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Table 4 Electroneurographical findings in affected, gray shading indicate men. Sensory nerve action potential (SNAP) (μV), compound muscle action potential (CMAP) (mV)

From: Two novel connexin32 mutations cause early onset X-linked Charcot-Marie-Tooth disease

Family member Sex Age at onset (yr) Age at examination (yr) Sensory Sural nerve Motor Peroneal nerve Motor Tibial nerve Sensory Median nerve Motor Median nerve EMG Chronic denervation
     SNAP CV CMAP CV CMAP CV SNAP CV CMAP/ CV  
Family 1     R L R L R L R L R L R L R L R L R L R L  
III-7 6 68 - - - - 3.3 1.7 43.8 37.0 - - - -          Present
IV-4 3 44 - - - - - - - - - - - -        33.5 36.6 Present
IV-8 10 46 - - - - 1 0.2 32.8 25.4 0.8 1.7 30.2 31.1 - - - - 1.0 0.3 36.8 34.6 Present
IV-12 6 36 - 0.6 - 30.4 - - - - 1.2 1.3 25.2 25.0          Present
IV-15 22 29 - - - - 5.5 4.0 39.5 41.9 4.0 5.8 44.1 46.9          Slightly
V-4 7 20 -   - 29.3 - - 31.8 31.7 - - 29.7 32.5          Present
Family 2                         
III-2 6 44 - -    1.5   29.0   0.5   30.0       2.5   44.0   Present
IV-1 4 37 - - - - - - - - - - - - - - - - 0.3 0.2 25.8 22.7 Present
IV-3 11 18        31.0       -   -     30.0*   Present
V-1   8 3.8   57,8   0.8 1.3 45.9 45.8              Not present
V-2 1 7 1.0   48.3   2.0 1.3 48.8 46.7              Not present
  1. - not recordable
  2. * ulnar nerve