A CASE OF EARLY MYOCLONIC ENCEPHALOPATHY WITH THE CONGENITAL NEPHROTIC SYNDROME

We describe a male infant with early myoclonic encephalopathy (EME) as sociated with the congenital nephrotic syndrome, microcephaly, multipl e minor anomalies, and cerebellar hypoplasia. He had erratic and massi ve myoclonus, and partial seizures from the neonatal period. Electroen cephalography showed the so-called suppression-burst pattern. He died of disseminated intravascular coagulation caused by the congenital nep hrotic syndrome at the age of two months. Our patient is the first rep orted case with EME associated with the congenital nephrotic syndrome. (C) 1997 Elsevier Science Ireland Ltd.