Melancon S B, Vanasse M, Geoffroy G, Barabe L, Proulx A, Fontaine G, Dallaire L, Potier M
Can J Neurol Sci. 1982 May;9(2):155-64. doi: 10.1017/s0317167100043870.
Twenty-two patients with Friedreich's Ataxia and ten normal controls were followed for one year and assessed as to their clinical performance after two successive six-month periods of lecithin or safflower oil. Results demonstrated no significant difference in performance scores according to group assignation, neither in patients nor in controls. According to stages, two patients in stage I and to a lesser degree, one patient in stage IV showed better scores for muscle strength and some motor accuracy and coordination tests with lecithin. Controls as groups maintained positive scores in all tests. Patients as groups showed negative mean values in nine out of eleven tests. Again as groups, patients receiving safflower oil demonstrated a mean 8% less deterioration than patients receiving lecithin. This study demonstrates that objective clinical tests and the participation of normal controls are a must in a therapeutic trial implicating patients with a progressive disorder such as Friedreich's Ataxia. The possible role of linoleic acid as the active factor from which clinical improvement proceeded in some specific patients and with early functional stages of the disease, has to be considered and reevaluated in the near future.
对22例弗里德赖希共济失调患者和10名正常对照者进行了为期一年的随访,并在连续两个六个月的卵磷脂或红花油治疗期后评估其临床表现。结果表明,根据分组情况,患者组和对照组的表现评分均无显著差异。根据疾病阶段,1期的2例患者以及在较小程度上,4期的1例患者在接受卵磷脂治疗后,肌肉力量、一些运动准确性和协调性测试的得分更高。对照组在所有测试中均保持正得分。患者组在11项测试中的9项中显示出负均值。同样作为分组,接受红花油治疗的患者比接受卵磷脂治疗的患者平均恶化程度低8%。这项研究表明,在涉及弗里德赖希共济失调等进行性疾病患者的治疗试验中,客观的临床测试和正常对照者的参与是必不可少的。在不久的将来,必须考虑并重新评估亚油酸作为某些特定患者和疾病早期功能阶段临床改善的活性因子的可能作用。