Fig. 2.

Total levels of key metabolic markers of cerebrotendinous xanthomatosis (CTX). A–D: Concentrations of the indicated metabolites measured by GC-MS in serum (upper panel) and CSF (lower panel) of controls (green), untreated patients with CTX (CTX, red) and patients treated with 750 mg/day CDCA (CDCA, blue). The data are presented as individual data points and mean ± SD on a logarithmic scale with antilog numbering. Absolute values of zero correspond to log-values of ∼0.7. Square-shaped data points highlight patients with CTX with low cholestanol levels and triangular points highlight the patient we obtained data from before and after CDCA treatment. ∗P < 0.0125 calculated by one-way ANOVA and Fisher’s LSD or by Kruskal-Wallis and Mann-Whitney-U tests. ∗ are only indicated for significant pair-wise comparisons. CDCA, chenodeoxycholic acid; CSF, cerebrospinal fluid.