Dysmyelinating disorders
Dysmyelinating disorders are a subset of white matter disorders characterised by abnormal myelination. Dysmyelination is characterized by a defective structure and function of the myelin sheath and is distinct from demyelination which describes the destruction or damage of previously normal myelination.
Dysmyelination tends to be caused by hereditary conditions that can be further subdivided into those which primarily affect white matter, and those that affect both white and grey matter.
Primarily affecting white matter
d
White and grey matter affected
-
mucopolysaccharidoses
- Hunter disease
-
Hurler disease
- N.B. Morquio disease spares the CNS
-
mitochondrial disorders
-
electron transport chain disorders
- Leigh syndrome (sub-acute necrotizing encephalomyelopathy)
- MELAS
- MERRF
- mitochondrial membrane abnormality
-
electron transport chain disorders
- leukodystrophies
-
lysosomal storage disorders
- hangliosidosis (GM2)
See also
-
white matter disorders
- demyelinating disorders
- dysmyelinating disorders
- hypomyelinating disorders

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