Remarkable improvement in mitral valve regurgitation in paediatric eosinophilic granuloma with polyangiitis

We report the case of a 12-year-old boy who presented with acute heart failure due to newly developed mitral valve regurgitation. The boy's history combined with raised levels of inflammatory markers was suggestive of paediatric eosinophilic granuloma with polyangiitis. The echocardiographic course and rapid response to therapy are presented

Cortical Sparing in Preterm Ischemic Arterial Stroke

Background and Purpose-Residual injury after perinatal arterial ischemic stroke in the middle cerebral artery territory usually involves the loss of cortical gray matter and subcortical white matter. In this article, we describe a different pattern of residual injury after middle cerebral artery stroke in preterm-born infants, in which the cortex is spared. Methods-Magnetic resonance imaging scans of 40 infants (12 preterm and 28 full-term infants) with a large middle cerebral artery stroke were reviewed and correlated with outcome. Results-Complete sparing of the cortex with cavitation of the underlying white matter was observed in 3 preterm infants, and partial sparing was noted in another 4 late preterm-born infants. One full-term infant had partial cortical sparing, and all others showed no sparing. Overall, 86% developed a hemiplegia and 30% had a developmental quotient below 85, but this did not vary between the different types of cortical injury. Conclusions-The pattern of cortical injury after middle cerebral artery stroke changes with gestational age and may be related to maturational changes of the vascular system. Outcome did not vary between the different patterns of cortical injury

Prediction of visual field defects in newborn infants with perinatal arterial ischemic stroke using early MRI and DTI-based tractography of the optic radiation

Purpose Visual field (VF) defects are common sequelae of perinatal arterial ischemic stroke (PAIS). The aim of this study was to investigate the predictive value of magnetic resonance imaging (MRI) and diffusion tensor imaging (DTI) for VF defects following PAIS. Methods Nineteen infants with unilateral PAIS, who underwent conventional MRI (T1/T2) and DTI at three months of age and a VF examination later in life (median age 3.2 yrs) were included. Conventional T1-weighted MRI was used to assess asymmetry of the optic radiation (OR). DTI-based tractography of the bilateral OR was performed, and the average fractional anisotropy (FA), axial (λ1), radial (λ23) and mean diffusivity (MD) were extracted. Asymmetry of the OR on MRI and DTI was used as a predictor of VF defects using receiver operating characteristic (ROC) analysis. Results Of the 19 infants, nine had a normal VF, eight had a VF defect (six hemianopia and two quadrantanopia), and two had an inconclusive VF test. The presence or absence of a VF defect could be correctly predicted using conventional MRI assessment in the majority of the infants, with an area under the curve (AUC) of 0.90 (95% CI 0.66-0.99). Prediction based on DTI parameter asymmetry indices showed an AUC of 0.96 (95% CI 0.74-1.00), 0.78 (95% CI 0.52-0.94), 0.93 (95% CI 0.70-1.00) and 0.90 (95% CI 0.66-0.99) for FA, λ1, λ23 and MD, respectively. Conclusions VF defects following PAIS can be reliably predicted by assessment of asymmetry of the OR at three months on conventional MRI and DTI-based tractography with comparable predictive values. Conventional T1-weighted MRI can be used in clinical practice

Prediction of visual field defects in newborn infants with perinatal arterial ischemic stroke using early MRI and DTI-based tractography of the optic radiation

Purpose Visual field (VF) defects are common sequelae of perinatal arterial ischemic stroke (PAIS). The aim of this study was to investigate the predictive value of magnetic resonance imaging (MRI) and diffusion tensor imaging (DTI) for VF defects following PAIS. Methods Nineteen infants with unilateral PAIS, who underwent conventional MRI (T1/T2) and DTI at three months of age and a VF examination later in life (median age 3.2 yrs) were included. Conventional T1-weighted MRI was used to assess asymmetry of the optic radiation (OR). DTI-based tractography of the bilateral OR was performed, and the average fractional anisotropy (FA), axial (λ1), radial (λ23) and mean diffusivity (MD) were extracted. Asymmetry of the OR on MRI and DTI was used as a predictor of VF defects using receiver operating characteristic (ROC) analysis. Results Of the 19 infants, nine had a normal VF, eight had a VF defect (six hemianopia and two quadrantanopia), and two had an inconclusive VF test. The presence or absence of a VF defect could be correctly predicted using conventional MRI assessment in the majority of the infants, with an area under the curve (AUC) of 0.90 (95% CI 0.66-0.99). Prediction based on DTI parameter asymmetry indices showed an AUC of 0.96 (95% CI 0.74-1.00), 0.78 (95% CI 0.52-0.94), 0.93 (95% CI 0.70-1.00) and 0.90 (95% CI 0.66-0.99) for FA, λ1, λ23 and MD, respectively. Conclusions VF defects following PAIS can be reliably predicted by assessment of asymmetry of the OR at three months on conventional MRI and DTI-based tractography with comparable predictive values. Conventional T1-weighted MRI can be used in clinical practice

MR imaging for accurate prediction of outcome after perinatal arterial ischemic stroke : Sooner not necessarily better

BACKGROUND: Involvement of the corticospinal tracts after perinatal arterial ischemic stroke (PAIS) is strongly correlated with adverse motor outcome. METHODS: Two full-term infants with PAIS, with two early MRI scans available, are reported. RESULTS: Diffusion weighted imaging (DWI)-MRI, performed within 24 h following onset of seizures and repeated 48 h later, clearly showed restricted diffusion within the middle cerebral artery territory on both MRIs, but clear patterns of signal intensity changes in the descending corticospinal tracts on the second MRI only. CONCLUSION: Since involvement of the corticospinal tracts is essential for prediction of motor outcome, we may need to reconsider optimal timing of MR imaging for prediction of neurodevelopmental outcome after PAIS

Feasibility and safety of erythropoietin for neuroprotection after perinatal arterial ischemic stroke

Objective To perform a feasibility and safety study with recombinant human erythropoietin (rhEPO) in neonates with perinatal arterial ischemic stroke. Study design Neonates with a magnetic resonance imaging-confirmed perinatal arterial ischemic stroke (n = 21) were treated with 1000 IU/kg rhEPO immediately after diagnosis and at 24 and 48 hours after the first dose. Repeat magnetic resonance imaging was performed when the patients were 3 months of age. Coagulation and hematologic variables (red blood cells, white blood cells, platelet counts) were performed in the first week after initiation of treatment. We also compared 10 patients who were treated with rhEPO with 10 historic infants with perinatal arterial ischemic stroke matched for the involved arterial branch to investigate whether rhEPO reduces the residual size of the infarction and subsequent brain growth between first and second scan. Results Seizures were a first symptom in 20 of 21 neonates. Heart rate, blood pressure, and coagulation function were in the normal range, as were red blood cells, white blood cells, and platelet counts. In a subgroup of 10 rhEPO-treated neonates, no differences were detected in residual infarction volumes or neurodevelopmental outcome compared with their historical nontreated counterparts. Conclusions rhEPO in neonates with perinatal arterial ischemic stroke had no adverse effects on red blood cells, white blood cells, platelets counts, or coagulation. rhEPO, 3000 IU/kg in total, given during a 3-day period, appears to be a safe therapy. The beneficial effects remains to be demonstrated in a larger, randomized, double-blind, placebo-controlled trial. © 2014 Mosby Inc

Preterm infants with isolated cerebellar hemorrhage show bilateral cortical alterations at term equivalent age

The cerebellum is connected to numerous regions of the contralateral side of the cerebrum. Motor and cognitive deficits following neonatal cerebellar hemorrhages (CbH) in extremely preterm neonates may be related to remote cortical alterations, following disrupted cerebello-cerebral connectivity as was previously shown within six CbH infants. In this retrospective case series study, we used MRI and advanced surface-based analyses to reconstruct gray matter (GM) changes in cortical thickness and cortical surface area in extremely preterm neonates (median age = 26; range: 24.9-26.7 gestational weeks) with large isolated unilateral CbH (N = 5 patients). Each CbH infant was matched with their own preterm infant cohort (range: 20-36 infants) based on sex and gestational age at birth. On a macro level, our data revealed that the contralateral cerebral hemisphere of CbH neonates did not show less cortical thickness or cortical surface area than their ipsilateral cerebral hemisphere at term. None of the cases differed from their matched cohort groups in average cortical thickness or average cortical surface area in the ipsilateral or contralateral cerebral hemisphere. On a micro (i.e. vertex) level, we established high variability in significant local cortical GM alteration patterns across case-cohort groups, in which the cases showed thicker or bigger volume in some regions, among which the caudal middle frontal gyrus, insula and parahippocampal gyrus, and thinner or less volume in other regions, among which the cuneus, precuneus and supratentorial gyrus. This study highlights that cerebellar injury during postnatal stages may have widespread bilateral influence on the early maturation of cerebral cortical regions, which implicate complex cerebello-cerebral interactions to be present at term birth

Preterm infants with isolated cerebellar hemorrhage show bilateral cortical alterations at term equivalent age

The cerebellum is connected to numerous regions of the contralateral side of the cerebrum. Motor and cognitive deficits following neonatal cerebellar hemorrhages (CbH) in extremely preterm neonates may be related to remote cortical alterations, following disrupted cerebello-cerebral connectivity as was previously shown within six CbH infants. In this retrospective case series study, we used MRI and advanced surface-based analyses to reconstruct gray matter (GM) changes in cortical thickness and cortical surface area in extremely preterm neonates (median age = 26; range: 24.9-26.7 gestational weeks) with large isolated unilateral CbH (N = 5 patients). Each CbH infant was matched with their own preterm infant cohort (range: 20-36 infants) based on sex and gestational age at birth. On a macro level, our data revealed that the contralateral cerebral hemisphere of CbH neonates did not show less cortical thickness or cortical surface area than their ipsilateral cerebral hemisphere at term. None of the cases differed from their matched cohort groups in average cortical thickness or average cortical surface area in the ipsilateral or contralateral cerebral hemisphere. On a micro (i.e. vertex) level, we established high variability in significant local cortical GM alteration patterns across case-cohort groups, in which the cases showed thicker or bigger volume in some regions, among which the caudal middle frontal gyrus, insula and parahippocampal gyrus, and thinner or less volume in other regions, among which the cuneus, precuneus and supratentorial gyrus. This study highlights that cerebellar injury during postnatal stages may have widespread bilateral influence on the early maturation of cerebral cortical regions, which implicate complex cerebello-cerebral interactions to be present at term birth