Can. J. Neurol. Sci. 2001; 28: 199-214

 Figure 1: Pes cavus. Twenty-year-old male with CMT 1A. This patient has high plantar arches with hammer toes and atrophy of foot muscles.

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 Figure 2: Uniform versus nonuniform conduction slowing of median motor nerve conduction in demyelinating neuropathies. Compound muscle action potentials were recorded from abductor pollicis brevis with stimulation at the wrist (top traces) and elbow (bottom traces). Panel A shows uniform slowing along the forearm segment in a patient with CMT 1A. Panel B shows nonuniform slowing with temporal dispersion in a patient with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). CV=conduction velocity; DL=distal latency.

 Figure 3: Onion bulb formation. Electron micrograph of sural nerve from an individual with CMT 1A showing characteristic concentric Schwann cell cytoplasmic processes surrounding a myelinated axon.

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Figure 4: CMTX. The pedigree in the top panel demonstrates maternal transmission of a mutant connexin 32 gene to a son and daughter. This family was shown to have a codon 3 mutation in the connexin 32 gene by polymerase chain reaction amplification followed by TaqI restriction analysis. The G'C mutation in codon 3 creates a novel TaqI restriction site. The mutant allele was detected in patients III-1, III-2, II-2 and I-1 (middle panel) by the presence of a 474 base pair fragment (lower panel). [From Gupta S, Benstead T, Neumann P, Guernsey D. A point mutation in codon 3 of connexin-32 is associated with X-linked Charcot-Marie-Tooth neuropathy. Hum Mutat 1996;8(4):375-376. Reprinted by permission of Wiley-Liss, Inc., a subsidiary of John Wiley & Sons, Inc.]

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 Figure 5: Hereditary neuropathy with liability to pressure palsies. Semithin section of sural nerve demonstrating several fibres within a fascicle surrounded by focal reduplication of the myelin sheath (arrows). (Methylene blue, 400X)

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