van Bogaert’s disease

van Bogaert’s disease /van bō″gərts/ [Ludo van Bogaert, Belgian neurophysiologist, b. 1897] , a rare familial disorder of lipid metabolism in which the substance cholestanol is deposited in the nervous system, blood, and connective tissue. Individuals with the disease develop progressive ataxia and dementia, premature atherosclerosis, cataracts, and xanthomas of the tendons. No effective treatment has been found. Also called cerebrotendinous xanthomatosis.