Dotti M T, Lütjohann D, von Bergmann K, Federico A
Department of Neurological and Behavioural Sciences, University of Siena, Viale Bracci, Siena, Italy.
Neurol Sci. 2004 Oct;25(4):185-91. doi: 10.1007/s10072-004-0320-6.
The concentrations of serum cholesterol, cholestanol and non-cholesterol sterols were measured in a patient with cerebrotendinous xanthomatosis under different therapeutic regimens. During treatment with chenodeoxycholic acid (CDCA) (750 mg/day) plus simvastatin (20 mg/day) for two years cholesterol and cholestanol concentrations averaged 188+/-10 mg/dl and 0.54+/-0.03 mg/dl. Thereafter treatment with simvastatin was discontinued. During treatment with low-density lipoprotein (LDL)-apheresis plus CDCA for 33 weeks, cholestanol concentrations reached almost normal levels (0.48+/-0.03 mg/dl immediately before and 0.32+/-0.02 mg/dl directly after LDL-apheresis, n=6). A further reduction of cholesterol and cholestanol was achieved by addition of simvastatin (20 mg/day). Cholesterol and cholestanol concentrations before and after LDL-apheresis during this treatment period averaged 122+/-4 mg/dl and 55+/-10 mg/dl, and 0.42+/-0.02 mg/dl and 0.18+/-0.06 mg/dl, respectively. Despite the consistent reduction of cholestanol to normal or even subnormal levels, a definite improvement of clinical symptoms was not noted. Our results suggest caution in the recourse to an aggressive cholestanol lowering therapy.
在不同治疗方案下，对一名患有脑腱性黄瘤病的患者测定了血清胆固醇、胆甾烷醇和非胆固醇甾醇的浓度。在用鹅去氧胆酸（CDCA）（750毫克/天）加辛伐他汀（20毫克/天）治疗两年期间，胆固醇和胆甾烷醇浓度平均为188±10毫克/分升和0.54±0.03毫克/分升。此后停用辛伐他汀治疗。在用低密度脂蛋白（LDL）血液分离术加CDCA治疗33周期间，胆甾烷醇浓度几乎达到正常水平（LDL血液分离术前立即为0.48±0.03毫克/分升，术后直接为0.32±0.02毫克/分升，n = 6）。通过加用辛伐他汀（20毫克/天），胆固醇和胆甾烷醇进一步降低。在此治疗期间，LDL血液分离术前和后的胆固醇和胆甾烷醇浓度平均分别为122±4毫克/分升和55±10毫克/分升，以及0.42±0.02毫克/分升和0.18±0.06毫克/分升。尽管胆甾烷醇持续降至正常甚至低于正常水平，但未观察到临床症状有明显改善。我们的结果提示在采用积极的降低胆甾烷醇治疗时应谨慎。
