BILATERAL, PERISYLVIAN AND ROLANDIC CORTICAL DYSPLASIA IN TRISOMY-13-SYNDROME

In patients with the trisomy 13 syndrome the most commonly encountered brain anomaly is holoprosencephaly, which occurs in approximately 80 % of cases. In trisomy 13 patients without holoprosencephaly, previous ly reported anomalies include callosal dysgenesis, hippocampal hypopla sia, olfactory hypoplasia, and cerebellar dysplastic changes such as v ermian hypoplasia and dysplastic cortices. Dysplasia of the cerebral c ortex, however, has not been reported before. We describe a newborn wi th bilateral, dysplastic cortices at the perisylvian and rolandic regi ons. These dysplastic cortices probably accounted for the clinical fin dings of seizures, oromotor dysfunction, dystonia and flexion contract ures in the hands, which were consistent with a recently described syn drome labelled as the ''congenital bilateral perisylvian syndrome''.