Dysmyelinating disorders

Last revised by Frank Gaillard on 5 Mar 2020

Dysmyelinating disorders are a subset of white matter disorders characterized by abnormal myelination 1. They include numerous inherited conditions that are characterized by a defective structure and function of the myelin sheath 2

Terminology

Dysmyelinating disorders are often thought of being synonymous with leukodystrophies, and traditionally they have been thought of as being distinct from demyelinating disorders where there is destruction or damage of previously normal myelination. 

More recently it has become apparent that many of the more common leukodystrophies (e.g. metachromatic leukodystrophyX-linked adrenoleukodystrophy) have a significant demyelinating component 3,4

As such is best to think of dysmyelinating and demyelinating as describing the dominant process involved in the larger group of leukodystrophies. ​

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