Abstract

Encephalopathy in an infant within the first few days of birth often reflects a major neurological disorder with a significant risk of long-term disability. 1 Ellenberg JH Nelson KB Cluster of perinatal events identifying infants at high risk for death or disability. J Pediatr. 1988; 113: 546-552 Summary Full Text PDF PubMed Scopus (113) Google Scholar Determining the cause and prognosis remains challenging. Signs of neonatal encephalopathy include seizures, lethargy or coma, poor tone and feeding, and difficulty maintaining respiration; and early descriptions focused on intrapartum asphyxia as the primary cause. In 1976 Sarnat and Sarnat 2 Sarnat HB Sarnat MS Neonatal encephalopathy following fetal distress. Arch Neurol. 1976; 33: 696-705 Crossref PubMed Scopus (1979) Google Scholar pointed out the importance of staging encephalopathy with behavioural and electroencephalographic criteria, and 10 years later Levene et al 3 Levene MI Grindulis H Sands C Moore JR Comparison of two methods of predicting outcome in perinatal asphyxia. Lancet. 1986; 1: 67-69 Summary PubMed Google Scholar reported that rating the clinical severity of encephalopathy is better than the Apgar score for predicting neurological outcome after asphyxia. Evidence, especially from the Western Australian case-control study by Badawi et al, 4 Badawi N Kurinczuk JJ Keogh JM et al. Antepartum risk factors for newborn encephalopathy: the Western Australian case-control study. BMJ. 1998; 317: 1549-1553 Crossref PubMed Scopus (750) Google Scholar suggests that a diverse group of disorders, many beginning antenatally, in addition to asphyxia are associated with encephalopathy in newborn babies. These disorders include maternal and fetal infections, maternal thyroid and nutritional deficiencies, placental disorders, and genetic diseases including Prader-Willi and Angelman's syndromes, Rett's syndrome, myotonic dystrophy, non-ketotic hyperglycinaemia, thrombophilic disorders, brain malformations, and family history of epilepsy. 4 Badawi N Kurinczuk JJ Keogh JM et al. Antepartum risk factors for newborn encephalopathy: the Western Australian case-control study. BMJ. 1998; 317: 1549-1553 Crossref PubMed Scopus (750) Google Scholar , 5 Ellis M Manandhar N Manandhar DS Costello AM Risk factors for neonatal encephalopathy in Kathmandu, Nepal, a developing country: unmatched case-control study. BMJ. 2000; 320: 1229-1236 Crossref PubMed Scopus (106) Google Scholar , 6 Grether JK Nelson KB Maternal infection and cerebral palsy in infants of normal birth weight. JAMA. 1997; 278: 207-211 Crossref PubMed Google Scholar , 7 Geerdink N Rotteveel JJ Lammens M et al. MECP2 mutation in a boy with severe neonatal encephalopathy: clinical, neuropathological and molecular findings. Neuropediatrics. 2002; 33: 33-36 Crossref PubMed Scopus (55) Google Scholar , 8 Richer LP Shevell MI Miller SP Diagnostic profile of neonatal hypotonia: an 11-year study. Pediatr Neurol. 2001; 25: 32-37 Summary Full Text Full Text PDF PubMed Scopus (77) Google Scholar , 9 Smith A Wiles C Haan E et al. Clinical features in 27 patients with Angelman syndrome resulting from DNA deletion. J Med Genet. 1996; 33: 107-112 Crossref PubMed Scopus (90) Google Scholar In the Western Australian study, 28% of infants with encephalopathy had birth defects compared with 4% of controls, and these defects were judged to have contributed to the encephalopathy in 37% of these cases. 10 Felix JF Badawi N Kurinczuk JJ Bower C Keogh JM Pemberton PJ Birth defects in children with newborn encephalopathy. Dev Med Child Neurol. 2000; 42: 803-808 Crossref PubMed Google Scholar Origin and timing of brain lesions in term infants with neonatal encephalopathyAlthough our results cannot exclude the possibility that antenatal or genetic factors might predispose some infants to perinatal brain injury, our data strongly suggest that events in the immediate perinatal period are most important in neonatal brain injury. Full-Text PDF

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