| GCL | globoid cell leukodystrophy |
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| GLD | globoid leukodystrophy; glutamate dehydrogenase |
| MLD | manual lymph drainage; median lethal dose; metachromatic leukodystrophy; minimal lesion disease; min... |
| OLD | obstructive lung disease; orthochromatic leukodystrophy |
| PML | peripheral motor latency; polymorphonuclear leukocyte; posterior mitral leaflet; progressive multifo... |
| GLD | Globoid cell leukodystrophy |
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| MLD | Metachromatic Leukodystrophy |
| leukodystrophy | <radiology> Type of dysmyelinating disease, hereditary, peripheral nervous system unaffected in some disorders Specific diseases: adrenoleukodystrophy, metachromatic leukodystrophy, spongy degeneration (Canavan), globoid cell (Krabbe) leukodystrophy, Alexander disease, Pelizaeus-Merzbacher disease, Cockayne syndrome (12 Dec 1998) |
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| leukodystrophy with diffuse Rosenthal fibre formation | A metabolic disorder whose onset can be in infancy, adolescence, or adulthood; characterised pathologically by widespread cerebral demyelination with astrocyte and primitive oligodendroglial cell proliferation; refractile Rosenthal fibres result from the degeneration of these proliferating cells; aetiology unknown, but possibly due to a metabolic defect of astrocytes; sex-linked recessive disorder. (05 Mar 2000) |
| leukodystrophy, globoid cell | An inherited metabolic disorder of the nervous system, particularly the white matter. It is characterised histologically by a paucity of myelin and oligodendroglia, severe astrocytic gliosis, and massive infiltration with unique multinucleated globoid cells which are enriched in galactosylceramide. The primary genetic defect is a deficiency of galactosylceramidase. (12 Dec 1998) |
| leukodystrophy, metachromatic | A sphingolipidosis where there is defective desulfation of galactosyl-3-sulfate ceramide due to a defective enzyme cerebroside sulfatase (arylsulfatase a). The result is an accumulation of sulfatide in neural and non-neural tissues which manifests as mental deterioration and severe disturbances of the central nervous system. (12 Dec 1998) |
| adrenal leukodystrophy | Sudanophilic leukodystrophy with bronzing of skin and adrenal atrophy. A metabolic disorder of young males, characterised by widespread myelin degeneration and associated adrenal insufficiency. The myelin degeneration is massive in various portions of the brain and sometimes the spinal cord, with the accumulation of degradation products of myelin in macrophages: sudanophilic demyelination; atrophy is present in the adrenal glands and testes, and markedly increased amounts of very long-chain fatty acid are present in both the brain and adrenal glands. Symptoms include bronzing of the skin, dysarthria, cortical blindness, bilateral hemiplegia, pseudobulbar paralysis, and progressive dementia. Probably sex-linked recessive inheritance. (05 Mar 2000) |
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| globoid cell leukodystrophy | <radiology> Dysmyelinating disease, autosomal recessive, usually presents by 1 yr, specific enzyme deficiency identified, rapid spontaneous nystagmus, poikilothermia Synonym: Krabbe leukodystrophy (12 Dec 1998) |
| metachromatic leukodystrophy | <radiology> Dysmyelinating disease, autosomal recessive, aryl sulfatase A -- absent from urine and serum, most present by 2 yrs, die at 3-4 yrs, may arise at any age, CT: decreased density of white matter, primarily in centrum semiovale, with or without focal gall bladder defects (!) (12 Dec 1998) |
Synonyms : Classic Globoid Cell Leukodystrophy, Early-Onset Globoid Cell Leukodystrophy, Galactosylceramide-beta-Galactosidase Deficiency Disease, Globoid Body Sclerosis, Diffuse, Globoid Cell Leukodystrophy, Globoid Leukodystrophy, Krabbe Leukodystrophy
Synonyms : Greenfield Disease, Greenfield's Disease, Leukodystrophy, Metachromatic, Adult, Leukodystrophy, Metachromatic, Juvenile, Metachromatic Leukodystrophy, Adult-Type, Metachromatic Leukodystrophy, Infant, Metachromatic Leukodystrophy, Infant-Type
| leukodystrophy |
Leukodystrophy refers to progressive degeneration of the white matter of the brain due to imperfect growth or development of the myelin sheath, the fatty covering that acts as an insulator around nerve fiber. Myelin, which lends its color to the white matter of the brain, is a complex substance made up of at least ten different chemicals. The leukodystrophies are a group of disorders that are caused by genetic defects in how myelin produces or metabolizes these chemicals. ...
Ãâó: en.wikipedia.org/wiki/Leukodystrophy
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| leukodystrophy |
a group of childhood genetic disorders in which the protective coverings of the nerves are destroyed
Ãâó: www.american-depot.com/services/resources_gl_l.asp
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| leukodystrophy |
A group of genetic disorders characterized by the imperfect development or maintenance of myelin. Diseases in this class include adrenoleukodystrophy, Alexander disease, Canavan disease, Krabbe disease, metachromatic leukodystrophy, Pelizaeus-Merzbacher disease, Refsum disease, and phenylketonuria.
Ãâó: www.myelin.org/glossary.htm
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