Diffuse liplfuscinosis of the central nervous system

Publisher Website
Google Scholar
Abstract
The case report is of a 49-yr. -old man who developed a condition diagnosed clinically as dyssynergia cerebellaris myoclonica. The disease ran a course lasting 6 years. At necropsy, the unexpected and outstanding feature was an intense lipofuscinosis of both neurons and glia, affecting all parts of the nervous system. Intracellular lipo-fuscin was found in relation to areas of high acid phosphatase activity. The known facts concerning the composition, staining properties, and ultrastructure of lipofuscin axe reviewed. Lipofuscin probably represents the end product of lipoprotein degeneration and that it may be related to altered lysosomes. It is argued that diffuse lipofuscinosis should be considered a form of lipidosis. The pathological basis of dyssynergia cerebellaris myoclonica is reviewed. Cerebral lipidosis has occasionally been reported as the basis of this syndrome. Previously described cases of adult lipidosis are reviewed, and an attempt is made to recognize various clinical patterns in this disorder.