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INV10 Imaging in Childhood Stroke
12
Abstracts: Invited Speakers, Seventh European Paediatric Neurology Society (EPNS) Congress
Childhood stroke mortality is ranging from 3% for previously healthy children to 40% for children with preexisting systemic problems. Several studies show that more than half of the surviving children show moderate to severe neurological deficits, most frequently hemiparesis and dysphasia. Neurocognitive problems result as well: In our own prospective study the group mean IQ was normal (94), but ranging from 50 120. Performance IQ (93) was reduced compared to verbal IQ (101). Those children with normal IQ presented with abnormalities in visuospatial and memory functions and symptoms of ADHD. A quality of life surveillance showed significantly reduced ratings of certain aspects (autonomy, parents’ relation, social acceptance). In a functional MR study we detected that intra-hemispherical language reorganisation was functionally superior to trans-hemispherical reorganisation of visuo-spatial functions. This may explain better verbal than visuospatial outcome and poorer prognosis for preschool children (who have less dominant lateralisation of function and probably more trans hemispherical reorganisation) compared to school aged children. INV10 Imaging in Childhood Stroke D. Saunders *. Great Ormond Street Hospital, London, UK Abstract not available at time of printing INV11 Aicardi-Goutieres syndrome ` J.B.P. Stephenson *. Fraser of Allander Neurosciences Unit, Royal Hospital for Sick Children, Glasgow, UK Aicardi-Goutieres syndrome (AGS) has become one of the ` most intellectually exciting disorders known to medical science. The original description by Jean Aicardi and Francoise ¸ Goutieres of Paris in 1984 was of a progressive ` familial disorder of the central nervous system (CNS) with mild persistent cerebrospinal fluid (CSF) lymphocytosis, calcifications of central grey nuclei and deep white matter changes. Aicardi and Goutieres suspected autosomal reces` sive inheritance, but noted that their syndrome was easily confused with congenital infection. An additional similarity to viral infection (and to neurological systemic lupus erythematosus, SLE) was intrathecal synthesis of interferonalpha (IFNa) as shown by Pierre Lebon in 1988. Since the original description it has become apparent that AGS is neither constantly progressive nor always accompanied by CSF lymphocytosis. Chilblains (pernio or chilblain lupus) were first reported in AGS in 1995, in 2000 an AGS-like family was described with features of infantile SLE, and in 2003 Yanick Crow and others showed that Cree encephalitis (in which extra-neurological features especially immunological abnormalities are prominent) was allelic to AGS. Crow’s group have now identified four of the AGS genes (at least one remains to be discovered), AGS1 being a DNA exonuclease TREX1 and AGS2−4 being genes encoding ribonuclease H2 subunits (AGS2 = RNaseH2B, AGS3 = RNaseH2C, AGS4 = RNaseH2A). Crow’s further studies have shown that in some cases the phenotype can predict the genotype. From a biological perspective, the unification of the IFNa-mediated responses to endogenous “self” nucleic acids and to virus is a fascinating synthesis. INV12 Acute disseminated encephalomyelitis R.S. Rust *. Charlottesville, USA Abstract not available at time of printing
INV13 Movement disorders with video case presentations N. Nardocci *. Neuropsichiatria Infantile, Istituto Nazionale Neurologico “C Besta”, Milan, Italy Abstract not available at time of printing INV14 Mechanisms of grey and white matter injury in the immature brain H. Hagberg *. Goteborg, Sweden ¨ The etiology behind grey and white matter lesions is multifactorial but cerebral hypoxia-ischemia (HI) and infectioninflammation are likely contributors. There are evidence that brain injury after severe birth asphyxia develops with a considerable delay and recent hypothermia studies provide proof of concept that post-HI neuroprotective treatment is a clinical reality. However, we urgently need to achieve a better understanding of the pathogenesis. Mitochondria are key regulators of cell death through their role in energy metabolism and calcium homeostasis, their ability to release apoptogenic proteins and to produce reactive oxygen species. We find that secondary brain injury is preceded by impairment of mitochondrial respiration, intra-mitochondrial accumulation of calcium, outer membrane permeabilization, and release of pro-apoptotic proteins. These mediators will induce both caspase-dependent and caspase non-dependent cell death. Apoptosis-inducing factor (AIF) translocation to the nucleus and induction of chromatolysis is of particular importance. There are several pathogenetic inducers of mitochondrial impairment and permeabilization. Opening of the membrane permeability transition (MPT) pore appears less important in the immature brain, whereas an increased pro-/antiapoptotic Bcl-2 family protein balance and Bax-mediated permeabilization seems critical. Mitogen activated protein kinases (MAPK) like JNK, PI3 kinase/Akt and activation of poly(ADP ribose) polymerase are involved in regulating Bcl2 family protein interactions after hypoxia-ischemia, which may be amenable to pharmacological intervention. INV15 Cerebral monitoring in the neonatal intensive care unit L.S. de Vries *, M.C. Toet, L.G.M. van Rooij, F. Groenendaal, A.C. van Huffelen. Dept of Neonatology, Wilhelmina Children’s Hospital, Utrecht, the Netherlands During the last decade continuous monitoring of the brain has become more routine. Monitoring will mostly be performed with a simple device, using single or two channel amplitude integrated EEG (aEEG). This type of monitoring was introduced in the sixties for monitoring adults during bypass surgery. Immediate access to aEEG monitoring in full-term infants with neonatal encephalopathy (NE), often admitted at night or during the weekend is one of many advantages of the aEEG, which is easy to learn by senior and junior doctors as well as the nursing staff. aEEG will provide immediate information about the background activity and it has been shown that a depressed background pattern (burstsuppression, continuous low voltage or iso-electric) will even as early as at 3−6 hours after birth be a strong predictor of neurodevelopmental outcome at 2 years of age. This information will be required as soon as possible after birth for selection of patients for neuroprotective intervention, and for early prediction of neurodevelopmental outcome. aEEG will also be able to detect some, but not all ictal discharges. A rapid rise of both the lower and the upper margins of the aEEG tracing is suggestive of an ictal discharge. Seizures can be recognised as single seizures, repetitive seizures and as a status epilepticus. The latter usually looks like a “saw-tooth” pattern. Correct interpretation is greatly improved by simultaneous original EEG recording available
Abstracts: Invited Speakers, Seventh European Paediatric Neurology Society (EPNS) Congress
Childhood stroke mortality is ranging from 3% for previously healthy children to 40% for children with preexisting systemic problems. Several studies show that more than half of the surviving children show moderate to severe neurological deficits, most frequently hemiparesis and dysphasia. Neurocognitive problems result as well: In our own prospective study the group mean IQ was normal (94), but ranging from 50 120. Performance IQ (93) was reduced compared to verbal IQ (101). Those children with normal IQ presented with abnormalities in visuospatial and memory functions and symptoms of ADHD. A quality of life surveillance showed significantly reduced ratings of certain aspects (autonomy, parents’ relation, social acceptance). In a functional MR study we detected that intra-hemispherical language reorganisation was functionally superior to trans-hemispherical reorganisation of visuo-spatial functions. This may explain better verbal than visuospatial outcome and poorer prognosis for preschool children (who have less dominant lateralisation of function and probably more trans hemispherical reorganisation) compared to school aged children. INV10 Imaging in Childhood Stroke D. Saunders *. Great Ormond Street Hospital, London, UK Abstract not available at time of printing INV11 Aicardi-Goutieres syndrome ` J.B.P. Stephenson *. Fraser of Allander Neurosciences Unit, Royal Hospital for Sick Children, Glasgow, UK Aicardi-Goutieres syndrome (AGS) has become one of the ` most intellectually exciting disorders known to medical science. The original description by Jean Aicardi and Francoise ¸ Goutieres of Paris in 1984 was of a progressive ` familial disorder of the central nervous system (CNS) with mild persistent cerebrospinal fluid (CSF) lymphocytosis, calcifications of central grey nuclei and deep white matter changes. Aicardi and Goutieres suspected autosomal reces` sive inheritance, but noted that their syndrome was easily confused with congenital infection. An additional similarity to viral infection (and to neurological systemic lupus erythematosus, SLE) was intrathecal synthesis of interferonalpha (IFNa) as shown by Pierre Lebon in 1988. Since the original description it has become apparent that AGS is neither constantly progressive nor always accompanied by CSF lymphocytosis. Chilblains (pernio or chilblain lupus) were first reported in AGS in 1995, in 2000 an AGS-like family was described with features of infantile SLE, and in 2003 Yanick Crow and others showed that Cree encephalitis (in which extra-neurological features especially immunological abnormalities are prominent) was allelic to AGS. Crow’s group have now identified four of the AGS genes (at least one remains to be discovered), AGS1 being a DNA exonuclease TREX1 and AGS2−4 being genes encoding ribonuclease H2 subunits (AGS2 = RNaseH2B, AGS3 = RNaseH2C, AGS4 = RNaseH2A). Crow’s further studies have shown that in some cases the phenotype can predict the genotype. From a biological perspective, the unification of the IFNa-mediated responses to endogenous “self” nucleic acids and to virus is a fascinating synthesis. INV12 Acute disseminated encephalomyelitis R.S. Rust *. Charlottesville, USA Abstract not available at time of printing
INV13 Movement disorders with video case presentations N. Nardocci *. Neuropsichiatria Infantile, Istituto Nazionale Neurologico “C Besta”, Milan, Italy Abstract not available at time of printing INV14 Mechanisms of grey and white matter injury in the immature brain H. Hagberg *. Goteborg, Sweden ¨ The etiology behind grey and white matter lesions is multifactorial but cerebral hypoxia-ischemia (HI) and infectioninflammation are likely contributors. There are evidence that brain injury after severe birth asphyxia develops with a considerable delay and recent hypothermia studies provide proof of concept that post-HI neuroprotective treatment is a clinical reality. However, we urgently need to achieve a better understanding of the pathogenesis. Mitochondria are key regulators of cell death through their role in energy metabolism and calcium homeostasis, their ability to release apoptogenic proteins and to produce reactive oxygen species. We find that secondary brain injury is preceded by impairment of mitochondrial respiration, intra-mitochondrial accumulation of calcium, outer membrane permeabilization, and release of pro-apoptotic proteins. These mediators will induce both caspase-dependent and caspase non-dependent cell death. Apoptosis-inducing factor (AIF) translocation to the nucleus and induction of chromatolysis is of particular importance. There are several pathogenetic inducers of mitochondrial impairment and permeabilization. Opening of the membrane permeability transition (MPT) pore appears less important in the immature brain, whereas an increased pro-/antiapoptotic Bcl-2 family protein balance and Bax-mediated permeabilization seems critical. Mitogen activated protein kinases (MAPK) like JNK, PI3 kinase/Akt and activation of poly(ADP ribose) polymerase are involved in regulating Bcl2 family protein interactions after hypoxia-ischemia, which may be amenable to pharmacological intervention. INV15 Cerebral monitoring in the neonatal intensive care unit L.S. de Vries *, M.C. Toet, L.G.M. van Rooij, F. Groenendaal, A.C. van Huffelen. Dept of Neonatology, Wilhelmina Children’s Hospital, Utrecht, the Netherlands During the last decade continuous monitoring of the brain has become more routine. Monitoring will mostly be performed with a simple device, using single or two channel amplitude integrated EEG (aEEG). This type of monitoring was introduced in the sixties for monitoring adults during bypass surgery. Immediate access to aEEG monitoring in full-term infants with neonatal encephalopathy (NE), often admitted at night or during the weekend is one of many advantages of the aEEG, which is easy to learn by senior and junior doctors as well as the nursing staff. aEEG will provide immediate information about the background activity and it has been shown that a depressed background pattern (burstsuppression, continuous low voltage or iso-electric) will even as early as at 3−6 hours after birth be a strong predictor of neurodevelopmental outcome at 2 years of age. This information will be required as soon as possible after birth for selection of patients for neuroprotective intervention, and for early prediction of neurodevelopmental outcome. aEEG will also be able to detect some, but not all ictal discharges. A rapid rise of both the lower and the upper margins of the aEEG tracing is suggestive of an ictal discharge. Seizures can be recognised as single seizures, repetitive seizures and as a status epilepticus. The latter usually looks like a “saw-tooth” pattern. Correct interpretation is greatly improved by simultaneous original EEG recording available