The vascular dementia of Fabry's disease


M.F. Mendez
Bok Engelsk 1997
Utgitt
1997
Omfang
Side 252- 257
Opplysninger
Fabry's disease, a rare X-linked disorder of glycosphingolipidmetabolism, can present as an insidious dementia in middle or laterlife. This genetic disorder produces a deficiency of a-galactosidaseA which results in the deposition of glycosphingolipids in bloodvessel walls in the brain as well as in the kidney, heart, peripheralnerves, and other organs. Among the cerebrovascular manifestations ofthis disorder is a vascular dementia from involvement of multiplesmall penetrating blood vessels, Fabry's disease is a considerationin the workup of an otherwise unexplained vascular dementia,particularly in males less than 65 years of age.
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