Spinal cord compression with paraplegia in xanthomatosis due to normocholesterolemic sitosterolemia
- 1 September 1990
- journal article
- research article
- Published by Wiley in Annals of Neurology
- Vol. 28 (3) , 390-393
- https://doi.org/10.1002/ana.410280316
Abstract
A 48‐year‐old woman with a 30‐year history of tendinous xanthomatosis developed paraplegia. The magnetic resonance image revealed extramedullary tumors. The analysis of her sera and tumors revealed increased amount of plant sterols, especially sitosterol, and the diagnosis of sitosterolemia was made. This is the first reported case of a patient with sitosterolemia who had the neurological complication of spinal cord compression due to extramedullary sitosterolemic xanthomas.This publication has 12 references indexed in Scilit:
- Competitive inhibition of bile acid synthesis by endogenous cholestanol and sitosterol in sitosterolemia with xanthomatosis. Effect on cholesterol 7 alpha-hydroxylase.Journal of Clinical Investigation, 1988
- The effect of abnormal plasma and cellular sterol content and composition on low density lipoprotein uptake and degradation by monocytes and lymphocytes in sitosterolemia with xanthomatosisMetabolism, 1988
- Normocholesterolemic XanthomatosisArchives of Dermatology, 1986
- Abnormal metabolism of shellfish sterols in a patient with sitosterolemia and xanthomatosis.Journal of Clinical Investigation, 1986
- A unique patient with coexisting cerebrotendinous xanthomatosis and β-sitosterolemiaThe American Journal of Medicine, 1981
- HYPERAPOBETALIPOPROTEINAEMIA IN TWO FAMILIES WITH XANTHOMAS AND PHYTOSTEROLAEMIAThe Lancet, 1981
- Phytosterolemia and type IIa hyperlipoproteinemia with tuberous xanthomasJournal of the American Academy of Dermatology, 1981
- Phytosterolaemia, xanthomatosis and premature atherosclerotic arterial disease: a case with high plant sterol absorption, impaired sterol elimination and low cholesterol synthesisEuropean Journal of Clinical Investigation, 1980
- β-Sitosterolemia and XanthomatosisNew England Journal of Medicine, 1976
- β-Sitosterolemia and XanthomatosisJournal of Clinical Investigation, 1974