCA, wherein the DCA is dosed at 25 mg/kg/day when the patient is an EGT carrier, or the DCA is dosed at 12.5 mg/kg/day when the patient is an EGT non-carrier, each day the patient is on the modified diet.