Object: The potential effects of branched-chain ammo acids (BCAAs) on spinocerebellar degeneration (SCD) were explored in eleven patients. Methods: The patients received 200 ml of BCAA-rich solution, 2 mg of thyrotropin-releasing hormone (TRH; protirelin), or a placebo daily for 7 days each in a random order. An SCD score was used to quantify the severity of symptoms. Patients: Eleven patients with SCD (7 male, 4 female; mean age 60±11; mean disease duration 5.5 years) participated in this study. Results: The mean SCD score of the eleven patients improved significantly by the BCAA treatment compared with the baseline. The conditions of five of the eleven patients (45%) were clearly improved by the BCAA treatment. All of the responders manifested predominantly cerebellar symptoms, but no prominent parkinsonian symptoms. Two patients with marked rigidity and akinesia did not respond to the treatment. Conclusion: We concluded that BCAAs do have a beneficial effect on functional improvement in patients with SCD, and that further large scale studies are needed.
(Internal Medicine 38: 401-406, 1999)