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.
Guzzetta
F
F
Istituto di Neuropsichiatria Infantile, Università di Messina, Policlinico Universitario, Italy.
Rodríguez
J
J
Deodato
M
M
Guzzetta
A
A
Ferrière
G
G
D000293:Adolescent; D001369:Axons / Q000648:ultrastructure; D002467:Cell Nucleus / Q000648:ultrastructure; D002648:Child; D002675:Child, Preschool; D003711:Demyelinating Diseases / Q000145:classification / Q000235:genetics / Q000473:pathology*; D005260:Female; D005808:Genes, Recessive; D015417:Hereditary Sensory and Motor Neuropathy / Q000145:classification / Q000235:genetics / Q000473:pathology; D006801:Humans; D007223:Infant; D007965:Leukodystrophy, Globoid Cell / Q000145:classification / Q000235:genetics / Q000473:pathology; D007966:Leukodystrophy, Metachromatic / Q000145:classification / Q000235:genetics / Q000473:pathology; D008297:Male; D008854:Microscopy, Electron; D009186:Myelin Sheath / Q000648:ultrastructure; D012583:Schwann Cells / Q000648:ultrastructure