Normalisation of serum cholestanol concentration in a patient with cerebrotendinous xanthomatosis by combined treatment with chenodeoxycholic acid, simvastatin and LDH apheresis

Publisher Website
Google Scholar
Abstract
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.

This publication has 0 references indexed in Scilit: