- Discussion:
    - includes the hypertrophic neuropathy of infancy (Dejerine-Scottas);
    - autosomal recessive trait;
    - begins in infancy;
    - EMG in demyelinating neuropathies:
           - marked slowing in nerve conduction velocity which is more extensive than those seen in type I or type II;
    - biopsy
           - biopsy of a distal muscle such as the gastrocnemius demonstrates a neuropathic pattern;
           - in pts for whom dx is unclear, definitive categorization is aided by biopsy of sural nerve, which demonstrates demyelination;

- Clinical Manifestations:
    - absent reflexes;
    - sensory loss is of stocking glove type (particularly w/ light touch, position & vibration sense);
    - hallmarks of these disorders involve muscle weakness of hands & feet;
           - pes cavus and drop foot deformities;
    - significant spinal deformity develops in most patients, & confinement to a wheel chair is common