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| | ==[[Lysosomal Storage Disease]] == | | ==[[Lysosomal Storage Disease]] == |
| − | (LSD)
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| − | * Occurs in many breeds of dogs and cats.
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| − | * Genetic defects give deficiencies of specific enzymes of degradation.
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| − | ** Disease only occurs in animals homozygous at the gene pair controlling enzyme activity.
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| − | *** The genes are co-dominant.
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| − | ** Enzyme activity is extremely low or entirely absent.
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| − | * Large amounts of waste products accumulate within cells.
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| − | ** Nervous system cells are predisposed.
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| − | ** Cells become engorged.
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| − | ** Normal function is prevented.
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| − | * [http://w3.vet.cornell.edu/nst/nst.asp?Fun=Display&imgID=20531 Image 1, courtesy of Cornell Veterinary Medicine] <BR>
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| − | * [http://w3.vet.cornell.edu/nst/nst.asp?Fun=Display&imgID=20530 Image 2, courtesy of Cornell Veterinary Medicine]
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| − | * [http://w3.vet.cornell.edu/nst/nst.asp?Fun=Display&imgID=20185 Image 3, courtesy of Cornell Veterinary Medicine]
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| − | ===Clinical Signs===
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| − | * Nervous system cells are predisposed to the disease, and so progressive neurological signs predominate.
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| − | ** Develop between 2 weeks and 12 months of age.
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| − | ** The disease is ultimately fatal.
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| − | * For example, Siamese cats show:
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| − | ** Ganglisidosis
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| − | ** Mucopolysaccharidosis
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| − | ===Diagnosis===
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| − | * Clinical signs are considered.
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| − | * Urine enzyme assays may be helpful.
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| − | * Post-mortem diagnosis is possible.
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| − | * More recently, gene tests have been available.
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| − | [[Category:Central Nervous System - Degenerative Pathology]]
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