Normal Brain Magnetic Resonance Imaging Research Articles

SAT-LB19 Is There a Need to Use Gadolinium Contrast for Pituitary MRI in the Evaluation of Pediatric Short Stature and Growth Hormone Deficiency?

SAT-LB19 Is There a Need to Use Gadolinium Contrast for Pituitary MRI in the Evaluation of Pediatric Short Stature and Growth Hormone Deficiency?

Peak blood pressure and prediction of posterior reversible encephalopathy syndrome in children.

Hypertension is a risk factor for posterior reversible encephalopathy syndrome (PRES), but the timing and severity of hypertension relative to PRES are unknown. The objective was to identify a clinically meaningful blood pressure (BP) threshold that predicts PRES development in high-risk children. We recorded peak systolic BP, diastolic BP, BP z-scores, and mean arterial pressure over the 14days preceding clinical concern for PRES in 35 subjects who developed PRES, compared to 14 controls who had normal brain magnetic resonance imaging and similar underlying disease, renal function, and medications. We used multivariable logistic regression models adjusted for fluid overload and obesity to estimate the association of peak BP with PRES. We used receiver operating characteristic curves to determine which peak BP thresholds best predicted PRES and calculated the corresponding sensitivity, specificity, and positive and negative predictive values. Peak systolic BP z-score was most strongly associated with PRES (OR 3.97, 95% CI 1.62-9.74), and peak systolic BP z-score ≥ 3.0 predicted PRES (area under the curve 0.95, 95% CI 0.88-1.0) with 91% sensitivity and 85% specificity, indicating 94% positive predictive value and 79% negative predictive value. We demonstrated that peak systolic BP z-score ≥ 3.0 in the preceding 14days predicted PRES development in cases compared with controls in children at high risk. Our study suggests that stage 2 hypertension, corresponding to a z-score ≥ 3.0, could help define hypertensive emergency in high-risk children and indicate when more aggressive treatment is warranted to prevent neurologic injury.

New-onset refractory status epilepticus and febrile infection-related epilepsy syndrome.

New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) are relatively rare clinical presentations. They are characterized by de novo onset of refractory status epilepticus (RSE) without clearly identifiable acute or active cause (structural, toxic, or metabolic). We reviewed the literature using PubMed reports published between 2003 and 2019 and summarized the clinical, neurophysiological, imaging, and treatment findings. Focal motor seizures, which tend to evolve into status epilepticus, characterize the typical presentation. Disease course is biphasic: acute phase followed by chronic phase with refractory epilepsy and neurological impairment. Aetiology is unknown, but immune-inflammatory-mediated epileptic encephalopathy is suspected. Electroencephalograms show variety in discharges (sporadic or periodic, focal, generalized, or more frequently bilateral), sometimes with a multifocal pattern. About 70% of adult NORSE have abnormal magnetic resonance imaging (MRI); in paediatric series of FIRES, 61.2% of patients have a normal brain MRI at the beginning and only 18.5% during the chronic phase. No specific therapy for FIRES and NORSE currently exists; high doses of barbiturates and ketogenic diet can be used with some effectiveness. Recently, anakinra and tocilizumab, targeting interleukin pathways, have emerged as potential specific therapies. Mortality rate is around 12% in children and even higher in adults (16-27%).

Magnetic Resonance Imaging Findings and Their Association with Electroencephalogram Data in Children with Partial Epilepsy.

BackgroundIt is important to identify the neuroimaging features that are associated with partial epilepsy in children. Advances in technology have recently been made to localize focal epileptogenic lesions, especially high-resolution structural imaging with magnetic resonance imaging (MRI). The recommendation that electroencephalography (EEG) should be the gold standard and that MRI should be optional has been questioned. The present study aims to evaluate the efficacy of MRI in children with partial epilepsy and to compare the diagnostic yields of MRI and EEG data.MethodsThe present study was conducted among one hundred twelve 1- to 6-year-old children with partial epilepsy. All patients underwent EEG and brain MRI. The epileptogenic lesions were identified on the basis of the signal intensities and morphological abnormalities seen on MRI. The correlation between MRI and EEG abnormal findings was analyzed using a chi-square test.ResultsAbnormal MRI findings were present in 34.8% (n = 39) of the sample. The EEG and MRI data agreed with respect to classifications into abnormal or normal in 48.2% of the sample (n = 54). Of the 27 patients with normal EEG findings, six (22.2%) had abnormal MRI findings. Inter-rater agreement showed the compatibility between EEG and MRI not significant (weighted Kappa = 0.105).ConclusionA number of MRI abnormalities were found in our study of otherwise normal children, although the correlation between these results was not clear. The follow-up of these children will help us identify the important abnormalities. Despite the small sample size, our results showed that normal EEG findings do not predict normal brain MRI data in children with partial epilepsy.

An automated computer-aided diagnosis system for classification of MR images using texture features and gbest-guided gravitational search algorithm

The segmentation and classification of brain magnetic resonance (MR) images are the crucial and challenging task for radiologists. The conventional methods for analyzing brain images are time-consuming and ineffective in decision-making. Thus, to overcome these limitations, this work proposes an automated and robust computer-aided diagnosis (CAD) system for accurate classification of normal and abnormal brain MR images. The proposed CAD system has the ability to assist the radiologists for diagnosis of brain MR images at an early stage of abnormality. Here, to improve the quality of images before their segmentation, contrast limited adaptive histogram equalization (CLAHE) is employed. The segmentation of the region of interest is obtained using the multilevel Otsu's thresholding algorithm. In addition, the proposed system selects the most significant and relevant features from the texture and multiresolution features. The multiresolution features are extracted using discrete wavelet transform (DWT), stationary wavelet transform (SWT), and fast discrete curvelet transform (FDCT). Moreover, the Tamura and local binary pattern (LBP) are used to extract the texture features from the images. These features are used to classify the brain MR images using feedforward neural network (FNN) classifier, where different meta-heuristic optimization algorithms, e.g., genetic algorithm (GA), particle swarm optimization (PSO), gravitational search algorithm (GSA), and gbest-guided gravitational search algorithm (GG-GSA) are employed for optimizing the weights and biases of FNN. The extensive experimental results on DS-195, DS-180, and three standard datasets show that the classification accuracy of GG-GSA based FNN classifier outperforms all mentioned meta-heuristic-based classifiers and several state-of-the-art methods.

Early neurodevelopmental characterization in children with cobalamin C/defect.

Cobalamin C (cblC) defect is the most common inherited disorder of cobalamin metabolism. Developmental delay, behavioral problems, and maculopathy are common, but they have not been systematically investigated. The aim of this study was to define early neurodevelopment in cblC patients and the possible contribution of different factors, such as mode of diagnosis, age at diagnosis, presence of brain lesions and epilepsy. Children up to the age of 4 years with a visual acuity ≥1/10 were evaluated using the Griffiths' Mental Development Scales. Eighteen children were enrolled (age range 12-48 months). Four were diagnosed by newborn screening (NBS); in the others mean age at diagnosis was 3.5 months (range 0.3-18 months). Eight had seizures: three in the first year, and five after the second year of life. Fourteen had brain lesions on magnetic resonance imaging (MRI). Neurovisual assessment evidenced low visual acuity (<3/10) in 4/18. NBS diagnosed patients had higher general and subquotients neurodevelopmental scores, normal brain MRI, and no epilepsy. The others showed a progressive reduction of the developmental quotient with age and language impairment, which was evident after 24 months of age. Our findings showed a progressive neurodevelopmental deterioration and a specific fall in language development after 24 months in cblC defect. The presence of brain lesions and epilepsy was associated with a worst neurodevelopmental outcome. NBS, avoiding major disease-related events and allowing an earlier treatment initiation, appeared to have a protective effect on the development of brain lesions and to promote a more favorable neurodevelopment.

Characteristics and influence of Mycoplasma/Ureaplasma cultures in amniotic fluid on perinatal outcomes.

To investigate the effects of Mycoplasma/Ureaplasma cultured in amniotic fluid on perinatal characteristics in preterm delivery between 22 and 33 weeks of gestation. The study was conducted in a tertiary perinatal center and involved 38 pregnant women who had undergone amniocentesis to evaluate intrauterine infection due to preterm labor or premature rupture of membranes. The subjects were divided into three groups based on the culture results: negative (Negative Group, n = 24), positive for Mycoplasma/Ureaplasma (M/U Group, n = 6), and positive for other pathogens (Other Pathogens Group, n = 8). One-way analysis of variance was used to compare the three groups. The incidence of histological chorioamnionitis and neonatal sepsis was significantly different among the three groups (the Negative Group and the Other Pathogens Group, P < 0.01; the M/U Group and the Other Pathogens Group, P = 0.03). In the M/U Group, no infants had sepsis, severe intraventricular hemorrhage, cystic periventricular leukomalacia, or poor neurological outcomes, but one infant developed bronchopulmonary dysplasia and needed home oxygen treatment. Although one died of gastrorrhexis, the remaining five patients had normal brain magnetic resonance imaging findings and developed normally. The presence of Mycoplasma/Ureaplasma isolated from amniotic fluid did not cause neonatal sepsis or poor prognosis. In some infants, there was no histological chorioamnionitis in the placenta. These pathogens thus seem to be less invasive than any other microbes with respect to perinatal outcomes.

Evaluation of cortical thickness and brain volume on 3 Tesla magnetic resonance imaging in children with frontal lobe epilepsy.

Frontal lobe epilepsy (FLE) is the most common epilepsy syndrome in the pediatric population; however, brain magnetic resonance imaging (MRI) of the children with FLE is frequently normal. We use both cortical thickness and brain volume measurements to report on cortical changes in children with FLE. Our aim was to determine cortical thickness and brain volume changes on 3 Tesla MRI of children with FLE and normal brain magnetic resonance imaging. Twenty-seven children with FLE and 27 healthy controls received brain magnetic resonance imaging. Cortical thickness and regional brain volumes were assessed using three-dimensional volumetric T1-weighted imaging and patients were compared with controls. In children with FLE, statistically significant (p < 0.05) cortical thinning were found in the bilateral middle frontal gyrus, bilateral occipitotemporal and medial lingual gyrus, left subcallosal gyrus, left short insular gyrus, and right long insular gyrus. Statistically significant volume reductions in right and left hemisphere cortical white matter, total cortical white matter, bilateral thalamus, bilateral putamen, bilateral globus pallidus, right caudate nucleus, brain stem, and right cerebellar cortex were found. Cortical thinning in frontal and extra-frontal lobes and volume loss in a variety of brain regions were found in children with FLE.

Germline de novo variants in CSNK2B in Chinese patients with epilepsy

CSNK2B, which encodes the beta subunit of casein kinase II (CK2), plays an important role in neuron morphology and synaptic transmission. Variants in CSNK2B associated with epilepsy and/or intellectual disability (ID)/developmental delay (DD) have been reported in five cases only. Among the 816 probands suspected hereditary epilepsy whose initial report of trio-based whole exome sequencing (WES) were negative, 10 de novo pathogenic or likely pathogenic variants of CSNK2B in nine probands were identified after reanalysis of their raw Trio-WES data. Six of the nine epileptic patients had ID/DD. The age of seizure onset of these nine patients with CSNK2B variants ranged from 2–12 months. Eight patients had age of seizure onset of less than 6 months. The epilepsy of most probands (8/9) was generalized tonic-clonic seizure and clustered (6/9). Most patients had normal electroencephalogram (5/9) and brain magnetic resonance image (7/9) results. Most patients (7/9) had easy-to-control seizures. Levetiracetam was the most commonly used drug in seizure-free patients (5/7). The variants detected in five patients (5/9, 55.6%) were located in the zinc-binding domain. In summary, our research provided evidence that variants in CSNK2B are associated with epilepsy with or without ID/DD. CSNK2B-related epilepsy is relatively easy to be controlled. The zinc-binding domain appears to be the hotspot region for mutation.

Learning to see the invisible: A data-driven approach to finding the underlying patterns of abnormality in visually normal brain magnetic resonance images in patients with temporal lobe epilepsy.

ObjectiveTo find the covert patterns of abnormality in patients with unilateral temporal lobe epilepsy (TLE) and visually normal brain magnetic resonance images (MRI‐negative), comparing them to those with visible abnormalities (MRI‐positive).MethodsWe used multimodal brain MRI from patients with unilateral TLE and employed contemporary machine learning methods to predict the known laterality of seizure onset in 104 subjects (82 MRI‐positive, 22 MRI‐negative). A visualization approach entitled "Importance Maps" was developed to highlight image features predictive of seizure laterality in both the MRI‐positive and MRI‐negative cases.ResultsSeizure laterality could be predicted with an area under the receiver operating characteristic curve of 0.981 (95% confidence interval [CI] =0.974‐0.989) in MRI‐positive and 0.842 (95% CI = 0.736‐0.949) in MRI‐negative cases. The known image features arising from the hippocampus were the leading predictors of seizure laterality in the MRI‐positive cases, whereas widespread temporal lobe abnormalities were revealed in the MRI‐negative cases.SignificanceCovert abnormalities not discerned on visual reading were detected in MRI‐negative TLE, with a spatial pattern involving the whole temporal lobe, rather than just the hippocampus. This suggests that MRI‐negative TLE may be associated with subtle but widespread temporal lobe abnormalities. These abnormalities merit close inspection and postacquisition processing if there is no overt lesion.

An image processing algorithm to aid diagnosis of mesial temporal sclerosis in children: a case-control study.

Mesial temporal sclerosis (MTS) is an important cause of intractable epilepsy. Early and accurate diagnosis of MTS is essential to providing curative and life-changing therapy but can be challenging in children in whom the impact of diagnosis is particularly high. Magnetic resonance imaging (MRI) plays an important role in the diagnosis of MTS, and image processing of MRI is a recently studied strategy to improve its accuracy. In a retrospective case-control study, we assessed the performance of an image processing algorithm (Correlative Image Enhancement [CIE]) for detecting MTS-related hippocampal signal abnormality in children. Twenty-seven pediatric MTS cases (9 males, 18 females; mean age: 16±standard deviation [SD] 6.7years) were identified from a pathology database of amygdylo-hippocampectomies performed in children with epilepsy. Twenty-seven children with no seizure history (9 males, 18 females; mean age: 13.8±SD 2.8years), and with normal brain MRI, were identified for the control group. Blinded investigators processed the MRI coronal FLAIR (fluid-attenuated inversion recovery) images with CIE, saved the processed images as a separate series, and made equivalent region of interest measurements on the processed and unprocessed series to calculate contrast-to-noise ratio. Six blinded reviewers then rated the randomized series for hippocampal signal abnormality and MTS disease status. CIE increased signal intensity and contrast-to-noise ratio in 26/27 hippocampi with pathologically confirmed MTS (96.3%) with the mean (SD) contrast-to-noise ratio of cases increasing from 14.9 (11.1) to 77.7 (58.7) after processing (P<0.001). Contrast-to-noise ratio increased in 1/54 normal control hippocampi (1.9%), with no significant change in the mean contrast-to-noise ratio of the control group after processing (P=0.57). Mean (SD) reader sensitivity for detecting abnormal signal intensity increased from 83.3% (14.2) to 94.8% (3.3) after processing. Mean specificity for abnormal signal intensity increased from 94.4% (7.3) to 96.3% (0). While sensitivity improved after processing for detection of MTS disease status in 4/6 readers, the mean reader sensitivity and specificity for MTS detection increased only minimally after processing, from 79.6% to 80.7% and from 95.7% to 96.3%, respectively. The CIE image processing algorithm selectively increased the contrast-to-noise ratio of hippocampi affected by MTS, improved reader performance in detecting MTS-related hippocampal signal abnormality and could have high impact on pediatric patients undergoing work-up for seizures.

Primary stabbing headache in children and adolescents.

To examine the characteristics of primary stabbing headache (PSH) in typically developing children and adolescents. In this prospective non-interventional hospital-based study, we examined 42 eligible individuals (24 females, 18 males; 26 white; mean age 12y 1mo, range 6y-16y 1mo) with spontaneous transient stabs. A headache focused history and/or a headache diary was obtained from each patient. All patients had normal neurological examination. Diagnosis of primary headache was based on the International Classification of Headache Disorders, Third Edition (ICHD-3) beta version. Duration of stabs ranged from a few seconds to up to 10minutes. Stabs were located in a variety of sites on the cranium but mostly in the extra-trigeminal regions (n=28). Intensity of stabs varied from moderate (n=4) to severe (n=38). The frequency of the stabs ranged from daily to monthly. There were up to 50 stabs per headache attack. Stabs among our patients occurred independently from those caused by other primary headache types. Accompanying symptoms during stabs were reported by eight patients. Family history of primary headaches was identified (n=17). Familial transmission of PSH was recognized among two patients. All patients had normal brain magnetic resonance imaging. The presentation and nature of PSH in children and adolescents varies widely. PSH in children may be a different entity to that in adults, and there is a need for further research to support changes in the ICHD-3 criteria for PSH in children and adolescents. Presentation of childhood primary stabbing headache (PSH) varies widely. Duration of PSH could last from a few seconds up to 10minutes.

FOXG1-Related Syndrome: From Clinical to Molecular Genetics and Pathogenic Mechanisms.

Individuals with mutations in forkhead box G1 (FOXG1) belong to a distinct clinical entity, termed “FOXG1-related encephalopathy”. There are two clinical phenotypes/syndromes identified in FOXG1-related encephalopathy, duplications and deletions/intragenic mutations. In children with deletions or intragenic mutations of FOXG1, the recognized clinical features include microcephaly, developmental delay, severe cognitive disabilities, early-onset dyskinesia and hyperkinetic movements, stereotypies, epilepsy, and cerebral malformation. In contrast, children with duplications of FOXG1 are typically normocephalic and have normal brain magnetic resonance imaging. They also have different clinical characteristics in terms of epilepsy, movement disorders, and neurodevelopment compared with children with deletions or intragenic mutations. FOXG1 is a transcriptional factor. It is expressed mainly in the telencephalon and plays a pleiotropic role in the development of the brain. It is a key player in development and territorial specification of the anterior brain. In addition, it maintains the expansion of the neural proliferating pool, and also regulates the pace of neocortical neuronogenic progression. It also facilitates cortical layer and corpus callosum formation. Furthermore, it promotes dendrite elongation and maintains neural plasticity, including dendritic arborization and spine densities in mature neurons. In this review, we summarize the clinical features, molecular genetics, and possible pathogenesis of FOXG1-related syndrome.

Intravenous Immunoglobulin as a Therapeutic Option for Mycoplasma pneumoniae Encephalitis.

To analyze the outcomes of a cohort of children diagnosed with Mycoplasma pneumoniae encephalitis whose treatment regimens included intravenous immunoglobulin (IVIG). A retrospective study was performed at a single center between 2011 and 2016 of children diagnosed with Mycoplasma pneumoniae encephalitis whose acute treatment regimen included IVIG. Details of therapeutic interventions and the clinical course were retrieved from medical records via an institutionally approved protocol. The modified Rankin score was used to quantify outcomes. Four children met inclusion criteria, 3 of whom had prodromal symptoms of infection lasting 5 to 7 days before onset of their neurologic symptoms. One patient presented with neurologic symptoms with no clinical prodrome. The initial treatment regimen included systemic corticosteroids, antibiotics, or both. IVIG was administered for a total dose of 2 g/kg divided over 2 to 4 days to all 4 children. All children showed clinical improvement after IVIG. The 3 children with prodromal symptoms showed immediate and dramatic clinical improvement after IVIG therapy. The immediate response to immunomodulatory therapy in the patients with prodrome suggests that the neurologic syndrome may be caused at least in part by an autoimmune process. The child who did not respond to IVIG had no prodrome, and also had normal electroencephalographic (EEG) and brain magnetic resonance imaging (MRI) findings. These cases suggest that early administration of IVIG should be considered in patients suspected of having Mycoplasma encephalitis, particularly in those who have had prodromal symptoms.

The Value of 1H-MRS and MRI in Combined Methylmalonic Aciduria and Homocystinuria.

ObjectiveThe aims of this study were to describe the brain magnetic resonance imaging (MRI) features of methylmalonic aciduria and homocystinuria and to evaluate the additional value of 1H-MRS.Patients and MethodsTwenty-eight children with methylmalonic aciduria and homocystinuria were included in this study. The control group included 21 healthy children. All the cases underwent MRI and 1H-MRS before treatment. We measured the N-acetylaspartate (NAA), choline (Cho), creatine (Cr), and myoinositol (mI) peaks in the basal ganglia regions. The NAA/Cr, Cho/Cr, mI/Cr, and NAA/Cho ratios were calculated. We also observed whether there were lactic acid peaks.ResultWe identified that NAA/Cr and NAA/Cho significantly decreased in the basal ganglia and that 3 patients showed lactate peaks, but other metabolites were not significantly altered. Hydrocephalus and diffuse supratentorial white matter edema were the primary MR findings; 7 patients had thinning of the corpus callosum, and 2 patients had subdural hematoma. Six patients showed normal brain MRI findings.ConclusionsMethylmalonic aciduria and homocystinuria patients with metabolite changes in the basal ganglia demonstrate compromised neuronal integrity, and anerobic metabolism occurs in acute encephalopathic episodes. 1H-MRS is a useful tool for evaluating brain damage. Hydrocephalus and diffuse supratentorial white matter edema are the main MRI features.

Automatic Classification of Brain Tumor and Alzheimer’s Disease in MRI

Computer vision (CV) and image processing techniques aim at the fast development of medical images diagnoses field. As the specialist takes a long time to diagnose one MRI images, CV techniques and machine learning algorithms make the process faster than the manual way, and these techniques save time and effort. In this paper, we developed an intelligent manner for the detection and classification of brain pathologies like tumors, Alzheimer’s disease (AD), or normal brain images. The proposed algorithm encompasses 4 stages: Magnetic Resonance Imaging (MRI) image acquisition, pre-processing, feature extraction, and classification. In this paper, the Bag of Features module has been used for the classification of the MRI of brain with tumor and MRI of brain of Alzheimer’s disease patients from normal brain MRI. Average classification accuracy of 97% is achieved for all three classes.

Comparison of brain magnetic resonance imaging findings before and after liver transplantation

Aim: The purpose was to compare the brain Magnetic Resonance Imaging (MRI) findings of the liver transplant recipients who had a neurological symptom, before and after liver transplantation. Material and Methods: Patients were evaluated in the terms of age, gender, etiology of chronic liver disease and the clinical symptoms. The brain MRI findings before and after liver transplantation were compared. Results: Thirty liver transplant patients with brain MRIs before and after liver transplantation were included. Hyper intensities on T1 weighted images which is compatible with manganese deposition in the basal ganglia was found from the first brain MRI of 18 patients. In thirteen of them the deposition was disappeared or decreased but in five of them the deposition persisted after transplantation. Hyper intensities on T2 weighted images which is compatible with copper deposition in the basal ganglia of four patients with Wilson’s disease persisted after transplantation. Five patients who had normal brain MRI findings before transplantation had posterior reversible encephalopathy syndrome (PRES) after the transplantation. One patient had focal cerebritis, three patients had acute infarct and one patient had intraparenchymal hemorrhage after transplantation. Conclusion: Most of the neurological symptoms during chronic liver disease are associated with the deposition of paramagnetic substances. After transplantation there could be regression in manganese deposition but the copper deposition stayed the same. Post-transplant PRES, central nervous system infections, infarct, and hemorrhage were not rare. Pre and post-transplant patients with neurological symptoms should be evaluated with brain MRI for rapid diagnosis and proper management.

Alterations of the brain network in idiopathic rapid eye movement sleep behavior disorder: structural connectivity analysis.

To evaluate and compare structural connectivity using graph theoretical analysis in patients with idiopathic rapid eye movement sleep behavior disorder (iRBD) and healthy subjects. Ten consecutive patients with iRBD were recruited from a single tertiary hospital. All patients had normal brain magnetic resonance imaging results on visual inspection. They did not have any other neurological disorder. Control subjects were also enrolled. All subjects underwent three-dimensional volumetric T1-weighted imaging. Absolute structural volumes were calculated using FreeSurfer image analysis software. Structural volume and connectivity analyses were performed with Brain Analysis using Graph Theory. Compared to healthy controls, patients with iRBD showed significant alterations in cortical and subcortical volumes, showing increased volumes of frontal cortex, thalamus, and caudate nucleus. In addition, patients with iRBD exhibited significantly different structural connectivity compared to healthy controls. In measures of global network, average degree, global efficiency, and local efficiency were decreased whereas characteristic path length was increased in iRBD patients. In measures of local network, there was significant hub reorganization in patients with iRBD. Betweenness centrality of caudate nucleus and frontal cortex was increased in patients with iRBD. This is the first study to report that structural volume and connectivity in patients with iRBD are significantly different from those in healthy controls. iRBD patients exhibited disrupted topological disorganization of the global brain network and hub reorganization. These alterations are implicated in the pathogenesis of iRBD. They might be potential biomarkers of iRBD.

Early Glycemic Profile Is Associated with Brain Injury Patterns on Magnetic Resonance Imaging in Hypoxic Ischemic Encephalopathy

To investigate whether the early glycemic profile in infants with hypoxic ischemic encephalopathy is associated with distinct patterns of brain injury on magnetic resonance imaging (MRI). We performed a secondary analysis of 178 prospectively enrolled infants who received therapeutic hypothermia for hypoxic ischemic encephalopathy. Glycemic profiles were identified by glucose concentrations within 24 hours after birth: normoglycemia (all glucose concentrations of >47 to ≤150 mg/dL; n = 62); hypoglycemia (≥1 concentration ≤47 mg/dL; n = 17); hyperglycemia (≥1 concentration >150 mg/dL; n = 76); and labile glucose (both hypoglycemia and hyperglycemia; n = 23). Patterns of brain injury were identified for 151 infants based on Barkovich scores from the postrewarming brain MRIs at a median age of 9 days. A normal brain MRI was reported in 37 of 62 infants (60%) with normal blood glucose values compared with 37 of 116 infants (32%) with an abnormal glucose profile (adjusted for Sarnat stage of encephalopathy and Apgar score at 5 minutes; P = .02). The distribution of MRI patterns of brain injury differed among the glycemic groups (P = .03). The odds of predominant watershed or focal-multifocal injury was higher in infants with hypoglycemia (aOR, 6; 95% CI, 1.5-24.2) and labile glucose (6.6; 95% CI, 1.6-27) compared with infants with normoglycemia. Infants with labile glucose had higher odds (5.6; 95% CI, 1.1-29.3) of predominant basal ganglia or global injury compared with infants with normal blood glucose values. The early glycemic profile in infants with hypoxic ischemic encephalopathy is associated with specific patterns of brain injury on MRI. Further investigation is needed to explore its prognostic significance and role as a phenotype biomarker.

(37) Pyruvate Dehydrogenase Complex Deficiency with normal Brain MRI: A Case report

Introduction: Pyruvate dehydrogenase complex deficiency (PDCD) is a rare genetic, life-threatening mitochondrial disorder causing lactic acidosis, and progressive neurological and neuromuscular degeneration. Structural abnormalities of the brain are observed in the majority of patients. We describe a patient with PDCD with a normal magnetic resonance Imaging (MRI). Case: A 20-month-old boy with history of hypotonia and developmental delay, presented with respiratory distress and hypoxia. He was diagnosed with bronchiolitis from Metapneumovirus. Two days after being started on PediaSure feeds, he developed respiratory arrest requiring intubation. Despite normal …