Myelin is a protective layer that enwraps the axonal terminals and is an
essential component of the central nervous system white matter. Loss of myelin leads
to conduction block in the axon leading to demyelinating disorders. Inherited poor
formation of myelin is known as hypomyelination, and abnormally formed myelin is
called dysmyelination. Demyelinating disorders exclude diseases where degeneration of
the axon is the initial event and myelin is degraded secondarily. Most neurologists use
the term demyelination only for acquired forms of loss of myelin with relative
preservations of axons due to inflammation such as multiple sclerosis. Demyelinating
disease in children may be monophasic (e.g., acute disseminated encephalomyelitis,
optic neuritis, and transverse myelitis) or chronic (multiple sclerosis and neuromyelitis
optica). Pediatric multiple sclerosis is the most common demyelinating disorder in
children. Recent genetic and clinical researches have significantly improved our
understanding of the diverse spectrum of pediatric demyelinating disorders. In this
chapter, an updated summary of the current knowledge on the categories, diagnosis, as
well as management of pediatric demyelinating disorders has been presented.
Keywords: Adolescent, Central Nervous System/immunology, Central Nervous System/pathology, Child, Demyelinating Diseases/diagnosis, Demyelinating Diseases/drug therapy, Demyelinating Diseases/immunology, Demyelinating Diseases/pathology, Encephalomyelitis, Acute Disseminated / cerebrospinal fluid, Acute Disseminated/diagnosis, Humans, Magnetic Resonance Imaging, Multiple Sclerosis/cerebrospinal fluid, Multiple Sclerosis/diagnosis, Multiple Sclerosis/drug therapy, Myelin-Oligodendrocyte Glycoprotein / immunology, Neuromyelitis Optica/cerebrospinal fluid, Neuromyelitis Optica/diagnosis, Neuromyelitis Optica/drug therapy, Serologic Tests, Treatment Outcome.