ABNORMAL EXPRESSION OF CELL-ADHESION MOLECULE L1 IN MIGRATION DISORDERS - A DEVELOPMENTAL IMMUNOHISTOCHEMICAL STUDY

We studied immunohistochemically the expression pattern of a neural ce ll adhesion molecule, L1, in various human migration disorders associa ted with polymicrogyria: 4 fetuses and 11 infants having Fukuyama type congenital muscular dystrophy (FCMD) (4 cases), Zellweger syndrome (6 ) or thanatophoric dysplasia (3) and intrauterine brain damages (2) at different development stages, comparing to age-matched controls. Ther e were different patterns of L1 expression, which suggested at least 3 pathogenetic mechanisms: high expression associated with neuoraxonal overgrowth (fetal FCMD and destructive event at intermigratory period) ; delayed expression with neuronal dysmaturation and dysmyelinogenesis (late infantile stage of Zellweger syndrome); no expression in toxic or destructive brain injury (Zellweger syndrome or destructive events at inter- or postmigratory periods).