The observation that inherited demyelinating neuropathies tend
to have uniform conduction slowing and acquired disorders (CIDP
and variants) have nonuniform or multifocal slowing was made
before the identification of genetic defects of specific myelin
constituents that cause the different forms of Charcot-Marie-Tooth
and other inherited disorders involving peripheral nerve myelin.
It is becoming clear that the electrophysiologic aspects of
these disorders are more complex than previously realized. We
review the current information available on the electrophysiologic
features of the inherited demyelinating neuropathies in hopes
of clarifying the clinical electrodiagnostic features of these
disorders as well as to shed light on the physiologic consequences
of the different genetic mutations.
