Treatment of cerebrotendinous xanthomatosis: effects of chenodeoxycholic acid, pravastatin, and combined use

M Kuriyama, Y Tokimura, J Fujiyama, Y Utatsu… - Journal of the …, 1994 - Elsevier
Treatments by oral administration of chenodeoxycholic acid (CDCA) alone, 3-hydroxy-3-
methylglutaryl (HMG) CoA reductase inhibitor (pravastatin) alone, and combination of the
two drugs were attempted for 7 patients with cerebrotendinous xanthomatosis (CTX). CDCA
treatment at a dose of 300 mg/day reduced serum cholestanol (67.3% reduction), lathosterol
(50.8%), campesterol (61.7%) and sitosterol (12.7%). However, the sera of the patients
changed to be “atherogenic”; total cholesterol, triglyceride and low-density lipoprotein (LDL) …