Electrophysiologic features of inherited demyelinating neuropathies: a reappraisal.
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.
Lewis
R A
RA
Department of Neurology, Wayne State University School of Medicine, Detroit, Michigan 48201, USA. rlewis@med.wayne.edu
Sumner
A J
AJ
D002607:Charcot-Marie-Tooth Disease / Q000235:genetics* / Q000503:physiopathology*; D017630:Connexins / Q000235:genetics; D003711:Demyelinating Diseases / Q000235:genetics* / Q000503:physiopathology*; D004594:Electrophysiology; D006801:Humans; D018993:Myelin P0 Protein / Q000235:genetics; D009185:Myelin Proteins / Q000235:genetics; D000097002:Gap Junction beta-1 Protein
D017630:Connexins; D018993:Myelin P0 Protein; D009185:Myelin Proteins; D000097002:Gap Junction beta-1 Protein; C073687:PMP22 protein, human