The sterol 27-hydroxylase (EC 1

We investigated the effect of chenodeoxycholic acid on cerebrospinal fluid sterol and protein composition in six patients with cerebrotendinous xanthomatosis, a progressive neurologic disease, and in 11 control subjects. In the cerebrospinal fluid from the controls, the mean (+/- SD) levels of cholesterol and cholestanol were 400 +/- 300 and 4 +/- 7 micrograms per deciliter, respectively. The levels were almost 1.5 and 20 times higher in cerebrospinal fluid from untreated patients with cerebrotendinous xanthomatosis. Cholestanol levels were also markedly elevated in the plasma of untreated patients, but their plasma cholesterol levels (215 +/- 61 mg per deciliter) were not different from control values. Treatment with chenodeoxycholic acid reduced cerebrospinal fluid cholesterol by 34 percent and cholestanol threefold. Plasma cholestanol levels also decreased sharply. Normal cerebrospinal fluid contained small quantities of albumin, apolipoproteins, and lecithin:cholesterol acyltransferase. In cerebrospinal fluid from untreated patients with cerebrotendinous xanthomatosis, immunoreactive apolipoprotein B or apolipoprotein B fragment was increased about 100-fold and albumin about 3.5-fold; apolipoprotein AI, apolipoprotein D, and lecithin:cholesterol acyltransferase were 1.5 to 3 times more concentrated. Apolipoprotein AIV and apolipoprotein E concentrations were comparable to those in controls, and apolipoprotein AII was considerably decreased. During treatment, the concentrations of albumin and apolipoproteins AI and B declined. These results suggest that increased cerebrospinal fluid sterols are derived from plasma lipoproteins by means of a defective blood-brain barrier in patients with cerebrotendinous xanthomatosis. Therapy with chenodeoxycholic acid reestablished selective permeability of the blood-brain barrier and normalized the concentrations of sterol and apolipoprotein in the cerebrospinal fluid.

Clinical observations of the apparent clustering of daily stroke admissions to a regional hospital in an arid climate prompted our investigation of possible meteorologic factors associated with stroke admissions. Daily hospitalization and meteorologic data were studied for 895 patients with stroke admitted to Soroka Medical Center, Beer-Sheva, Israel, during 1981, 1982, and 1983. The average daily incidence of stroke was about twice as great on relatively warm days as on relatively cold ones. This increase may be explained by increases in thromboembolic mechanisms secondary to physiologic changes in response to heat. When heat waves are predicted, information on the added risk for stroke needs to be disseminated to both the population and to health care providers so preventive measures can be instituted. Special attention should be devoted to air conditioning and adequate consumption of liquids, and antiplatelet aggregation medication such as aspirin should be considered. (Stroke 1989;20:65-69) S troke is the third leading cause of death in Israel, as in other countries. 1 -5 Epidemiologic studies have confirmed a significant decline in the mortality rate from both cardiovascular disease and stroke over the past 20 years. 5 -9 Possible reasons include treatment of modifiable risk factors such as hypertension, cardiac diseases, hyperglycemia, and hypercholesterolemia. One of us observed for many years that stroke admissions to Soroka Medical Center appear to occur in clusters. On some days there are one or no admissions, and these days tend to be grouped; on other days or groups of days there are multiple admissions. In such a short period as a few days, risk factors such as age, sex, and heredity and variable disease risk factors such as hypertension, cardiac disease, and diabetes mellitus are unlikely to change. We looked for risk factors that could change over a period of hours to days and decided that meteorologic factors should be evaluated. This

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