UNUSUAL COURSE OF DE BARSY-MOENS-DIERCKX SYNDROME IN A NEONATE

We report about a premature infant with a De Barsy-Moens-Dierckx-syndr ome, which is a rare cutaneo-oculo-cerebral malformation-syndrome. It is defined by the combination of a progeroid aspect, cutis laxa, growt h retardation, cornea clouding, mental retardation and athetoid moveme nts. Furthermore, the reported case showed a remarkable thermolability and suffered from generalised seizures resistant to therapy. Despite extensive sonographic examinations the prepartal diagnosis seems to be very difficult.