Confirmation of the efficacy of vitamin B6 supplementation for McArdle disease by follow-up muscle biopsy.

Abstract

No effective treatment for McArdle disease exists.We report a Japanese patient with McArdle disease who was treated with vitamin B(6) supplementation (60-90 mg/day). After treatment, increased muscle phosphorylase activity was confirmed by follow-up muscle biopsy (3.8 times higher than pretreatment levels). Increased lactate levels were seen on the forearm exercise test, and regular work activities could be resumed. Vitamin B(6) supplementation can enhance residual phosphorylase activity and improve insufficient anaerobic glycolysis of skeletal muscle.

• Authors: Shinya Sato, Takekazu Ohi, Ichizo Nishino, Hideo Sugie
• Journal: Muscle & nerve (Muscle Nerve) Vol. 45 Issue 3 Pg. 436-40 (Mar 2012) ISSN: 1097-4598 [Electronic] United States
• PMID: 22334182 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2011 Wiley Periodicals, Inc.

Chemical References

• Vitamin B Complex
• Pantothenic Acid
• Phosphorylases

Topics

• Adult
• Biopsy
• Dietary Supplements
• Exercise Therapy
• Follow-Up Studies
• Glycogen Storage Disease Type V (diet therapy, pathology, rehabilitation)
• Humans
• Male
• Muscle, Skeletal (drug effects, pathology)
• Pantothenic Acid (administration & dosage, pharmacology)
• Phosphorylases (metabolism)
• Vitamin B Complex (pharmacology)