leukodystrophy

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leukodystrophy

 [loo″ko-dis´tro-fe]
disturbance of the white substance of the brain. See also adrenoleukodystrophy and leukoencephalopathy.
metachromatic leukodystrophy a hereditary leukoencephalopathy, marked by accumulation of a sphingolipid (sulfatide) in tissues, with diffuse loss of myelin in the central nervous system and progressive dementia and paralysis; classified according to age of onset as infantile, juvenile, and adult.
Miller-Keane Encyclopedia and Dictionary of Medicine, Nursing, and Allied Health, Seventh Edition. © 2003 by Saunders, an imprint of Elsevier, Inc. All rights reserved.

leu·ko·dys·tro·phy

(lū'kō-dis'trŏ-fē),
Term for a group of white matter diseases, some familial, characterized by progressive cerebral deterioration usually in early life, and pathologically by primary absence or degeneration of the myelin of the central and peripheral nervous systems with glial reaction; probably related to a defect in lipid metabolism; most leukodystrophies are autosomal recessive, several are X-linked recessive, and a few are autosomal dominant.
See also: Canavan disease.
[leuko- + G. dys, bad, + trophē, nourishment]
Farlex Partner Medical Dictionary © Farlex 2012

leukodystrophy

(lo͞o′kō-dĭs′trə-fē)
n.
Any of various genetic, usually inherited diseases characterized by a defect in the development or maintenance of myelin.
The American Heritage® Medical Dictionary Copyright © 2007, 2004 by Houghton Mifflin Company. Published by Houghton Mifflin Company. All rights reserved.

leukodystrophy

Neurology A heterogeneous group of disorders of cerebral myelin or its metabolism–eg, Krabbe's disease, metachromatic leukodystrophy, 'sphingolipidoses', that share certain pathological features Clinical 'White matter disease', ie predominantly motor, dominated by progressive paralysis and ataxia rather than dementia. See Globoid cell leukodystrophy.
McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.

leu·ko·dys·tro·phy

(lū'kō-dis'trŏ-fē)
A group of white matter diseases, some familial, characterized by progressive cerebral deterioration in early life and primary absence or degeneration of the myelin of the central and peripheral nervous systems; probably related to a defect in lipid metabolism; the adult type of Pelizaeus-Merzbacher disease is inherited as an autosomal dominant trait.
Synonym(s): leucodystrophy.
[leuko- + G. dys, bad, + trophē, nourishment]
Medical Dictionary for the Health Professions and Nursing © Farlex 2012