Hanisch Frank, Zierz S
Neurological Departement, University of Halle-Wittenberg, Ernst-Grube-Strasse 40, D-06097 Halle/Saale, Germany.
Eur J Med Res. 2003 Nov 12;8(11):485-91.
Coenzyme Q10 (CoQ10) is frequently administered in mitochondrial diseases. Mitochondrial dysfunction and CoQ10 treatment was also proposed in neurodegenerative disorders as amyotrophic lateral sclerosis and Parkinsons disease.
Seventeen patients with mitochondrial CPEO were treated with CoQ10 (dosage: 0.60 1.80 mg/kg body wt) in an open trial. Serum levels of CoQ10 were monitored before and after 6-9 and 12-15 months of CoQ10 therapy. CoQ10 concentration in muscle was measured in all patients before treatment.
Prior to treatment CoQ10 concentration in muscle was normal in all patients. Eight patients completed the study after 12-15 months. Prior to treatment there was no correlation between CoQ10 in muscle and serum. There was no inverse correlation of serum lactate with CoQ10 in muscle before and in serum before and during therapy. CoQ10 serum level and body weight related CoQ10 dosage correlated significantly after 6-9 months but not after 12-15 months (p = 0.043 and n. s., respectively). During continued administration of CoQ10 the CoQ10 serum level was increased 2.76 +/- 1.00-fold after 6-9 months (range: 1.04-3.80). but returned to 1.70 +/- 0.98-fold after 12-15 months (range: 0.91-3.83). Serum lactate did not significantly change during treatment. There was no effect of CoQ10 treatment on signs and symptoms.
The only transient increase of CoQ10 in serum has to be considered in any low dose long-term treatment with CoQ10.
辅酶Q10（CoQ10）常用于线粒体疾病的治疗。线粒体功能障碍以及CoQ10治疗也被应用于神经退行性疾病，如肌萎缩侧索硬化症和帕金森病。
在一项开放性试验中，17例线粒体性慢性进行性眼外肌麻痹（CPEO）患者接受了CoQ10治疗（剂量：0.60 - 1.80mg/kg体重）。在CoQ10治疗6 - 9个月以及12 - 15个月前后监测血清CoQ10水平。在所有患者治疗前测量肌肉中的CoQ10浓度。
治疗前所有患者肌肉中的CoQ10浓度均正常。8例患者在12 - 15个月后完成了研究。治疗前肌肉和血清中的CoQ10之间无相关性。治疗前、治疗期间血清乳酸与肌肉及血清中的CoQ10均无负相关性。CoQ10血清水平与体重相关的CoQ10剂量在6 - 9个月后显著相关，但在12 - 15个月后无相关性（分别为p = 0.043和无显著性差异）。在持续给予CoQ10期间，CoQ10血清水平在6 - 9个月后升高了2.76±1.00倍（范围：1.04 - 3.80），但在12 - 15个月后降至1.70±0.98倍（范围：0.91 - 3.83）。治疗期间血清乳酸无显著变化。CoQ10治疗对体征和症状无影响。
在任何CoQ10低剂量长期治疗中，都必须考虑血清中CoQ10仅出现短暂升高这一情况。
