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The congenital bilateral perisylvian syndrome is characterized by pseudobulbar palsy, moderate delay in mental and motor development and epilepsy. Three characteristic case stories are presented. Epileptic seizures are most frequently generalized: tonic, astatic, atypical absences and tonic-clonic seizures. Partial seizures are less frequent. Seizure control is often unsatisfactory. Neuroimaging demonstrates thickening of the cerebral cortex in the perisylvian area bilaterally; these changes together with the clinical picture establish the diagnosis. The etiology is unknown.

Fifty-two children with intractable epilepsy received lamotrigine as add-on therapy on a compassionate basis. The results were reviewed after three and six months of treatment in order to evaluate the efficacy in different epilepsy syndromes. Mental retardation was present in 60% of the children. Ictal EEG was obtained in 38 children. At three months the median monthly seizure frequency was reduced from 46 to 14 in the 37 children that still received lamotrigine (p < 0.01). Seven children were seizure-free. Seizure reduction was most impressive in generalized epilepsy, since 63% of these had more than 50% seizure reduction compared to 18% in partial epilepsies (p < 0.05). This difference was unchanged after six months of treatment. Side effects were reported in 18 of the children. In 13 children the parents reported an improved wellbeing. Lamotrigine seems to be an efficient antiepileptic drug-especially in generalized epilepsy.

The aim of The Cerebral Palsy Register is to follow the development of cerebral palsy in East Denmark as reported from the children's departments within the area. The prevalence among Danish children born in 1971-1982 is reported. Postnatal cases have been excluded. The live-birth prevalence has increased from 1.7 per 1000 in 1971-1974 to 2.3 per 1000 in 1979-1982. An increased proportion with birthweight under 2500 g and neuroimpairment was found in the latest birth year period. A re-evaluation of diagnostic information confirmed the increasing trend of the prevalence. Preterm births were dominated by perinatal etiology, while term births equally often had a prenatal etiology. It is worrying that the prevalence is rising and that the children have more disabilities. The reason for the increasing trend is most probably due to the decreasing neonatal mortality.

Pseudoepileptic seizures (pseudoseizures) are defined as episodic disturbances of behaviour with elements of epileptic seizures, but with atypical features and not accompanied by abnormal EEG discharges. They are frequently seen in patients with epilepsy, but also as the only disturbance of function. In children, the possible pathogenetic mechanisms are less understood than in adults. We present a series of cases, showing that an important factor in development of pseudoseizures lies in disturbances of communication between children and parents within the family. However, there are large interindividual variations in the most probable underlying mechanisms. Some recommendations as regards treatment are given.