Demyelinating Hereditary Neuropathies in Children - a Morphometric and Ultrastructural-study

Twenty-three cases of hereditary demyelinating neuropathies are reported, 13 with different types of hereditary motor and sensory neuropathy (HMSN) and 9 with globoid cell or meta-chromatic leucodystrophies. Ultrastructural and morpho-metric studies showed some critical pathological features emphasizing: 1) the variability of the recessive forms of HMSN; 2) the morphological distinction between HMSN type I and type III; and 3) differences between these types of HMSN and other ''onion bulb'' neuropathies such as those found in leucodystrophies, which account for distinct underlying mechanisms.