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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.
Miller-Keane Encyclopedia and Dictionary of Medicine, Nursing, and Allied Health, Seventh Edition. © 2003 by Saunders, an imprint of Elsevier, Inc. All rights reserved.