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leukodystrophy
leukodystrophy (ˌluːkəʊˈdɪstrəfɪ) n, pl -phies (Pathology) pathol any of several progressive disorders characterized by the degeneration of white matter or myelinleukodystrophy
leukodystrophy[‚lü·kə′dis·trə·fē] (medicine) A condition thought to result from an inborn error of metabolism and characterized by progressive degeneration of the white matter of the cerebrum, or by defective buildup of myelin. leukodystrophy
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.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. Synonym(s): leukodystrophia cerebri progressiva, leukodystrophia, sclerosis of white matter [leuko- + G. dys, bad, + trophē, nourishment] 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.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. 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] |