Neuropathies with Deranged Metabolism

  • Hugo W. Moser


The WHO Task Force on Neuropathology has suggested the classification of neuropathies shown in Table 1.1 World-wide, the most common causes of neuropathy are leprosy, trauma, nutritional deficiency and alcoholism, and diabetes. Several comprehensive reviews are available.2–4 The first three categories are beyond the scope of this chapter. We focus here mainly on the inherited neuropathies and on the diabetic neuropathies. Although the inherited neuropathies do not approach in frequency those resulting from the main environmental causes (infection, trauma, nutritional deficiency), they are highly significant causes of disability. In a recent survey of 205 patients with neuropathy referred to the Mayo Clinic, it was found that 42% suffered from inherited neuropathies.5 In addition to their etiologic classification, neuropathies are subdivided in accordance with basic pathological mechanisms. These mechanisms are discussed in detail in standard reference works2,3 and are summarized in Fig. 1. Wallerian degeneration results from transection of the axon through trauma. In segmental demyelination, there is primary destruction of the myelin sheath, although the axon is left intact. In axonal degeneration, there is a metabolic derangement of the whole neuron, and the myelin sheath breaks down concomitantly with the axon. Most commonly, the distal portion of the axon is most severely affected (“dying back phenomenon”).


Nerve Growth Factor Schwann Cell Phytanic Acid Sural Nerve Familial Dysautonomia 
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Copyright information

© Plenum Press, New York 1985

Authors and Affiliations

  • Hugo W. Moser
    • 1
  1. 1.John F. Kennedy InstituteBaltimoreUSA

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