Search

The mechanism responsible for prenatal bowing of long bones was investigated based on the results of morphological observation and biochemical analysis of glycosaminoglycans of the autopsied bone from a newborn patient with classic campomelic dysplasia and the result of an experiment which produced a similar deformity in rat fetuses. An excised bone with postnatal bowing from a patient with osteogenesis imperfecta was also examined for comparison. In the case of campomelic dysplasia, marked hypertrophic change of the cambium layer of the periosteum at the concave side of the bone was noted. Parallel rays of the periosteal bone extended from the concave (posterior) side toward the apex of the bowing at the anterior side. Increased amounts and differences in composition of glycosaminoglycans were found in the diaphyseal bone on the concave side, suggesting the existence of bone with maturity retardation. In contrast, there were no such differences in the case with postnatal bowing and in the control. Congenital bowing of long bone in the patients with multiple malformations such as campomelic dysplasia is probably manifested as early as the cartilaginous model as the experimental result suggested.

• Electroclinical features of convulsive apnea and its relation to the behavioral state were described on the basis of polygraphic recordings from 21 newborns with various underlying disorders, including perinatal anoxia, purulent meningitis, and intracranial bleeding. The most frequent ictal discharges were rhythmic alpha waves, but other types of discharges, such as repeated sharp waves, rhythmic theta waves, delta waves, and repeated paroxysmal wave complexes, were also frequently seen. The area where the ictal discharges initially occurred or were most prominent was the temporal area, suggesting the limbic origin of apneic seizures. In more than half of the cases, the sleep cycle was abolished. In those cases where the sleep cycle was preserved, the seizures occurred most frequently in active sleep, but never in quiet sleep. ( Am J Dis Child 1982;136:980-984)

Neonatal findings and evolutionary courses of epileptic seizures were described in newborns who had seizures during the neonatal period and later developed epilepsy. Neonates with CNS dysgenesis ran the highest risk (80%) for subsequent epilepsy. About 30% of those who suffered from perinatal hypoxia and/or intracranial birth injury and 30% of those who had meningitis developed subsequent epilepsy. In perinatal brain injury, those who developed epilepsy showed more abnormal neurological and electro encephalographic findings than those who did not. The more abnormal the neonatal background EEG, the more frequently epileptic fits developed later. There was no such relationship in CNS dysgenesis. Myoclonic seizures were associated with the most severe brain damage. Newborns who later displayed West syndrome had also very abnormal neonatal EEG whereas those who manifested other types of fits more often had less abnormal neonatal EEGs. In West syndrome, hypsarrhythmia was preceded by focal or multifocal spikes, and closely temporally related with the onset of spasms, but there was a variety of intervals between the onset of clinical fits and EEG spikes in generalized or focal motor seizures.

SUMMARY Electroclinical features of neonatal seizures were studied in 215 neonates who developed seizures during the first three weeks of life. In 62 of them, clinical fits occurred during the actual polygraphic recording. The electro-clinical correlations were very variable, although there were some statistically significant associations between certain types of seizures and electrical discharges. The relation between seizures and sleep states was investigated in 40 infants who had more than three fits during the recording. The state in which more than 70% of seizures occurred was active sleep in 28% of the cases, quiet sleep in 13%, indeterminate sleep in 8%, and wakefulness in 8%. In 50%, seizures did not tend to occur in a given state. The authors thank Professor Suzuki for his continued interest and encouragement.