Table 1.
Clinical features
Abbreviations: BAEP = brainstem auditory evoked potential; CMCT = central motor conduction time; CMTES = Charcot-Marie-Tooth evaluation score (max score 28/28); CMTNS = Charcot-Marie-Tooth Neuropathy Score (max score 36/36); F = family; LL = lower limbs; N = normal; ND = not done; UL = upper limbs; VEP = visual evoked potential.
a
Weakness: − normal, + ≥4 in distal muscles, ++ <4 in distal muscles, +++ proximal weakness (knee flexion and extension, elbow flexion and extension, or above).
b
Pinprick and vibration sense: − normal, + reduced below wrist/ankle, ++ reduced below elbow/knee, +++ reduced at or above elbow/knee.