- See:
CMT Menu:
- 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;
