(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 ( Vogt-Spielmeyer disease ), it is not associated with ocular lesions.
