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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.
Etymology: H. Kufs, German psychiatrist, 1871-1955
an adult form of hereditary cerebral sphingolipidosis (amaurotic familial idiocy) characterized by cerebromacular degeneration, hypertonicity, and progressive spastic paralysis. Also called adult ceroid lipofuscinosis.