[Actual nosology of polyneuropathies in childhood]

The current nosological classification and terminology of the hereditary peripheral neuropathies in childhood are in a instable state. Linkage of the genes of these disorders to chromosomes 1, 8, 17 and X and the finding of altered peripheral myelin proteins are open the way to understand their physiopathology. The genetic heterogeneity and variability in the hereditary pattern obligates to reconsider the nosology of these disorders specially concerning the so called Déjerine-Sottas disease. In this paper a short review of the actual situation of the hereditary neuropathies with motor and sensory disturbances is made.