The vascular dementia of Fabry's disease.

Autor: Mendez MF; Department of Neurology, University of California at Los Angeles, USA. mmendez@UCLA.edu, Stanley TM, Medel NM, Li Z, Tedesco DT
Jazyk: angličtina
Zdroj: Dementia and geriatric cognitive disorders [Dement Geriatr Cogn Disord] 1997 Jul-Aug; Vol. 8 (4), pp. 252-7.
DOI: 10.1159/000106640
Abstrakt: Fabry's disease, a rare X-linked disorder of glycosphingolipid metabolism, can present as an insidious dementia in middle or later life. This genetic disorder produces a deficiency of alpha-galactosidase A which results in the deposition of glycosphingolipids in blood vessel walls in the brain as well as in the kidney, heart, peripheral nerves, and other organs. Among the cerebrovascular manifestations of this disorder is a vascular dementia from involvement of multiple small penetrating blood vessels. Fabry's disease is a consideration in the workup of an otherwise unexplained vascular dementia, particularly in males less than 65 years of age.
Databáze: MEDLINE