Co-Q-10 Enzyme in ALS Dr. Petra Kaufmann, Assistant Professor of Neurology at Columbia University, sees patients with a variety of neuromuscular disorders. Her special interests are muscle diseases, mitochondrial disorders, and amyotrophic lateral sclerosis. In addition to her clinical work, she is involved in clinical research in the fields of mitochondrial diseases and ALS. She is recipient of the Herbert Irving Clinical Investigator Award and has been awarded a pilot grant to assess the use of high-dose Co-Q-10 in ALS patients. Dr. Kaufmann discussed her Co-Q-10 study at the recent Support Group meeting at All Souls Church in Manhattan. She highlighted the following issues: Coenzyme-Q-10 (Co-Q-10) is an anti-oxidant that is naturally occurring in the body. It performs the important function of cleaning up "free radicals." Overly abundant free radicals are understood to have a deleterious effect on motor nerves cells (motor neurons). In past studies using ALS mice, high-dose Co-Q-10 had a positive effect in slowing the progression of the disease. Co-Q-10 also has shown promise in Huntington’s and Parkinson’s diseases. In 2000, Hiroshi Mitsumoto, MD, and Salvatore Di Mauro, MD, tested Co-Q-10 in a small, open-label study and found positive trends in survival, inspiring Dr. Kaufmann to initiate a controlled pilot study of Co-Q-10 in ALS patients. Although Co-Q-10 is available over the counter, usually in low dose preparations, we only know that it is safe, as there is no proof at this time that it truly benefits ALS patients. Since taking many vitamins and supplements can be expensive, and difficult for individuals with swallowing changes, Dr. Kaufmann hopes that the results of the trial will make it clear whether or not Co-Q-10 should be part of the ALS treatment regimen. For the currently enrolling 4-week clinical trial of Co-Q-10at Columbia, the dose of Co-Q-10 is 1000 mg/day. The study drug is in a liquid form, and is available to participants for 6 months free of charge after the 4 weeks of trial participation.