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leukodystrophy

Pronunciation: lu'ko-dis'tro-fe

Definition: Term for a group of white matter diseases, some familial, characterized by progressive cerebral deterioration usually in early life, and pathologically by primary absence or degeneration of the myelin of the central and peripheral nervous systems with glial reaction; probably related to a defect in lipid metabolism; most leukodystrophies are autosomal recessive, several are X-linked recessive, and a few are autosomal dominant.

Synonym(s): leukodystrophia cerebri progressiva, leukodystrophia, sclerosis of white matter

[leuko- + G. dys, bad, + trophe, nourishment]

See Also: Canavan disease

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review date: Sep 19, 2016.

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