In 1971, doctors in Montreal Children's Hospital gave a female infant with elevated branched-chain amino acids 10 milligrams of thiamine. Her BCAA plasma levels returned to normal. That was the first observed case of thiamine-responsive MSUD. Thiamine or vitamin B1 helps in amino acid metabolism, and this less severe subtype responds well to large dosages of the vitamin. Between 10 and 100 milligrams improve enzyme action, compared to a daily requirement of just 1.1 milligrams for healthy people.

This site offers information designed for educational purposes only. You should not rely on any information on this site as a substitute for professional medical advice, diagnosis, treatment, or as a substitute for, professional counseling care, advice, diagnosis, or treatment. If you have any concerns or questions about your health, you should always consult with a physician or other healthcare professional.