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Leukodystrophy
--Definition:
It can manifests as leukoencephalomyelopathy, leukoencephalopathy, and leukomyelopathy.
Defective formation and maintenance of myelin. The white matter but not the gray matter is affected.
Leukodystrophies usually occur as hereditary diseases in infants and children.
Some milder forms can have initial presentation can occur in adults.
--Etiology:
Storage disorder: peroxisomal and lysosomal, e.g., X-linked adrenoleukodystrophy, Krabbe disease.
Myelin formation: e.g., Pelizaeus-Merzbacher disease.
Others: e.g., Alexander’s disease, ataxia with vitamin E deficiency, subacute combined degeneration.
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