Kufs' disease


Kufs' disease

 [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.