Dysmyelinating disorders

Last revised by Daniel J Bell on 8 May 2024

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


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. 

It is now apparent that many of the more common leukodystrophies (e.g. metachromatic leukodystrophyX-linked adrenoleukodystrophy) have a significant demyelinating component 3,4

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

ADVERTISEMENT: Supporters see fewer/no ads