Medical Definition of Hypomyelination

  • Medical Author:
    Melissa Conrad Stöppler, MD

    Melissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology.

Hypomyelination: an abnormally low amount of myelination (the process by which an insulating layer of fatty tissue develops surrounding nerve cells). A number of conditions (hypomyelinating disorders) are characterized by an abnormally low amount of myelination. These may involve the central nervous system (brain and spinal cord) only or both the central and peripheral nervous systems. Examples of hypomyelinating disorders include Pelizaeus-Merzbacher disease, Tay Syndrome (trichothiodystrophy with hypersensitivity to sunlight), Salla disease (free sialic acid storage disease), hypomyelination with congenital cataract, 4H syndrome, and Cockayne syndrome.

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Reviewed on 12/21/2018

REFERENCE: Barkovich AJ1, Deon S2. "Hypomyelinating disorders: An MRI approach." Neurobiol Dis. 2016;87:50-8.

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