dysmyelination
Abnormal formation or structure of myelin sheaths due to genetic or metabolic disorders.
Full Definition
Dysmyelination refers to the abnormal formation, composition, or maintenance of myelin sheaths in the central or peripheral nervous system, typically resulting from genetic mutations affecting myelin proteins or metabolic pathways. Unlike demyelination, which involves the loss of previously normal myelin, dysmyelination represents a primary failure of proper myelin formation. This condition is characteristic of leukodystrophies and other inherited disorders affecting oligodendrocytes or Schwann cells.
Usage
Usage note: Distinguish from demyelination; implies primary developmental abnormality.
In Context
- "The MRI pattern was consistent with dysmyelination rather than acquired demyelinating disease." — Leukodystrophy diagnosis
- "Genetic testing confirmed a mutation causing dysmyelination in this pediatric case." — Metabolic disorder workup