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Demyelinating disease
Demyelinating disease is characteristically scattered throughout the brain and spinal cord, with small foci where the myelin is destroyed and replaced with glial scar tissue. Nerve fibres running through such areas may remain undamaged, but the lack of insulation normally provided by the myelin leads to a blockage of conduction of electrical impulses. In certain very rare diseases, known as leukodystrophies, large areas of demyelination occur in the white matter of the brain as a result of inherited metabolic disorders. Demyelination is also the result of an immune attack upon the central nervous system, which may follow viral infections such as measles, or of immune deficiency states such as pernicious anemia. Schwann cells, which form the myelin surrounding the axons of peripheral nerves, may suffer immune or toxic attack, as in Guillain-Barré syndrome and diphtheria. This also leads to a blockage of electrical conduction. When an injury is primarily to axons, the Schwann cells are also damaged, producing “secondary demyelination.”
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