OBJECTIVES: I. Assess the biosynthesis of cholesterol and cholestanol, and measure the turnover of individual sterols and bile acids in patients with cerebrotendinous xanthomatosis before and after a cholesterol- and cholestanol-free diet. II. Assess the biosynthesis of cholesterol and cholestanol, and measure the turnover of individual sterols and bile acids in these patients before and after lovastatin and chenodeoxycholic acid.

Condition	Treatment or Intervention	Phase
Cerebrotendinous Xanthomatosis	Drug: chenodeoxycholic acid  Drug: lovastatin	Phase II

Further Study Details: 

Expected Total Enrollment:  5

PROTOCOL OUTLINE: Following a 2-week baseline assessment, patients participate in a feeding study for up to 3 weeks. The diet is free of cholesterol and cholestanol. For the next 4 weeks, patients return to their typical diet and are medicated with daily lovastatin and chenodeoxycholic acid. The feeding study is repeated for an additional 3 weeks, with the patient taking either lovastatin or chenodeoxycholic acid.

Genders Eligible for Study:  Both

Criteria

PROTOCOL ENTRY CRITERIA: Cerebrotendinous xanthomatosis

Oregon
      Oregon Health Sciences University, Portland,  Oregon,  97201-3098,  United States; Recruiting

Study chairs or principal investigators

William Connor,  Study Chair,  Oregon Health and Science University   

Study ID Numbers:  NCRR-M01RR00334-0067; OHSU-4008
Record last reviewed:  December 2003
Last Updated:  October 13, 2004
Record first received:  October 18, 1999
ClinicalTrials.gov Identifier:  NCT00004346
Health Authority: United States: Federal Government
ClinicalTrials.gov processed this record on 2005-04-08