cerebral sphingolipidosis, adult type ... 
(koofs) the rare adult form of neuronal ceroid-lipofuscinosis, usually seen before age 40. Clinical findings include progressive neurologic degeneration, excessive storage of lipofuscin in the central nervous system, and shortened life expectancy. Unlike the late infantile (Janský-Bielschowsky disease), and juvenile forms (Vo...
Type: Term Pronunciation: kūfs Definitions: 1. cerebral sphingolipidosis, adult type.