TY - T1的脂质性肌病的真面目防心绞痛的药物Ranolazine (P3.292) JF -神经学乔-神经学六世- 82 - 10补充SP - P3.292 AU -查尔斯Kassardj首页ian盟Duygu Selcen AU - Georgirene Vladutiu AU -黄李进AU -玛格丽塔Milone Y1 - 2014/04/08 UR - //www.ez-admanager.com/content/82/10_Supplement/P3.292.abstract N2 -目的:报告的脂质性肌病防心绞痛的药物Ranolazine揭露。背景:Ranolazine表示慢性心绞痛的治疗。它施加antiischemic属性通过减少细胞乙酰辅酶a的内容通过抑制脂肪酸β-oxidation和丙酮酸脱氢酶的激活。这导致心肌的转变能源生产从脂肪酸β-oxidation葡萄糖氧化。因为后者需要更少的氧气比脂肪酸β-oxidation ranolazine帮助维持由于缺血心肌功能在氧气供应不足。设计/方法:病例报告。结果:54岁妇女面对亚急性进步近端肢体软弱和运动性肌痛。她的症状恰逢ranolazine增加剂量后1.5年的稳定的低剂量ranolazine和辛伐他汀疗法。中断这两种药物导致改善强度和肌痛,虽然后来引入核黄素和辅酶Q10(辅酶q)导致决议的症状。没有证据表明心肌病。 CK values were normal throughout. Lactate and urine organic acids were normal. Several long-chain plasma acylcarnitines were mildly elevated. EMG showed myopathic changes, and muscle biopsy demonstrated numerous muscle fibers harboring lipid-containing vacuoles. Metabolic studies in muscle detected reduced CoQ10 content (9.6 micrograms/g muscle; nl 24-33), reduced activity of all respiratory chain enzymes, and normal ratio of esterified-carnitine/total carnitine in the absence of carnitine palmitoyltransferase deficiency. Multiple mitochondrial DNA deletions were present in muscle. Additional molecular analysis is ongoing. CONCLUSIONS: To our knowledge this is the first report of lipid storage myopathy unmasked by ranolazine. Although the underlying genetic defect of the myopathy is still under investigation, it is likely that ranolazine triggered the weakness and myalgia via its inhibitory effect on fatty acid β-oxidation. Despite the co-therapy with simvastatin, the clinical history and laboratory findings point away from simvastatin as the etiology of the patient’s symptoms.Disclosure: Dr. Kassardjian has nothing to disclose. Dr. Selcen has nothing to disclose. Dr. Vladutiu has nothing to disclose. Dr. Wong has nothing to disclose. Dr. Milone has nothing to disclose.Tuesday, April 29 2014, 3:00 pm-6:30 pm ER -