ABNORMALITY OF CEREBRAL GANGLIOSIDES IN FUKUYAMA TYPE CONGENITAL MUSCULAR-DYSTROPHY

Compared with DMD cases and non-neuromuscular disease controls, FCMD c ases showed a reduction of total gangliosides, and an abnormal, immatu re ganglioside pattern in the cerebral gray and white matter. However, G(M4), which is only found in myelin and oligodendroglia, and is a un ique quantitative marker of myelination, was present in a relatively h igh percentage in the white matter, which showed frontal lobe micropol ygyria and diffuse low density on CT and MR T-1-imaging.