Demyelinating hereditary neuropathies in

Abstract

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 critica1 pathological features emphasizing: 1) the variability of the recessive forms of HMSN; 2) the morphological distinction between HMSN type 1 and type 111; 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.