Pilot trial of branched-chain aminoacids in amyotrophic lateral sclerosis.
22 patients with amyotrophic lateral sclerosis were entered into a double-blind, randomised, placebo-controlled trial of treatment with branched-chain aminoacids. 11 received daily 12 g L-leucine, 8 g L-isoleucine, and 6.4 g L-valine, by mouth, and the remainder received placebo. During the one-year trial, patients in the placebo group showed a linear decline in functional status consistent with the natural history of the disease. Those treated with aminoacids showed significant benefit in terms of maintenance of extremity muscle strength and continued ability to walk.
D000597:Amino Acids, Branched-Chain / Q000627:therapeutic use*; D000690:Amyotrophic Lateral Sclerosis / Q000188:drug therapy* / Q000503:physiopathology; D002986:Clinical Trials as Topic; D004338:Drug Combinations; D005260:Female; D006801:Humans; D007532:Isoleucine / Q000008:administration & dosage; D007930:Leucine / Q000008:administration & dosage; D008124:Locomotion; D008297:Male; D008875:Middle Aged; D009132:Muscles / Q000503:physiopathology; D009460:Neurologic Examination; D010349:Patient Compliance; D013116:Spinal Cord / Q000503:physiopathology; D014633:Valine / Q000008:administration & dosage
D000597:Amino Acids, Branched-Chain; D004338:Drug Combinations; D007532:Isoleucine; D007930:Leucine; D014633:Valine