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.

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]

leukodystrophy

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

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

[-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

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]

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.

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.
References in periodicals archive ?
Keywords: Cerebral palsy, Epilepsy, Intracranial hemorrhage, Meningoencephalitis, Leukodystrophies, Seizures.
The report reviews key players involved in the therapeutics development for Leukodystrophies and enlists all their major and minor projects
The final chapters address therapeutic approaches in leukodystrophies and present a clinical approach to diagnosing leukoencephalopathies in children and adults.
Leukodystrophies are disorders which are characterized by the progressive destruction of myelin owing to the accumulation of various catabolize depending on the specific enzyme deficiency.
In the last few years a couple studies using human neural stem cells in the nervous system have begun in children with incurable brain diseases known as pediatric leukodystrophies.
2] gangliosidoses, neutral glycosphingolipidoses, glycoproteinoses, mucolipidoses, leukodystrophies, glycogen storage diseases, disorders of neutral lipids, and disorders of protein transport or trafficking (22, 23).
Canavan Disease: Canavan disease is a fatal neurological disorder that belongs to a group of genetic disorders called leukodystrophies, characterized by the abnormal development or degeneration of myelin.
February 7, 2013--A new study shows that human brain cells created by reprogramming skin cells are highly effective in treating myelin disorders, a family of diseases that includes multiple sclerosis and rare childhood disorders called pediatric leukodystrophies.
Pelizaeus-Merzbacher disease (PMD) is one of the many leukodystrophies in which patients struggle to produce the myelin sheath.
Leukodystrophies involve abnormal growth of the myelin sheath which is the fatty substance--or insulator--on nerve fibers in the brain and spinal cord.
Washington, Sept 13 (ANI): A Montreal led international team has identified the mutated gene responsible for three forms of leukodystrophies, a group of childhood-onset neurodegenerative disorders.

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