Charcot-Marie-Tooth Disease and Other Peripheral Neuropathies
- Pp. 269-325 (57)Veronick Benoy, Constantin d`Ydewalle and Ludo Van Den Bosch
Peripheral nerves connect the central nervous system with peripheral tissues in the body and are therefore crucial for all living animals to communicate with the environment. Due to the length of their axons, peripheral neurons are extremely vulnerable to insults. Inherited peripheral neuropathies comprise a large group of disorders characterized by progressive loss of axons or myelin that affect motor, sensory and/or autonomic nerves. Charcot-Marie-Tooth disease is the most common form of these inherited peripheral neuropathies. Peripheral nerves can also be damaged by a wide variety of stressors such as inflammation, infection, trauma, systemic disease, toxins/drugs and metabolic disturbances giving rise to several clinical subtypes of the disease. These disorders are referred to as acquired peripheral neuropathies. Ongoing research is focused on unraveling the pathogenic mechanisms underlying these debilitating diseases in order to find possible therapeutic strategies. So far, no drug therapy has been proven effective and patients have to rely on symptomatic treatments that are largely insufficient. Although there is no existing cure for peripheral neuropathies to date, some encouraging advances have been made which are also discussed in this chapter.