Abstracts

CBPP describes a congenital brain malformation with particular features detected through MRI. This well known syndrome usually includes clinical signs such as developmental delay, pseudobulbar palsy and epilepsy. However, the intensity of each symptom can vary largelly among patients. We report fourteen patients with CBPP calling attention to the variability of clinical presentation. All patients had CBPP detected by MRI, relatively symmetrical in most of them (11 patients). In addition, bifrontal periventricular nodular heterotopia, bifrontal schizencephaly, and biparietal schizencephaly were associated in three cases. There were 8 males and 6 females. The pregnancy was uneventful in 7 cases, and some unexpected event was observed in 7 (toxoplasmosis was detected and treated during the 4th month in 1, vaginal bleeding in 2, hypertension in 1, polihydramnio in 1, unsuccessful premature labor in 2). Deliveries were at normal expected time in all but one patient, that born at 28 weeks[acute] gestation. Age at first consultation ranged from 3 months to 15 years (mean: 4.1 years). Six patients were referred for developmental delay, 5 for seizures and 6 for speech disturbance. Ten patients (71%) had microcephalic, and macrocephaly was observed in 1 (7.1%). Six patients were severely handycaped. Motor examination was normal in 3 patients. Pyramidal and/or extrapyramidal signs were observed in the remaining. Pseudobulbar signs were detected in all but one patient (93%). Nine patients (64.2%) had epilepsy whose onset varied between 2 days of life to 12 years (mean: 1.8 years, median: 1year). Three of these patients (33.3%) are controled with one antiepileptic drug. We conclude that CBPP is not a homogeneous syndrome in pediatric patients. In this series, 57% of patients does not have epilepsy, or present controled seizures. (Supported by FAPESP - Funda[ccedil]ao de Auxilio a Pesquisa do Estado de Sao Paulo.)