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The Newborn «Neonatal Stroke»

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The Newborn «Neonatal Stroke» 1/25/2020 Neonatal (perinatal) stroke NICH-NINDS: “a group of heterogeneous conditions with focal disruption of cerebral blood flow secondary to arterial or cerebral venous thrombus or embolization between 20w of fetal life through the 28th postnatal day The newborn and confirmed by NEUROIMAGING or neuropathological studies.” «neonatal stroke» - It can be focal or multifocal and both ischaemic and Andrea Righini MD, Elisa Scola MD*, Cecilia Parazzini MD, Fabio Triulzi MD. PhD* haemorrhagic. Radiology and Neuroradiology Dept., Children’s Hospital V. Buzzi, Milan, Italy - The first week of life carries the highest period risk for *Neuroradiology Dept., University Hospital-Policlinic, Milan, Italy [email protected] stroke in paediatric age 1 2 Lancet Child Adolesc Classification Health. 2018. Perinatal stroke: «typical-common» arterial acute stroke «Typical-common» arterial acute stroke (AIS) mechanisms, AJNR 2009 management, and Evolution of Unilateral Perinatal Arterial Ischemic Stroke on Conventional outcomes of early «Atypical-uncommon» arterial acute stroke and Diffusion-Weighted MR Imaging J. Dudink et Al.. cerebrovascular brain and arterial acute ischemia–PLUS injury. Dunbar M et AL. T1 Deep medullary vein territory infarctions-haemorrhages 2d old Periventricular venous T2 infarction (mostly prematures) Sinus thrombosis and related haemorrhagic DD DWI infarctions Rapid necrosis (T1-hyper) Rapid and malacia (T1-CSF like signal) wallerian degen. 3 4 «typical-common» arterial acute stroke «typical-common» arterial acute stroke often asymptomatic or acute symptoms nonspecific presentation such as hypotonia, lethargy, apnea - perinatal arterial ischemic stroke (AIS) occurs in or feeding difficulties. around 1 in 1600 to 5000 births - Typically a term baby with a “normal “ «presumed perinatal stroke» prenatal history who - male predominance of approximately 60% appears well. - Seizures are the most haemorrhagic - left hemisphere predominates. common symptoms of arterial venous infarction AIS in term infants (25- ischemia 40%) - Hemiparesis is rarely periventricular left found in acute phase venous infarction late detection 5 6 1 1/25/2020 «typical-common» arterial acute stroke «typical-common» arterial acute stroke aetiologies Often not so «normal» history J Pediatr. 2016 In more than 50% of AIS the cause is never found. Risk Factors for Neonatal Arterial - More frequent family history of seizures/neurologicIschemic Stroke: The Normal neonates already have risk factors for thrombus formation including: raised Importance of the diseases, primiparous Intrapartum Period. hematocrit, presence of fetal hemoglobin, high procoagulant proteins, and increased blood Martinez-Biarge M et Al. viscosity. mothers, and male sex. Placental factors Maternal factors/condition Placental thrombosis - More intrapartum complications: prolonged rupture of Infertility and infertility treatment Placental abruption membranes, fever, thick meconium, prolonged second Autoimmune disorders Placental infection stage, tight nuchal cord, and abnormal Coagulation disorders (Protein C deficiency, Protein S deficiency, Factor V Leiden) cardiotocography. Anticardiolipin antibodies Fetal/ Neonatal Cardiac disorders Preeclampsia Congenital heart disease Placental abnormalities more common than Infection, chorioamniositis Patent ductus arteriosus J Pediatr. 2018 Prolonged rupture of membrane (>24h) in controls: Placental Pathology Pulmonary valve atresia in Neonatal Stroke: A Twin-to-twin transfusion syndrome Cardiac surgery (associated with cardiac - Fetal vascular malperfusion. Retrospective Case-Control Study. In utero cocaine exposure bypass, atrial balloon, septostomy) - Amniotic fluid inflammation Bernson-Leung ME et Al. 7 8 Thrombophilic factors? Arterial ischemic stroke topography Blood. 2017 NeurodevelopmentAfter Perinatal Arterial Ischemic Stroke posterior Thrombophilia risk is not increased in children after perinatal stroke. Nienke Wagenaar, M et Al.. PEDIATRICS 2018 anterior Curtis C et Al.. cerebral cerebral Thrombophilia evaluations: MCA territory 90% artery 9% artery 1% proteins C and S, antithrombin, factors VIII/IX/XI, fibrinogen, lipoprotein(a), homocysteine, lupus anticoagulant, anticardiolipin antibodies and genotyping of factor V Leiden (FVL), factor II G20210A (FII), and methylenetetrahydrofolate reductase C677T. ”minimal association between perinatal stroke and thrombophilia.” Multiple infarctions: 6% to 25% of perinatal are bilateral. Arch Pediatr. 2017. [Neonatal arterial ischemic stroke: Review of the current guidelines]. Saliba E The extent and location of the stroke influence the long-term AIS has a low recurrence rate (1%), except in children with congenital outcome. Therefore MRI is crucial for the assessment of the heart disease or multiple genetic thrombophilia. topography of the chronic infarct area. 9 10 Eur J Radiol. 2016 MR angiography findings «typical-common» arterial acute stroke MR-angio variability in infants with neonatal arterial ischemic stroke in the middle cerebral artery territory: A prospective Frequent post-rolandic involvement and prognosis issue MRA should be performed BUT often of the arterial defects study using circle of Willis MR angiography. Three-Dimensional Map of Neonatal Arterial Ischemic can quickly regress. Husson B et Al.. Stroke Distribution From Early Multimodal Brain Imaging “33 arterial changes, occlusion in 6 thrombus-type flow defect in 9, and Christian Stephan-Otto et Al.. Stroke. 2017 unilateral increased flow in enlarged insular arteries in 8”. “71% lesions posterior to central sulcus” complete carotid none to mild MCA asymmetry occlusion exemplificative cases remarkable MCA asymm. MCA ditation Dev Med Child Neurol. 2019 ”new evidence that children with remarkable periph. reactive Neuroanatomical correlates of sensory deficits in AIS may experience difficulty processing branches asymm. Hyper children with neonatal arterial ischemic stroke. perperfusion Giudice C et Al.. sensory information” MCA perf. arteries involvement (basal ganglia) 11 12 2 1/25/2020 «typical-common» arterial acute stroke» Pathophysiology spot Arterial Spin Labeling perfusion in AIS early phases of wallerian degeneration Arterial Spin Labeling Perfusion Magnetic Resonance Imaging Performed in Acute Perinatal 73% demonstrated Stroke Reveals very fast in neonates! Hyperperfusion Associated With hyperperfusion... Ischemic Injury Christopher G. Watson et Al. higher freq. than Stroke. 2016 adults. NEWBORN FOCAL INFARCTION (15 DAYS) ASL Reperfusion effect?, Excitoxicity? Seizures? FSE-T2 adult studies: lambda 1 lambda 2 and 3 (Thomalla G. et Al., 2004) pCASL-3D Fractional Anisotropy 13 14 Pathophysiology spot «typical-common» arterial acute stroke AIS secondary changes ADC and cortical neuron / axonal damage Acute changes are also seen within the medial thalamus and (early wallerian degeneration) hypothesis pulvinar, which are likely attributable to secondary degeneration of ACUTE SUBACUTE corticothalamic projections. AJNR 2009 Evolution of Unilateral Perinatal Arterial Ischemic Stroke on Conventional and Diffusion-Weighted MR Imaging J. Dudink et Al.. In the chronic phase contralateral cerebellar hemisphere atrophy may be seen as a result of crossed cerebellar diaschisis following MCA infarction. Cortical ADC pseudonormalization 15 16 DTI/RGB/FA maps may help in prognosis Differential diagn. I Signs of cortical-spinal tract degeneration are not good signs for Some of the more tricky cases for neuroradiologists motor prognosis Acute infective Encephalopathies 15d old 15d old 13d old no CST, but optic rad.degen. moderate to severe degen. moderate degen. neonat. Herpes encephalitis atypical but true hypoglycaemic encephalopathy* 5d old 4w old *hypoglycaemia itself is a risk factor for none to mild degen.? AIS or can be associated with AIS none to mild degen.? 5d old prolonged hypoglycaemia 17 18 3 1/25/2020 Differential diagn. II Some of the more tricky cases for neuroradiologists «shaken baby syndrome» cortical necrosis «Atypical» arterial acute stroke Molybdenum defic. and arterial acute ischemia–PLUS acute SBS phase especially if asymmetric Some neurometabolics: Clinical Neuroimaging Features and Outcome molybdenum defic., mitochondria in Molybdenum Cofactor Deficiency Kayal Vijayakumar MBBSa, et al.. Focal prolonged seizures (overlapping effect). ? 19 20 «Atypical» arterial acute stroke «Atypical» arterial acute ischemia and arterial acute ischemia–PLUS and arterial acute ischemia–PLUS AIS may present with uncommon features complete sparing of the cortex with cavitation of the underlying or with associated additional findings white matter was observed in 3 preterms Stroke. 2016 Risk Factors, Clinical Presentation, and Neuroimaging Cortical Sparing in Findings of Neonatal Perforator Stroke Preterm Ischemic Arterial Ginette M. Ecury-Goossen, et Al.. Stroke. 2013 Stroke. pure perforator art. stroke: van der Aa NE et Al. mostly detected by US, AIS-PLUS perivenular lesions in no heralding symptoms cortical mild unclear HIE history sparing 21 22 Deep medullary vein territory infarctions-haemorrhages - Premature and term newborn - Rarerly secondary to intracranial sinus thrombosis - Sometime associated with central venous hyepertension – jugular v. occlusion - May be associated with CHD Deep medullary vein territory - Unclear relation with perinatal asphyxia - Sometime with systemic infections infarctions-haemorrhages - Neonatal immature deep medullary vein system more susceptible than in adult. deep medullary veins:
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