Migration disorders leading to a wide spectrum of brain malformations in a case with multiorganic dysgeneses: A new syndrome?
A case of a preterm infant who died with multiorgan, mainly cerebro-oculo-cutaneous malformations is presented. The brain dysgenesias consist of early disturbances of neuronal migration. They result on appearance of nodular subcortical heterotopias, cortical anomalies including pachy- and polymicrogyria and focal intrusion of numerous abnormally migrating nerve cells into leptomeninges. A various degree of nerve and glial cell maturity was observed within heterotopic tissue. The other malformations include eye, skin and internal organs anomalies. Similarities and differences between our case and another previously described cases were discussed but it seems difficult to include the analysed case into one of the known syndromes.
Dambska
M
M
Laboratory of Developmental Neuropathology Medical Research Centre, Polish Academy of Sciences, Warszawa.
Kuchna
I
I
Bobkiewicz
P
P
Wisniewski
K
K
D001921:Brain / Q000002:abnormalities*; D002465:Cell Movement; D002828:Choristoma; D005260:Female; D005904:Glial Fibrillary Acidic Protein; D006801:Humans; D007231:Infant, Newborn
D005904:Glial Fibrillary Acidic Protein