Neonatal seizures with tonic clonic sequences and poor developmental outcome.

Seizures consisting of a tonic followed by a clonic phase have rarely been described in neonates and are not included in the current classifications of neonatal seizures. Our video archive of 105 neonates with seizures or suspected seizures revealed six neonates with such tonic clonic or tonic myoclonic sequences. Two of those neonates had pyridoxine dependent seizures. The other four neonates had drug refractory seizures and demonstrated similarities in electro-clinical pattern, clinical course and outcome. Their seizures started with tonic posturing and after 10-20s tonic posturing was superimposed by focal or multifocal cloni or myocloni. Ictal EEG started with voltage attenuation followed by bilateral or alternating focal epileptic discharges. The interictal EEG was abnormal. One child died, while the other three children became seizure free but had severe motor delay and mental retardation. In one of those three children, a de novo missense mutation was detected in the voltage gated potassium channel gene KCNQ2, indicating a genetic relationship between drug refractory neonatal seizures of unknown etiology with tonic clonic or myoclonic sequences and the well-known syndrome of benign familial neonatal convulsions (BFNC).

Improved outcome for very low birth weight multiple births.

This study describes time trends for very low birth weight multiple births in relation to very low birth weight singletons. Two cohorts of very low birth weight (less than 1250 gm) children recruited between 1983-85 (cohort 1, n = 115) and 1992-94 (cohort 2, n = 144) were compared. The Bayley Scales of Infant Development and a standardized neurologic examination were administered at 2 years corrected age. Neurodevelopmental outcome did not change between cohort 1 and 2 for singletons. For multiple births, mean Mental Developmental Index increased after adjustment for neonatal risk factors [adjusted mean (standard deviation) 81.8 (11.7) to 96.5 (18.6), analysis of covariance P = 0.007]. The prevalence of cerebral palsy decreased, however not significantly [adjusted odds ratio (95% confidence interval) 0.3 (0.1-1.5), P = 0.14]. The proportion of disease-free survival (no cerebral palsy and no developmental delay) increased for multiple births (7-37%, P = 0.002), but not for singletons. In cohort 2, neurodevelopmental outcome of multiple births was similar to that of singletons. The cognitive outcome of very low birth weight multiple births improved, possibly because of changes in perinatal practice. However, neurodevelopmental outcome was similar to that of very low birth weight singletons who were unaffected by changes in neonatal care with high proportions of motor delay and cerebral palsy.

Is exposure to cocaine or cigarette smoke during pregnancy associated with infant visual abnormalities?

The aim of this study was to assess the association between cocaine or cigarette smoke exposure in utero and visual outcome. A total of 153 healthy infants (89 males, 64 females; gestational age range 34 to 42 weeks) were prospectively enrolled in a masked, race-matched study. Quantitative analyses of urine and meconium were used to document exposure to cigarette smoke and cocaine. Infants with exposure to other illicit drugs, excepting marijuana, were excluded. At 6 weeks of age, grating acuity and visual system abnormalities (VSA; eyelid oedema, gaze abnormalities, and visual inattention) of 96 infants from the original study sample were assessed with the Teller acuity card procedure and a detailed neurological examination. Neither cocaine nor cigarette smoke exposure was associated with acuity or VSA. However, VSAs were associated with abnormal neurological examination, independent of drug exposure and other risk factors (odds ratio 7.9; 95% confidence interval 2.0 to 31.5;p=0.004). This unexpected finding could prove a helpful clinical marker for the infant at risk for neurological abnormalities.