Article

Hereditary sensorimotor neuropathies, or Charcot-Marie-Tooth disease (CMT)
comprise a group of diseases with heterogeneous clinical, electrophysiological
and genetic expression. They are classified by the mode of inheritance (autosomal
dominant, X-linked dominant, autosomal recessive) and their electrophysiological
characteristics taking into account the speed of motor conduction of the median
nerve (demyelinating, intermediary and axonal forms). Certain purely motor forms
are called spinal CMT or hereditary distal motor neuropathy, or distal spinal
amyotrophy. CMT involving an important sensorial component, trophic disorders, or
signs of dysautonomia are included in the classification of hereditary sensory
and autonomic neuropathies.
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Langue : FRANCAIS