Brain Malformation of the Surviving Twin of Intrauterine Co-twin Demise

Abstract

The authors report the case of a surviving twin patient with severe brain malformation due to intrauterine fetal demise of the other twin. The patient was a boy with 37 weeks of gestational age. Intrauterine death of the co-twin was discovered at 18 weeks of gestation. No major anomalies were recognized at birth except for microcephalus. Magnetic resonance imaging demonstrated wide sylvian fissures, bilateral ventriculomegaly, and smooth brain surface of the temporoparieto-occipital lobes. He had spastic quadriplegia and severe mental retardation. Brief tonic seizures appeared at 7 months of age. Clonazepam was administered, and seizures disappeared at 24 months of age. Complex partial seizures began at 8 years of age. Seizures were observed weekly despite treatment with carbamazepine. The malformation of our patient was considered to be a result of a mixture of destructive process and disorders of neuronal proliferation and migration.