Encephalopathies Year 4 Paediatrics Notes OmpathStudy

Encephalopathies Year 4 · Paediatrics Collection: 4TH YEAR ALL NOTES / PAEDS / PAEDS 4.3 / Munge / CNS -- Slide 1 Encephalopathies Dr Rose Munge Slide 2 Encephalopathy is a generalized disorder of cerebral function that may be acute or chronic, progressive, or static.\n The etiologies of the encephalopathies in children include infectious, toxic (carbon monoxide, drugs, lead), metabolic, genetic, and ischemic causes. Slide 3 Cerebral Palsy Cerebral palsy (CP) is a diagnostic term used to describe a group of permanent disorders of movement and posture causing activity limitation that are attributed to nonprogressive disturbances in the developing fetal or infant brain. The motor disorders are often accompanied by disturbances of sensation, perception, cognition, communication, and behavior as well as by epilepsy and secondary musculoskeletal problems.\n CP is caused by a broad group of developmental, genetic, metabolic, ischemic, infectious, and other acquired etiologies that produce a common group of neurologic phenotypes. Slide 4 CP has historically been considered a static encephalopathy, but some of the neurologic features of CP, such as movement disorders and orthopedic complications, including scoliosis and hip dislocation, can change or progress over time.\n Many children and adults with CP function at a high educational and vocational level, without any sign of cognitive dysfunction Slide 5 Epidemiology and Etiology CP is the most common and costly form of chronic motor disability that begins in childhood\nThe incidence is 3.6 per 1,000 children with a male:female ratio of 1.4: 1. The Collaborative Perinatal Project, in which approximately 45,000 children were regularly monitored from in utero to the age of 7 yr, found that most children with CP had been born at term with uncomplicated labors and deliveries.\nIn 80% of cases, features were identified pointing to antenatal factors causing abnormal brain development. A substantial number of children with CP had congenital anomalies external to the central nervous system (CNS).\nFewer than 10% of children with CP had evidence of intrapartum asphyxia. Slide 6 Intrauterine exposure to maternal infection (chorioamnionitis, inflammation of placental membranes, umbilical cord inflammation, foul-smelling amniotic fluid, maternal sepsis, temperature 38°C during labor, urinary tract infection) was associated with a significant increase in the risk of CP in normal birthweight infants. Elevated levels of inflammatory cytokines have been reported in heelstick blood collected at birth from children who later were identified with CP.\n Genetic factors may contribute to the inflammatory cytokine response, and a functional polymorphism in the interleukin-6 gene is associated with a higher rate of CP in term infants Slide 7 The prevalence of CP has increased as a result of the enhanced survival of very premature infants weighing < 1,000 g, who go on to develop CP at a rate of approximately 15 per 100.\n The major lesions that contribute to CP in preterm infants are intracerebral hemorrhage and periventricular leukomalacia (PVL).\n Although the incidence of intracerebral hemorrhage has declined significantly, PVL remains a major problem. Slide 8 PVL reflects the enhanced vulnerability of immature oligodendroglia in premature infants to oxidative stress caused by ischemia or infectious/inflammatory insults. White matter abnormalities (loss of volume of periventricular white matter, extent of cystic changes, ventricular dilation, thinning of the corpus callosum) present on MRI at 40 wk of gestational age in former preterm infants are a predictor of later CP. Slide 9 The European Cerebral Palsy Study examined prenatal and perinatal factors as well as clinical findings and results of MRI in a contemporary cohort of more than 400 children with CP.\nMore than half the children with CP in this study were born at term, and less than 20% had clinical or brain imaging indicators of possible intrapartum factors such as asphyxia.\nThe contribution of intrapartum factors to CP is higher in some underdeveloped regions of the world.\nAntenatal infection was strongly associated with CP and 39.5% of mothers of children with CP reported having an infection during the pregnancy, with 19% having evidence of a urinary tract infection and 11.5% reporting taking antibiotics. Slide 10 Multiple pregnancy was also associated with a higher incidence of CP and 12% of the cases in the European CP study resulted from a multiple pregnancy, in contrast to a 1.5% incidence of multiple pregnancy in the study.\nOther studies have also documented a relationship between multiple births and CP, with a rate in twins that is 5-8 times greater than in singleton pregnancies and a rate in triplets that is 20-47 times greater.\nDeath of a twin in utero carries an even greater risk of CP; it is 8 times that of a pregnancy in which both twins survive and approximately 60 times the risk in a singleton pregnancy. Sli