A case of early myoclonic encephalopathy with the congenital nephrotic syndrome

We describe a male infant with early myoclonic encephalopathy (EME) associated with the congenital nephrotic syndrome, microcephaly, multiple minor anomalies, and cerebellar hypoplasia. He had erratic and massive myoclonus, and partial seizures from the neonatal period. Electroencephalography showed the so-called suppression-burst pattern. He died of disseminated intravascular coagulation caused by the congenital nephrotic syndrome at the age of two months. Our patient is the first reported case with EME associated with the congenital nephrotic syndrome. (C) 1997 Elsevier Science Ireland Ltd.