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Of the primary dementing disorders that cause frontotemporal dementia, the best-known is Pick disease. We report on a 44-year-old woman with progressive frontal lobe dementia and spastic paraplegia. Examination revealed increased serum levels of cholestanol with abnormal cholesterol metabolism and a heterozygous mutation of the sterol 27-hydroxylase gene (CYP27). Biochemical findings were compatible with cerebrotendinous xanthomatosis (CTX); however, the clinical manifestations were very dissimilar. To our knowledge, a symptomatic carrier of this mutation among CTX patients has not been reported. We speculate that the present patient has a previously undescribed neurodegenerative disease related to abnormal cholesterol metabolism with this heterozygous mutation.

Mutations in the sterol 27-hydroxylase gene (CYP27) cause cerebrotendinous xanthomatosis (CTX). Early diagnosis of CTX is crucial because treatment with chenodeoxycholic acid can prevent or reverse some of the neurologic disability associated with the disease. We report the identification of three types of mutations (Arg441Trp, Arg372Gln, and Arg441Gln) in the CYP27 gene in five patients with suspected CTX from four unrelated families by restriction endonuclease analysis.

Surrogate testing is often used to examine the possible deterministic nature of time series at the first step. In this study, the method of surrogate testing was applied to hydrodynamic time series in bubble columns. The hydrodynamic time series were measured in three gas-liquid systems bubble columns, with inner diameters of 20, 40, and 80 cm. The gas velocity varied from U g ) 3.3 cm/s to U g ) 9.0 cm/s. The determinism of these hydrodynamic time series was examined in terms of three methods of surrogate testing: local flow, local dispersion, and nonlinear prediction. Three types of surrogate data algorithmssFourier-shuffled, phaserandomized, and Gaussian-scaledsserved as stochastic controls. Therefore, a total of nine sets of tests were conducted for each experimental time series. If any of these surrogate data exhibits a similar distribution with the original time series, the hypothesis that the hydrodynamic time series in bubble column is generated with linear stochastic process is assumed not to be able to be rejected. Two samples were given in detail to show how to implement the calculation. Measurements for one experimental time series were obtained from the 80-cm bubble column with U g ) 3.3 cm/s; eight of the nine tests indicated that the original time series was not linear-stochastic. Results for another experimental time series were obtained from the 20-cm bubble column, with U g ) 9.0 cm/s; five of the nine tests indicated that the original time series was not linear-stochastic, and four of the nine show that the null hypothesis was assumed not to be able to be rejected at a 99% level of significance. Such test results indicates that the dynamic nature of hydrodynamics in bubble columns may be rather complex and may contain a stochastic component. The strength of the stochastic component is dependent on the flow conditions in the bubble columns.