leukodystrophy (redirected from Leukodystrophies)

leukodystrophy /leu·ko·dys·tro·phy/ (-dis´trah-fe) disturbance of the white substance of the brain; see also leukoencephalopathy.

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globoid cell leukodystrophy  Krabbe's disease.

hereditary adult-onset leukodystrophy  an inherited leukoencephalopathy characterized by progressive degeneration of the white matter, with motor disturbances, bowel and bladder incontinence, and orthostatic hypotension.

metachromatic leukodystrophy  an inherited disorder due to accumulation of sulfatide in tissues with a diffuse loss of myelin in the central nervous system; it occurs in several forms, with increasing age of onset correlated to decreasing severity, all initially presenting as mental regression and motor disturbances.

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leu·ko·dys·tro·phy (lk-dstr-f)

n.

Degeneration of the white matter of the brain characterized by demyelination and glial reaction, probably related to defects of lipid metabolism. Also called leukoencephalopathy.

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leukodystrophy

[-dis′trəfē]

Etymology: Gk, leukos, white, dys + trophe, nourishment

a disease of the white matter of the brain, characterized by demyelination. See also leukoencephalopathy.

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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.

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leukodystrophy

disorders or metabolic defects in the system required for the maintenance and degradation of normally formed myelin in newborn and young animals. Clinical signs begin some time after birth. See also leukoencephalopathy.

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Afghan leukodystrophy

see hereditary myelopathy of Afghan hounds.

cavitating leukodystrophy

a familial, probably inherited, disease of Dalmatian dogs in which there is blindness and progressive motor dysfunction from a few months of age.

fibrinoid leukodystrophy

a familial disease of astrocytes causing ataxia, paresis and behavioral changes from an early age; reported in Labrador retrievers. Similar to Alexander's disease in humans.

globoid cell leukodystrophy

an inherited lysosomal storage disease of polled Dorset sheep, cats, and dogs, particularly Cairn terriers and West Highland white terriers, caused by a deficiency of the enzyme beta-galactocerebrosidase. From a young age, there is weakness that progresses to paralysis. Called also galactocerebrosidosis and Krabbe's disease.

metachromatic leukodystrophy

a leukoencephalopathy reported in cats, caused by a deficiency of the enzyme arylsulfatase A. There is accumulation of a sphingolipid (sulfatide) in tissues, with diffuse loss of myelin in the central nervous system and progressive neurological dysfunction.

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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.