Neurological Context of Charcot-Marie-Tooth Disease: Implication of Molecular Mechanisms and Therapeutical Approaches

Hereditary neuropathies represent a genetically heterogeneous group of diseases that affect the Peripheral Nervous System (PNS). The most common form is the Charcot-Marie-Tooth disease (CMT), also called Hereditary Motor and Sensory Neuropathy (HMSN), with a reported prevalence of one in 2,500 people worldwide. Almost 125 years have elapsed since the fi rst contemporary description of the same familial neurological syndrome, “peroneal muscular atrophy”, by two French doctors (Charcot and Marie) and an English doctor (Tooth) (Banchs et al., 2009). In general, this syndrome has an infantile or juvenile onset, with motor and sensory polyneuropathic semiology and pes cavus (Harding and Thomas, 1980).