Molecular diagnostic testing in Charcot-Marie-Tooth disease and related disorders. Approaches and results

Ann N Y Acad Sci. 1999 Sep 14:883:389-96.

Abstract

The inherited neuropathies of the peripheral nervous system are clinically and genetically a heterogeneous group of disorders. Molecular genetic studies have made major breakthroughs in unraveling the underlying gene defects, and DNA diagnosis can now be offered to a large number of families with distinct forms of hereditary peripheral neuropathies. With the currently available technology, however, molecular genetic diagnosis still remains a labor-intensive and costly procedure. We have developed an algorithm for mutation screening based on clinical phenotype, electrophysiological findings, and the relative frequencies of mutations in the distinct peripheral myelin genes.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Charcot-Marie-Tooth Disease / diagnosis*
  • Charcot-Marie-Tooth Disease / genetics*
  • Female
  • Gene Frequency
  • Genetic Testing / methods
  • Genetic Testing / standards
  • Guidelines as Topic
  • Humans
  • Male
  • Mutation