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Demyelinating diseases
Are there other demyelinating diseases?
Yes, but they are rare. Multiple Sclerosis is the only common demyelinating disease in adults.
Other rare conditions include:
• Central pontine myelinolysis , a syndrome of myelin destruction in the pons, associated with rapid correction of hyponatremia.
• Progressive multifocal leukoencephalopathy , an opportunistic infection of oligodendrocytes by the John Cunningham (JC) virus, seen most often in patients with acquired immunodeficiency syndrome or other immunosuppression.
• Acute disseminated encephalomyelitis , a monophasic, acute, autoimmune demyelination producing encephalopathy, especially in childhood.
• Inborn errors of myelin metabolism , usually presenting in childhood:
• Metachromatic leukodystrophy, a deficiency of the enzyme aryl sulfatase
• Adrenoleukodystrophy, a defect in metabolism of very long chain fatty acids
• Krabbe’s globoid leukodystrophy, a deficiency of the enzyme galactosylceramidase
• Neuromyelitis optica or Devic’s disease , an autoimmune disease predominantly affecting the optic nerves and spinal cord. Patients have relapses, usually every few years, causing primarily visual and spinal cord deficits. Severe disability often results. Patients have antibodies to aquaporin-4, regulating a water channel on astrocytes, leading to inflammation and secondary demyelination.
Sources
- Sobel RA, Moore GRW: Demyelinating diseases. In Love S, Louis DN, Ellison DW (eds): Greenfield’s neuropathology , ed 8. London: Oxford University Press, pp. 1513-1608, 2008.
- Papadopoulos MC, Verkamn AS: Aquaporin 4 and neuromyelitis optica. Lancet Neurol 11(6):525-544, 2012.
