Purpose
In moyamoya vasculopathy, prolonged arterial transit time may increase the arterial spin labeling (ASL) signal heterogeneity, which can be quantitatively expressed by the spatial coefficient ...of variation of ASL-CBF (ASL-sCoV). The aim was to compare the accuracy of ASL-sCoV and ASL-CBF with dynamic susceptibility contrast (DSC)-CBF and time-to-peak (DSC-TTP) in the evaluation of perfusion changes and clinical outcome after encephalo-duro-arterio-myo-synangiosis (EDAMS) in pediatric moyamoya patients.
Methods
A total of 37 children with moyamoya vasculopathy (mean age 6.31 years (1.12–15.42)) underwent ASL and DSC perfusion imaging at 3T before and up to 24 months after EDAMS. Mean DSC-CBF, mean DSC-TTP, mean ASL-CBF, and ASL-sCoV were calculated in middle cerebral artery territories. Generalized linear model analyses were used to evaluate temporal variations of postoperative perfusion changes and to compare these variations between patients developing valid pial collateralization and those without angiographic improvement. Relationship between perfusion parameters and clinical outcome after surgery was tested using multivariate regression analysis.
Results
Significant reduction was observed after EDAMS for ASL-sCoV (
P
= .002; eta-squared (η
2
) = 0.247) and DSC-TTP (
P
< .001; η
2
= 0.415), whereas only a trend of increase was observed for DSC-CBF and ASL-CBF, with larger discrepancy before and 6 months after surgery. At last follow-up, children developing pial collateralization showed lower absolute ASL-sCoV (
P
= .002 Cohen’s d = 0.84) and DSC-TTP (
P
= .027; Cohen’s d = 0.64) and higher DSC-CBF (
P
= .002; Cohen’s d = − 0.55) compared with those without vascular improvement. Low preoperative and early post-surgical ASL-sCoV predicted better long-term neurological outcome (
P
< .001; ß = − 0.631).
Conclusions
ASL-sCoV may contribute to predict surgical outcomes in pediatric moyamoya patients undergoing EDAMS.
Background
Germinal matrix‐intraventricular hemorrhage (GMH‐IVH) is a common form of intracranial hemorrhage occurring in preterm neonates that may affect normal brain development. Although the ...primary lesion is easily identified on MRI by the presence of blood products, its exact extent may not be recognizable with conventional sequences. Quantitative susceptibility mapping (QSM) quantify the spatial distribution of magnetic susceptibility within biological tissues, including blood degradation products.
Purpose/Hypothesis
To evaluate magnetic susceptibility of normal‐appearing white (WM) and gray matter regions in preterm neonates with and without GMH‐IVH.
Study Type
Retrospective case‐control.
Population
A total of 127 preterm neonates studied at term equivalent age: 20 had mild GMH‐IVH (average gestational age 28.7 ± 2.1 weeks), 15 had severe GMH‐IVH (average gestational age 29.3 ± 1.8 weeks), and 92 had normal brain MRI (average gestational age 29.8 ± 1.8 weeks).
Field Strength/Sequence
QSM at 1.5 Tesla.
Assessment
QSM analysis was performed for each brain hemisphere with a region of interest‐based approach including five WM regions (centrum semiovale, frontal, parietal, temporal, and cerebellum), and a subcortical gray matter region (basal ganglia/thalami).
Statistical Tests
Changes in magnetic susceptibility were explored using a one‐way analysis of covariance, according to GMH‐IVH severity (P < 0.05).
Results
In preterm neonates with normal brain MRI, all white and subcortical gray matter regions had negative magnetic susceptibility values (diamagnetic). Neonates with severe GMH‐IVH showed higher positive magnetic susceptibility values (i.e. paramagnetic) in the centrum semiovale (0.0019 versus ‐0.0014 ppm; P < 0.001), temporal WM (0.0011 versus ‐0.0012 ppm; P = 0.037), and parietal WM (0.0005 versus ‐0.0001 ppm; P = 0.002) compared with controls. No differences in magnetic susceptibility were observed between neonates with mild GMH‐IVH and controls (P = 0.236).
Data Conclusion
Paramagnetic susceptibility changes occur in several normal‐appearing WM regions of neonates with severe GMH‐IVH, likely related to the accumulation of hemosiderin/ferritin iron secondary to diffusion of extracellular hemoglobin from the ventricle into the periventricular WM.
Level of Evidence: 4
Technical Efficacy: Stage 3
J. Magn. Reson. Imaging 2018;47:1199–1207.
Purpose
The long-term impact of low-grade germinal matrix-intraventricular hemorrhage (GMH-IVH) on brain perfusion has not been fully investigated. We aimed to compare cortical and deep gray matter ...(GM) cerebral blood flow (CBF) obtained with pseudo-continuous arterial spin labeling (pCASL), among preterm neonates with and without low-grade GMH-IVH and full-term controls.
Methods
3T-pCASL examinations of 9 healthy full-term neonates (mean gestational age 38.5 weeks, range 38–39) and 28 preterm neonates studied at term-equivalent age were analyzed. Eighteen preterm neonates presented normal brain MRI (mean gestational age 30.50 weeks, range 29–31) and 10 low-grade GMH-IVH according to Volpe’s grading system (mean gestational age 32 weeks, range 28–34). A ROI-based mean CBF quantification was performed in 5 cortical (frontal, parietal, temporal, insula, occipital), and 4 subcortical GM regions (caudate, putamen, pallidum, thalamus) for each cerebral hemisphere. CBF differences were explored using a nonparametric analysis of covariance.
Results
Low-grade GMH-IVH hemispheres showed consistently lower CBF in all GM regions when compared with healthy preterm neonates, after controlling the confounding effect of gestational age, postmenstrual age, and birth weight
P
< .001, η
2
= .394. No significant differences were observed between neonates with low-grade GMH and full-term controls. Healthy preterm neonates showed significantly higher CBF than full-term controls in parietal (
P
= .032), temporal (
P
= .016), and occipital cortex (
P
= .024), and at level of thalamus (
P
= .023) and caudate nucleus (
P
= .014).
Conclusion
Low-grade GMH-IVH is associated with lower CBF in posterior cortical and subcortical gray matter regions in preterm neonates, suggesting regional vulnerability of these developing brain structures.
Morning glory disc anomaly is a congenital abnormality of the optic disc and peripapillary retina reported as an isolated condition or associated with various anomalies, including basal ...encephaloceles and moyamoya vasculopathy. However, the co-occurrence of these three entities is extremely rare and the pathogenesis is still poorly understood. Moreover, data on the surgical management and long-term follow-up of the intracranial anomalies are scarce. Here, we describe the case of a 11-year-old boy with morning glory disc anomaly, transsphenoidal cephalocele, and moyamoya vasculopathy, who underwent bilateral indirect revascularization with encephalo-duro-myo-arterio-pericranio-synangiosis at the age of 2 years, and endoscopic repair of the transsphenoidal cephalocele at the age of 6 years. A rare missense variant (c.1081T>C,p.Tyr361His) was found in
OFD1
, a gene responsible for a X-linked ciliopathy, the oral-facial-digital syndrome type 1 (OFD1; OMIM 311200). This case expands the complex phenotype of OFD1 syndrome and suggests a possible involvement of
OFD1
gene and
Shh
pathway in the pathogenesis of these anomalies.
In animal and human neonates, expansion of the extracellular fluid volume is associated with “wet” lung and poor respiratory outcomes. To define fluid status changes during the transition from fetal ...to neonatal life in infants of diabetic mothers (IDM), we conducted a single-centre (Policlinico Abano Terme, Abano Terme, Italy) study of 66 IDM and a 1:2 matched control group from January 1 to September 30, 2020. Fluid status changes were assessed by computing Δ Hct from umbilical cord blood at birth and capillary heel Hct at 48 h, accounting for body weight decrease. IDM presented with significantly lower cord blood Hct levels in comparison to controls (47.33 ± 4.52 vs 50.03 ± 3.51%,
p
< 0.001), mainly if delivered by elective cesarean Sect. (45.01 ± 3.77 vs 48.43 ± 3.50%,
p
= 0.001). Hct levels at 48 h were comparable (55.18 ± 5.42 vs 54.62 ± 7.41%,
p
= 0.703), concurrently with similar body weight decrease (− 217.21 ± 113.34 vs − 217.51 ± 67.28 g,
p
= 0.614). This supports significantly higher ∆ Hct in IDM (5.13 ± 5.24 vs 7.29 ± 6.48,
p
< 0.01) and extra circulating fluid loss of 2–3%.
Conclusion:
Gestational diabetes is associated with an excess of circulating fluids during the transition from fetal to neonatal life, challenging the current assumption that is per se at risk of
wet
lung.
What is Known:
• In neonates, evidence suggests that expansion of the extracellular fluid volume prior to the postnatal diuresis is associated with poor respiratory outcomes.
What is New:
• Gestational diabetes is associated with an excess of circulating fluids during the transition from fetal to neonatal life, challenging the current assumption that is per se at risk of wet lung.
To grade brain gliomas by using a data-driven analysis of multiparametric magnetic resonance (MR) imaging, taking into account the heterogeneity of the lesions at MR imaging, and to compare these ...results with the most widespread current radiologic reporting methods.
One hundred eighteen patients with histologically confirmed brain gliomas were evaluated retrospectively. Conventional and advanced MR sequences (perfusion-weighted imaging, MR spectroscopy, and diffusion-tensor imaging) were performed. Three evaluations were conducted: semiquantitative (based on conventional and advanced sequences with reported cutoffs), qualitative (exclusively based on conventional MR imaging), and quantitative. For quantitative analysis, four volumes of interest were placed: regions with contrast material enhancement, regions with highest and lowest signal intensity on T2-weighted images, and regions of most restricted diffusivity. Statistical analysis included t test, receiver operating characteristic (ROC) analysis, discriminant function analysis (DFA), leave-one-out cross-validation, and Kendall coefficient of concordance.
Significant differences were noted in age, relative cerebral blood volume (rCBV) in contrast-enhanced regions (cutoff > 2.59; sensitivity, 80%; specificity, 91%; area under the ROC curve AUC = 0.937; P = .0001), areas of lowest signal intensity on T2-weighted images (>2.45, 57%, 97%, 0.852, and P = .0001, respectively), restricted diffusivity regions (>2.61, 54%, 97%, 0.808, and P = .0001, respectively), and choline/creatine ratio in regions with the lowest signal intensity on T2-weighted images (>2.07, 49%, 88%, 0.685, and P = .0007, respectively). DFA that included age; rCBV in contrast-enhanced regions, areas of lowest signal intensity on T2-weighted images, and areas of restricted diffusivity; and choline/creatine ratio in areas with lowest signal intensity on T2-weighted images was used to classify 95% of patients correctly. Quantitative analysis showed a higher concordance with histologic findings than qualitative and semiquantitative methods (P < .0001).
A quantitative multiparametric MR imaging evaluation that incorporated heterogeneity at MR imaging significantly improved discrimination between low- and high-grade brain gliomas with a very high AUC (ie, 0.95), thus reducing the risk of inappropriate or delayed surgery, respectively.
This article was published online with incorrect alignment in Table 4. Column and rows are out of order. The correct Table 4 is presented here. The original article has been corrected.
The mammillary bodies (MBs) have repeatedly been shown to be critical for memory, yet little is known about their involvement in numerous neurological conditions linked to memory impairments, ...including neonatal encephalopathy.
We implemented a multicentre retrospective study, assessing magnetic resonance scans of 219 infants with neonatal encephalopathy who had undergone hypothermia treatment in neonatal intensive care units located in the Netherlands and Italy.
Abnormal MB signal was observed in ~40% of infants scanned; in half of these cases, the brain appeared otherwise normal. MB involvement was not related to the severity of encephalopathy or the pattern/severity of hypoxic-ischaemic brain injury. Follow-up scans were available for 18 cases with abnormal MB signal; in eight of these cases, the MBs appeared severely atrophic.
This study highlights the importance of assessing the status of the MBs in neonatal encephalopathy; this may require changes to scanning protocols to ensure that the slices are sufficiently thin to capture the MBs. Furthermore, long-term follow-up of infants with abnormal MB signal is needed to determine the effects on cognition, which may enable the use of early intervention strategies. Further research is needed to assess the role of therapeutic hypothermia in MB involvement in neonatal encephalopathy.
The MBs are particularly sensitive to hypoxia in neonates. Current hypothermia treatment provides incomplete protection against MB injury. MB involvement is likely overlooked as it can often occur when the rest of the brain appears normal. Given the importance of the MBs for memory, it is necessary that this region is properly assessed in neonatal encephalopathy. This may require improvements in scanning protocols.
Early exposure to nociceptive events may cause brain structural alterations in preterm neonates, with long-lasting consequences on neurodevelopmental outcome. Little is known on the extent to which ...early pain may affect brain connectivity. We aim to evaluate brain functional connectivity changes in preterm neonate that underwent multiple invasive procedures during the postnatal period, and to correlate them with the neurodevelopmental outcome at 24 months.
In this prospective case-control study, we collected information about exposure to painful events during the early postnatal period and resting-state BOLD-fMRI data at term equivalent age from two groups of preterm neonate: 33 subjected to painful procedures during the neonatal intensive care (mean gestational age 27.9 ± 1.8 weeks) and 13 who did not require invasive procedures (average gestational age 31.2 ± 2.1 weeks). A data-driven principal-component-based multivariate pattern analysis (MVPA) was used to investigate the effect of early pain exposure on brain functional connectivity, and the relationship between connectivity changes and neurodevelopmental outcome at 24 months, assessed with Griffiths, Developmental Scale-Revised: 0-2.
Early pain was associated with decreased functional connectivity between thalami and bilateral somatosensory cortex, and between the right insular cortex and ipsilateral amygdala and hippocampal regions, with a more evident effect in preterm neonate undergoing more invasive procedures. Functional connectivity of the right thalamocortical pathway was related to neuromotor outcome at 24 months (
= 0.003).
Early exposure to pain is associated with abnormal functional connectivity of developing networks involved in the modulation of noxious stimuli in preterm neonate, contributing to the neurodevelopmental consequence of preterm birth.
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