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Disorders of Myelin

  • Larry E. DavisEmail author
  • Sarah Pirio Richardson
Chapter

Abstract

The chapter begins with a discussion of common major clinical features and mechanisms of damage to myelin. Myelin is produced in the peripheral nervous system (PNS) by Schwann cells and in the central nervous system (CNS) by oligodendrocytes. Key functions of myelin are to house axons and to provide for the axons hollow tubular channels of extracellular matrix, provide physical strength to the axon, insulate the axon from environmental toxins, and allow saltatory conduction increasing nerve conduction velocity as much as 100-fold. The four major mechanisms of demyelination are death of oligodendrocyte or Schwann cell, interference with myelin synthesis, interference with myelin turnover, and immune-mediated destruction of myelin. In the CNS, myelin tracts commonly damaged are the corticospinal tract (weakness, spasticity), spinothalamic tract (sensory loss), visual pathway (visual disturbance), and spinocerebellar pathways (ataxia). In the PNS, motor (flaccid weakness) and sensory (position sense loss) are often involved. The chapter then discusses multiple sclerosis, neuromyelitis optica, acute disseminated encephalomyelitis, and Guillain–Barré syndrome, with attention to their pathophysiology, major clinical features, major laboratory findings, and principles of management and prognosis.

Keywords

Myelin Multiple sclerosis Neuromyelitis optica Devicʼs disease Guillain–Barré syndrome Acute inflammatory demyelinating polyneuropathy Acute disseminated encephalomyelitis 

Recommended Reading

  1. Bradl M, Lassmann H. Oligodendrocytes: biology and pathology. Acta Neuropathol. 2010;119:37–53. (Nice review of oligodendrocyte and myelin development with a focus on types of their pathology).Google Scholar
  2. Prineas JW, Parratt JDE. Oligodendrocytes and the early multiple sclerosis lesion. Ann Neurol. 2012;72:18–31. (Excellent careful review of possible pathogenetic mechanisms in the development of an acute MS plaque).Google Scholar
  3. Pelletier D, Hafler DA. Fingolimod for multiple sclerosis. N Engl J Med. 2012;366:339–47. (Nice review of the first oral anti-MS drug and its current indications).Google Scholar
  4. Nandhagopal R, Al-Asmi A, Gujjar AR. Neuromyelitis optica: an overview. Postgrad Med J 2010;86:153–9. (Clear review of the clinical, pathological neuroimaging, and management plus a good review of the role aquaporin 4 antibodies in the pathogenesis).Google Scholar
  5. Yuki N, Hartung H-P. Guillain-Barre Syndrome. N Engl J Med 2012;366:2294–304. (Nice review of the clinical features, proposed mechanisms of immunopathogenesis, treatment and prognosis of GBS).Google Scholar
  6. Tenembaum S, Chitnis T, Ness J, et al. Acute disseminated encephalomyelitis. Neurology 2007;68(suppl2): S23–36. (Reviews epidemiology, clinical features, neuroimaging, and treatment of ADEM).Google Scholar

Copyright information

© Springer Science+Business Media, LLC 2015

Authors and Affiliations

  1. 1.Chief Neurology ServiceDistinguished Professor of Neurology New Mexico VA Health Care SystemAlbuquerqueUSA
  2. 2.Department of Neurology Health Sciences CenterUniversity of New MexicoAlbuquerqueUSA

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