1999
DOI: 10.2169/internalmedicine.38.401
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Branched-Chain Amino Acid Therapy for Spinocerebellar Degeneration: A Pilot Clinical Crossover Trial.

Abstract: Object: The potential effects of branched-chain amino 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 SCDscore was used to quantify the severity of symptoms. Patients: Eleven patients with SCD(7 male, 4 female; mean age 60±ll; mean disease duration 5.5 years) participated in this study. Results: The me… Show more

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Cited by 7 publications
(4 citation statements)
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“…[17][18][19] The mean values of LNG, ENV-AREA, and REC-AREA in EC sessions of the migraine group were significantly greater than in controls, however, the degree of abnormality of stabilometric parameters were smaller than the results of ataxic patients in the literature. 20,21 Because we examined patients with migraine headache without any clinically apparent ataxic symptoms during the headache-free period, it is reasonable that we found only small difference in migrainous patients compared with ataxic patients. Romberg quotients of all stabilometric parameters except L/E in the migraine group were significantly greater than in the controls.…”
Section: Discussionmentioning
confidence: 99%
“…[17][18][19] The mean values of LNG, ENV-AREA, and REC-AREA in EC sessions of the migraine group were significantly greater than in controls, however, the degree of abnormality of stabilometric parameters were smaller than the results of ataxic patients in the literature. 20,21 Because we examined patients with migraine headache without any clinically apparent ataxic symptoms during the headache-free period, it is reasonable that we found only small difference in migrainous patients compared with ataxic patients. Romberg quotients of all stabilometric parameters except L/E in the migraine group were significantly greater than in the controls.…”
Section: Discussionmentioning
confidence: 99%
“…Interestingly, ALS has been associated with abnormal glutamate metabolism, and since branched-chain amino acids (BCAAs; e.g., l -leucine, l -isoleucine, and l -valine) can modify glutamate metabolism, these have been evaluated with respect to their therapeutic potential [ 49 ]. However, clinical studies have provided conflicting evidence with some studies reporting negative effects or a lack of efficacy [ 49 , 50 , 51 ], whereas others reported amelioration of ALS symptoms [ 52 , 53 ]. More recently, in vivo preclinical evidence suggests that BCAA, tryptophan, and particularly arginine and proline metabolic pathways are associated with disease progression [ 54 ].…”
Section: Discussionmentioning
confidence: 99%
“…Parkinsonian features, but not a true PD‐like phenotype, have also been described in patients with SCA1, SCA6, SCA12, SCA14, and SCA21 12, 29, 43–50…”
Section: Types Of Movement Disordersmentioning
confidence: 97%