Leukodystrophies

The leukodystrophies (,Table,30:6 ) are genetic diseases that show morphological changes in the white matter of the central nervous system. Each of the leukodystrophies most likely represents an inborn error of metabolism due to a defective gene that produces an enzymatic abnormality and metabolic derangement that affects myelin. Myelin dysfunction is the basic feature of all these disorders, and demyelination or destruction of normally formed myelin can occur as well as loss of defective myelin, called dysmyelination. Simple demyelination is typical of many sudanophilic leukodystrophies. Dysmyelination is related to an underlying genetic or metabolic disturbance, and in this type of leukodystrophy, abnormal lipids incorporated into the defective myelin sheath tend to be metachromatic when stained. Leukodystrophies may be caused by enzymatic defects that produce aberrations in peroxisome function, which induces demyelination, or to enzymatic defects at specific points in the metabolic pathways of the sphingo- lipids.

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