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leukodystrophy, metachromatic



leukodystrophy, metachromatic Causes


metachromatic leukodystrophy is caused by a lack of the arylsulfatase A enzyme. This enzyme is needed to keep sulfatides from building up and destroying the myelin.


leukodystrophy, metachromatic Definition


Metachromatic leukodystrophy is a kind of leukodystrophy that destructs the development of myelin, the fatty white matter that protects the nerve fibers in the the central and peripheral nervous system.


leukodystrophy, metachromatic Symptoms and Signs


The disease shows signs of motor development difficulties during the infant's first year. Children with metachromatic leukodystrophy often suffer from dementia and mental deterioration, followed by eventual death.


leukodystrophy, metachromatic Treatment


There is no standard cure for metachromatic leukodystrophy, but gene therapy as well as enzyme replacement therapy are currently under investigation as potential cures to the disease.


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