Magnetic Resonance Imaging Abnormalities Research Articles

Clinical Relevance of Painful Congenital Early-onset Scoliosis: A Magnetic Resonance Image-based Study.

Back pain, as a clinical marker in scoliosis, has been associated with underlying pathology for many years, warranting further magnetic resonance imaging (MRI). Failures of segmentation, mixed defects, female gender, rib anomalies, congenital thoracic anomalies, and neurocutaneous markers are known risk factors for abnormal MRI pathology findings in patients with congenital early-onset scoliosis (Congenital-EOS). Yet, back pain has not been evaluated as a risk factor for underlying MRI pathology in patients with Congenital-EOS. This study aimed to assess back pain as a risk factor for underlying pathology in Congenital-EOS using MRI as a diagnostic tool. A retrospective database review from the Pediatric Spine Study Group (PSSG) of all patients with Congenital-EOS who reported a back pain complaint, and underwent a spinal MRI study before surgical intervention was performed. Patients were divided into those with an underlying MRI pathology and those without. Demographics were compared between groups. From a total of 2355 patients with Congenital-EOS registered in PSSG, 107 patients reported a back pain complaint, with only 42 patients fulfilling the inclusion criteria (being evaluated with an MRI study). Overall group mean age was 8.1±4.5 years, with 25 of the 42 patients (60%) being females. Twenty-four of 42 patients (57%) had a comorbidity reported such as cardiac problems, musculoskeletal complaints, neurological deficits/myelopathy, gastrointestinal symptoms, developmental delay, respiratory problems, craniofacial abnormalities, and chromosomal conditions. An underlying MRI pathology was found in 21 of 42 patients with Congenital-EOS (50%) with back pain. The underlying MRI pathologies found were tethered spinal cord, spinal canal stenosis, syringomyelia, Arnold-Chiari malformation, and arachnoid cyst. Abnormal MRI findings are common in patients with Congenital-EOS who report back pain. Gender, age, major coronal curve angle, thoracic or lumbar predominance deformity, and comorbidities type or amount were not associated with abnormal MRI findings. Level II-Prognostic study.

Comparing clinical features, severity and prognosis of autoimmune encephalitis and with and without oligoclonal bands.

This study aimed to examine the clinical distinctions among patients diagnosed with autoimmune encephalitis (AE) based on the presence or absence of cerebrospinal fluid (CSF) oligoclonal bands (OCBs). Additionally, it sought to explore the relationship between OCBs and the severity and prognosis of autoimmune encephalitis. A retrospective analysis was conducted on 94 patients diagnosed with AE at the People's Hospital of Zhengzhou University between October 2016 and June 2022. The patients were divided into OCB-positive and OCB-negative groups based on CSF-OCBs. Patient severity at admission was assessed utilizing the Clinical Assessment Scale for Autoimmune Encephalitis (CASE) and the modified Rankin scale (mRS). Additionally, some oligoclonal-positive patients underwent dynamic longitudinal analysis of cerebrospinal fluid test indices. The mRS score was ultimately employed to evaluate patients' short-term prognosis (6 months) and long-term prognosis (at least 12 months) following immunotherapy. Of the 94 patients, 34 (36.2%) belonged to the OCB-positive group, while 60 (63.8%) belonged to the OCB-negative group. The group with anti-n-methyl-d-aspartate receptor (anti-NMDAR) encephalitis exhibited the highest rate of oligoclonal positivity at 27 (49.1%), followed by anti-aminobutyric acid B receptor (GABABR) encephalitis with 4 cases (30.8%), anti-contactin-associated protein-like 2 (CASPR2) encephalitis with 2 cases (20%), and anti-leucine-rich glioma inactivating protein 1 (LGI1) encephalitis with 1 case (6.25%). No statistically significant differences were found between the two groups regarding gender, age, prodromal symptoms, psychiatric disorders, seizures, language disorders, motor dysfunction, cognitive dysfunction, tumor incidence, and magnetic resonance imaging (MRI) abnormalities (p > 0.05). The OCB-positive group exhibited higher rates of autonomic dysfunction, intensive care unit (ICU) admission, CSF leukocytes, and IgG index compared to the OCB-negative group (p < 0.05). Additionally, the OCB-positive group had significantly higher median CASE and mRS scores prior to immunotherapy than the OCB-negative group (p < 0.001 and p < 0.001). Furthermore, in both short-term follow-up and long-term follow-up, the OCB-positive group had a significantly lower proportion of patients with a favorable prognosis compared to the OCB-negative group (50% vs. 71.7, 61.8% vs. 83.3%; p = 0.036, p = 0.002). Autonomic dysfunction, ICU admission, leukocytes in the cerebrospinal fluid, and elevated IgG index are more commonly observed in OCB-positive patients. OCB-positivity has also been linked to the severity and prognosis of AE, making it a potential biomarker. Initial OCB testing aids clinicians in identifying potentially critically ill patients early and monitoring disease progression, thereby optimizing clinical treatment decisions.

Accumulation Area of a Japanese PRNP P102L Variant Associated With Gerstmann-Sträussler-Scheinker Disease: The Ariake PRNP P102L Variant.

The coast of Kyushu Island on Ariake Sea in Japan is known to be an accumulation area for patients with a proline-to-leucine substitution mutation at residue 102 (P102L) of the human prion protein gene (PRNP), which is associated with Gerstmann-Sträussler-Scheinker disease. We designated this geographical distribution as the "Ariake PRNP P102L variant." The purpose of this study was to characterize the clinical features of this variant. We enrolled patients with the PRNP P102L variant who were followed up at the Saga University Hospital from April 2002 to November 2019. The clinical information of patients were obtained from medical records, including clinical histories, brain magnetic resonance imaging (MRI), and electroencephalography (EEG). A brain autopsy was performed on one of the participants. We enrolled 24 patients from 19 family lines, including 12 males. The mean age at symptom onset was 60.6 years (range, 41-77 years). The incidence rate of the Ariake PRNP P102L variant was 3.32/1,000,000 people per year in Saga city. The initial symptoms were ataxia (ataxic gait or dysarthria) in 19 patients (79.2%), cognitive impairment in 3 (12.5%), and leg paresthesia in 2 (8.3%). The median survival time from symptom onset among the 18 fatal cases was 63 months (range, 23-105 months). Brain MRI revealed no localized cerebellar atrophy, but sparse diffusion-weighted imaging abnormalities were detected in 16.7% of the patients. No periodic sharp-wave complexes were identified in EEG. Neuropathological investigations revealed uni- and multicentric prion protein (PrP) plaques in the cerebral cortex, putamen, thalamus, and cerebellum of one patient. Western blot analysis revealed 8-kDa proteinase-K-resistant PrP. This is the first report of the accumulation area of a PRNP P102L variant on the coast of Ariake Sea. The Ariake PRNP P102L variant can be characterized by a relatively long disease duration with sparse abnormalities in brain MRI and EEG relative to previous reports. Detailed interviews to obtain information on the birthplace and the family history of related symptoms are important to diagnosing a PRNP P102L variant.

Relationship of Clinical Profile with Neuroimaging and Electroencephalogram Changes in First Unprovoked Seizure in Children: A Cross-sectional Study

Introduction: Unprovoked seizures are common events in children, occurring as single episodes or recurring, as seen in epilepsy. The First Unprovoked Seizure (FUS) poses diagnostic challenges and raises concerns about aetiology, the initiation of anti-seizure medication and prognosis. The roles of Electroencephalogram (EEG) and neuroimaging in the diagnosis and management of FUS are debated. While EEGs play a crucial role in identifying seizure onset and epilepsy syndromes, neuroimaging aids in diagnosing underlying pathologies. Despite recommendations for routine EEGs in evaluating FUS, the role of neuroimaging remains less defined. Aim: To characterise the clinical profile of children presenting with FUS and investigate the relationship between clinical features, EEG findings and neuroimaging findings. Additionally, it explores the aetiology of FUS based on patterns of neuroradiological abnormalities and EEG changes. Materials and Methods: A cross-sectional study was conducted from April 2021 to July 2022 in the Paediatric Intensive Care Unit (PICU) and Paediatric Ward at Government TD Medical College, Alappuzha, Kerala, India. A total of 72 children aged beyond the neonatal period up to 12 years who were admitted with FUS at a tertiary care centre over an 18-month period were included in the study. Clinical data were recorded and standard techniques were used to perform EEGs and Magnetic Resonance Imaging (MRI) on all subjects. Results: The minimum age of participants in the study was two months, with a median age of 3.9 years. Among the 72 participants, most were in the infantile age group, with 23 (31.94%) falling into this category. The most frequently observed seizure type was generalised onset, with 47 (65.28%) cases. EEG irregularities were noted in 25 (34.72%) subjects, while 21 (29.16%) showed MRI abnormalities. Most frequent EEG abnormalities included generalised spike-and-wave discharges in 7 (28%) cases and modified hypsarrhythmia in 6 (24%) cases. The MRI scans revealed ischaemic changes and cerebral dysgenesis, each occurring in 7 out of 21 cases. A considerable proportion of children with abnormal EEGs had abnormal neuroimaging findings. Conclusion: The study recommends that both EEG and MRI should be included as part of the routine evaluation for children presenting with FUS. In cases with abnormalities in EEG, the detection rate of abnormalities through MRI was statistically significant. Additionally, MRI should be performed even when the EEG results are normal.

Hybrid Feature Extraction Technique-based Alzheimer’s Disease Detection Model Using MRI Images

Detecting Alzheimer’s disease (AD) using magnetic resonance imaging (MRI) is essential for early diagnosis and management. This study introduces a new method for detecting AD by combining three robust models: DenseNet201, EfficientNet B7, and extremely randomized trees (ERT). We improve the ability to extract features in DenseNet201 by including a self-attention mechanism. Additionally, we use early stopping techniques on EfficientNet B7 to address the issue of overfitting. In addition, Bayesian Optimization and Hyperband optimization techniques are used to adjust the hyperparameters of extra-trees to differentiate normal and abnormal MRI images. In addition, the authors used SHapley Additive exPlanations to understand the model’s decision. With minimal computer resources, the proposed model achieved a remarkable accuracy of 98.9% in detecting AD. The findings highlight the effectiveness of recommended feature extraction and ERT models and optimization methods to accurately identify AD using MRI images.

Brain magnetic resonance imaging as predictors in pediatric anti-N-methyl-D-aspartate receptor encephalitis

ObjectiveTo investigate the associations between brain magnetic resonance imaging (MRI) changes and clinical profiles in children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. MethodsClinical data and brain MRI results of children diagnosed with anti-NMDAR encephalitis in Guangzhou Women and Children's Medical Center from October 2014 to June 2022 were retrospectively studied. ResultsA total of 143 children (Male: female 54:89) were enrolled, with a mean onset age of 6.8 years (6.8 ± 3.1). 40.6 % (58/143) of patients had abnormal initial brain MRI. Lesions in temporal lobe (34.5 %, 20/58) and frontal lobe (25.9 %, 15/58) were relatively common. Children with abnormal initial brain MRI were prone to have fever (P = 0.023), dystonia (P = 0.037), positive MOG antibodies (P = 0.015), higher cerebrospinal fluid (CSF) white blood cell count (WBC) (P = 0.019) and to receive rituximab treatment (P = 0.037). There were no significant differences in modified Rankin Scale (mRS) scores before immunotherapy, after immunotherapy and at last follow-up between the normal initial brain MRI group and abnormal group. No initial brain MRI changes were found to be associated with relapses. Brain MRI was reviewed in 72 patients at last follow-up with a median follow-up time of 25.5 months and 48.6 % (35/72) of patients had abnormal brain MRI. The mRS score of the group with normal brain MRI at last follow-up was significantly lower than that of the abnormal group. ConclusionsAbout 40.0 % of children with anti-NMDAR encephalitis had abnormal initial brain MRI. Initial brain MRI was associated with certain clinical profiles, but not with relapse and prognosis. Around half of patients had abnormal brain MRI at last follow-up and were prone to have higher mRS score.

The congenital muscular dystrophies

AbstractBackgroundCongenital muscular dystrophies (CMDs) are genetically and clinically heterogeneous inherited conditions. Onset is typically within the first year of life. Most CMDs are autosomal recessive, except for de novo dominant mutations in LMNA‐related muscular dystrophy and some collagen‐6‐associated disorders.ResultsCMD is characterized by progressive muscular weakness, hypotonia, multiple contractures with a variable degree, spinal stiffness, delay in motor milestones acquisition, and histologically dystrophic lesions. Also, some forms of CMD may feature structural and myelination abnormalities on brain magnetic resonance imaging, intellectual impairment, and structural abnormalities of the eye. Muscle biopsy specimens exhibit a dystrophic pattern, but the appearance is quite variable depending on the different stages and severity of the disorder. The prevalence of CMD is estimated to be one in 100 000 people. Over the last few years, with advances in molecular genetic diagnostics, knowledge about neuromuscular disorders, particularly CMDs, has increased dramatically. Thus, the incidence may be higher than originally thought.ConclusionThis article reviews the recent achievements related to the clinical, diagnostic, pathogenic, and therapeutic aspects of CMDs.

An observational, multicenter, registry-based cohort study of Turkish Neonatal Society in neonates with Hypoxic ischemic encephalopathy

Background Hypoxic ischemic encephalopathy (HIE) is a significant cause of mortality and short- and long-term morbidities. Therapeutic hypothermia (TH) has been shown to be the standard care for HIE of infants ≥36 weeks gestational age (GA), as it has been demonstrated to reduce the rates of mortality, and adverse neurodevelopmental outcomes. This study aims to determine the incidence of HIE in our country, to assess the TH management in infants with HIE, and present short-term outcomes of these infants. Methods The Turkish Hypoxic Ischemic Encephalopathy Online Registry database was established for this multicenter, prospective, observational, nationally-based cohort study to evaluate the data of infants born at ≥34 weeks GA who displayed evidence of neonatal encephalopathy (NE) between March, 2020 and April 2022. Results The incidence of HIE among infants born at ≥36 weeks GA (n = 965) was 2.13 per 1000 live births (517:242440), and accounting for 1.55% (965:62062) of all neonatal intensive care unit admissions. The rates of mild, moderate and severe HİE were 25.5% (n = 246), 58.9% (n = 568), and 15.6% (n = 151), respectively. Infants with severe HIE had higher rates of abnormal magnetic resonance imaging (MRI) findings, and mortality (p&lt;0.001). No significant difference in mortality and abnormal MRI results was found according to the time of TH initiation (&lt;3 h, 3–6 h and &gt;6 h) (p&gt;0.05). TH was administered to 85 (34.5%) infants with mild HIE, and of those born of 34–35 weeks of GA, 67.4% (n = 31) received TH. A total of 58 (6%) deaths were reported with a higher mortality rate in infants born at 34–35 weeks of GA (OR 3.941, 95% Cl 1.446–10.7422, p = 0.007). Conclusion The incidence of HIE remained similar over time with a reduction in mortality rate. The timing of TH initiation, whether &lt;3 or 3–6 h, did not result in lower occurrences of brain lesions on MRI or mortality. An increasing number of infants with mild HIE and late preterm infants with HIE are receiving TH; however, the indications for TH require further clarification. Longer follow-up studies are necessary for this vulnerable population.

Brain imaging abnormalities in children with dysphagia with aspiration: A retrospective study.

Head imaging is often performed in children with persistent dysphagia with aspiration to evaluate for Chiari malformations that may be associated with dysphagia. Unfortunately, the frequency of Chiari malformations or other head imaging abnormalities in children who aspirate is unknown. The goal of this study is to determine the frequency of head imaging abnormalities in children with evidence of aspiration or penetration on video fluoroscopic swallow study (VFSS). Tertiary Children's Hospital. We performed retrospective analysis of children with a diagnosis of aspiration evaluated at our center from January 2010 through April 2021. In this study, we included children with VFSS confirmedaspiration or penetration, brain magnetic resonance imaging (MRI) performed at our center, and without known genetic, congenital craniofacial, or neurologic abnormalities. Of the 977 patients evaluated in our system during that time with a diagnosis of aspiration, 185 children met the inclusion criteria. Eight children were diagnosed with Chiari malformations (4.3%) and 94 head MRIs were abnormal (51.4%). There was no difference in VFSS findings (frequency of aspiration, penetration, penetration-aspiration score, or recommended thickness of liquid) in children with a Chiari malformation versus other abnormalities or normal brain imaging. The majority of other non-Chiari brain imaging abnormalities were nonspecific. There was no difference in VFSS findings in children with abnormal MRI findings versus normal MRI. Brain imaging abnormalities are common in children who aspirate. Intervenable lesions are rare. Further studies are required to determine patients that will most likely benefit from brain imaging.

Correlation between Persistent Serum Lactate Elevation and Brain Magnetic Resonance Imaging Abnormalities in Children with Status Epilepticus

Purpose: This study was conducted to evaluate the correlation between persistent serum lactate elevation and brain magnetic resonance imaging (MRI) in children with status epilepticus (SE). Methods: In this prospective analytical study, serum lactate levels were measured 24 hours after episodes of SE, and brain MRI was performed within 7 to 14 days after SE termination. MRI abnormalities were classified as acute encephalopathy (AE) grade I to III. The Kruskal-Wallis test was utilized for statistical analysis. Results: The study included 42 participants with SE, of whom 85.70% were boys, with a mean age of 4.94 years. Viral encephalitis was the most common diagnosis, accounting for 47.60% of cases. Elevated serum lactate levels were detected in 71.40% of patients, and approximately 47.60% exhibited abnormal MRI findings consistent with AE grade I. The median serum lactate levels for AE grades I, II, and III were 1.50, 3.10, and 0.78 mmol/L, respectively. Two patients died, and 66.70% experienced neurologic sequelae. A significant correlation (&lt;i&gt;P&lt;/i&gt;=0.021) was observed between persistent serum lactate elevation and abnormal brain MRI findings. Conclusion: In children with SE, AE grade I was the most common neuroimaging pattern observed, and persistently elevated serum lactate level was correlated with abnormal brain MRI findings.

Neonatal Hypoxic-Ischemic Encephalopathy Spectrum: Severity-Stratified Analysis of Neuroimaging Modalities and Association with Neurodevelopmental Outcomes

To compare hypoxic-ischemic injury on early cranial ultrasonography (cUS) and post-rewarming brain magnetic resonance imaging (MRI) in newborn infants with hypoxic-ischemic encephalopathy (HIE) and to correlate that neuroimaging with neurodevelopmental outcomes. This was a retrospective cohort study of infants with mild, moderate, and severe HIE treated with therapeutic hypothermia and evaluated with early cUS and postrewarming MRI. Validated scoring systems were used to compare the severity of brain injury on cUS and MRI. Neurodevelopmental outcomes were assessed at 18months of age. Among the 149 included infants, abnormal white matter (WM) and deep gray matter (DGM) hyperechogenicity on cUS in the first 48hours after birth were more common in the severe HIE group than the mild HIE group (81% vs 39% and 50% vs 0%, respectively; P<.001). In infants with a normal cUS, 95% had normal or mildly abnormal brain MRIs. In infants with severely abnormal cUS, none had normal and 83% had severely abnormal brain MRIs. Total abnormality scores on cUS were higher in neonates with near-total brain injury on MRI than in neonates with normal MRI or WM-predominant injury pattern (adjusted P<.001 for both). In the multivariable model, a severely abnormal MRI was the only independent risk factor for adverse outcomes (OR: 19.9, 95% CI: 4.0-98.1; P<.001). The present study shows the complementary utility of cUS in the first 48hours after birth as a predictive tool for the presence of hypoxic-ischemic injury on brain MRI.

Detection of Abnormality in Coronary Artery Magnetic Resonance Imaging using Bit Plane Slicing and Deep Learning.

This paper presents a novel approach for detecting abnormality in coronary arteries using MRI data in RGB images. The study evaluates the test accuracy of the weak classifiers and the test accuracy and F1 score of the strong classifier. The method involves separating the image into information planes, including R, G, and B color space, or bit-planes, and training a VGG-like convolutional neural network model on each plane separately, referred to as a "weak classifier." The classification results of these planes are aggregated using a proposed soft voting method, forming a "strong classifier," with the weights for the aggregation determined by the model's performance on the training set. The results indicate that the strong classifier achieves a test accuracy and F1 score of around 68% to 74% on our private coronary artery dataset. Moreover, by aggregating the top three highest bit-plane levels in a grayscale image, the accuracy is slightly lower than that of the three color spaces but requires a significantly smaller CNN model of nearly 4M parameters. The potential of bit-planes in reducing model storage costs is suggested. This approach holds promise for improving the detection of abnormalities in coronary arteries using MRI data.

Leukoencephalopathy with Brain stem and Spinal cord involvement and Lactate elevation (LBSL): Report of a new family and a novel DARS2 mutation

BackgroundLBSL is a mitochondrial disorder caused by mutations in the mitochondrial aspartyl-tRNA synthetase gene DARS2, resulting in a distinctive pattern on brain magnetic resonance imaging (MRI) and spectroscopy. Clinical presentation varies from severe infantile to chronic, slowly progressive neuronal deterioration in adolescents or adults. Most individuals with LBSL are compound heterozygous for one splicing defect in an intron 2 mutational hotspot and a second defect that could be a missense, non-sense, or splice site mutation or deletion resulting in decreased expression of the full-length protein. AimTo present a new family with two affected members with LBSL and report a novel DARS2 mutation. ResultsAn 8-year-old boy (Patient 1) was referred due to headaches and abnormal MRI, suggestive of LBSL. Genetic testing revealed a previously reported c.492 + 2 T > C mutation in the DARS2 gene. Sanger sequencing uncovered a novel variant c.228-17C > G in the intron 2 hotspot. Family studies found the same genetic changes in an asymptomatic 4-year-old younger brother (Patient 2), who was found on follow-up to have an abnormal MRI. mRNA extracted from patients' fibroblasts showed that the c.228-17C > G mutation caused skipping of exon 3 resulting in lower DARS2 mRNA level. Complete absence of DARS2 protein was also found in both patients. SummaryWe present a new family with two children affected with LBSL and describe a novel mutation in the DARS2 intron 2 hotspot. Despite findings of extensive white matter disease in the brain and spine, the proband in this family presented only with headaches, while the younger sibling, who also had extensive white matter changes, was asymptomatic. Our in-vitro results confirmed skipping of exon 3 in patients and family members carrying the intron 2 variant, which is consistent with previous reported mutations in intron 2 hotspots. DARS2 mRNA and protein levels were also reduced in both patients, further supporting the pathogenicity of the novel variant.

Magnetic resonance imaging findings in autoimmune hepatitis: how frequent and reproducible are they?

To determine the frequency and interobserver reproducibility of the magnetic resonance imaging (MRI) features considered diagnostic for autoimmune hepatitis. Two abdominal radiologists, blinded to pathology data, reviewed the MRI examinations of 20 patients with autoimmune hepatitis, looking for liver enhancement, lymphadenopathy, portal hypertension, and chronic liver disease. The pattern of liver fibrosis was categorized as reticular, confluent, or mixed. Interobserver agreement was assessed by calculating intraclass correlation coefficients and kappa statistics. The most common abnormal finding on MRI was surface nodularity (in 85%), followed by liver fibrosis with a reticular pattern (in 80%)-categorized as mild (in 25.0%), moderate (in 43.8%), or severe (in 31.2%)-; heterogeneous liver enhancement (in 65%); splenomegaly (in 60%); caudate lobe enlargement (in 50%); and lymphadenopathy (in 40%). The interobserver agreement was almost perfect for surface nodularity (0.83), ascites (0.89), and liver volume (0.95), whereas it was just slight and fair for the degree of fibrosis and for heterogeneous liver enhancement (0.12 and 0.25, respectively). It was also slight and fair for expanded gallbladder fossa and enlarged preportal space (0.14 and 0.36, respectively), both of which are indicative of chronic liver disease. The interobserver agreement was satisfactory for surface nodularity (the most prevalent abnormal MRI finding), ascites, liver volume, and splenomegaly. Conversely, it was only slight or fair for common but less objective criteria.

Multimodality imaging and immunophenotyping of COVID-19 related myocardial injury (the MIIC-MI Study)

Abstract Background Cardiac injury is a well-recognised complication of acute COVID-19 infection, with varied aetiologies ranging from myocarditis to endothelial dysfunction, micro-embolic phenomena, and acute coronary syndromes. While persistent cardiac symptoms in patients with post-acute COVID-19 syndrome are also common, the underlying mechanisms remain unclear. A better understanding of immune-mediated drivers of chronic cardiac injury after COVID-19 infection is urgently needed to inform the management of patients with, or at risk of, long-term sequelae. Purpose To examine immune mechanisms associated with clinically detectable imaging abnormalities across a spectrum of patients with unexplained cardiac injury and/or symptoms after COVID-19 infection. Methods Participants with a history of COVID-19 infection and suspected cardiac involvement based on: a) troponin I elevation (&amp;gt;99th percentile upper reference limit), b) new-onset heart failure not attributable to another cause, or c) persistence of unexplained cardiac symptoms, were enrolled at a single centre in the United Kingdom between October 2020 and February 2022. Individuals with prior myocardial infarction or heart failure and those treated with immunosuppression within the past four weeks, were excluded. Cardiac magnetic resonance imaging (MRI) was performed on a 1.5T scanner following a standard clinical protocol. In parallel to cardiac imaging, peripheral blood samples were analysed for immunophenotyping using cytometry by time of flight (Fluidigm, 32 marker panel) and proteomic analysis (Olink, Target 96 Inflammation panel). Results Twenty-one patients (mean age 47 (SD 13) years, 71% female) were enrolled who had unexplained cardiac symptoms (n=17), raised troponin I (n=2), or new-onset heart failure (n=2). In total, 9/21 (43%) patients had MRI abnormalities, including a non-ischaemic pattern of late gadolinium enhancement (Figure 1a; arrow) and/or visually overt myocardial oedema on T2-weighted imaging (Figure 1b; arrow) in 8 patients, and one with impaired ventricular function in the absence of fibrosis. Patients with MRI abnormalities had higher CCL3, CCL7 (MCP-3), FGF21, and CD4 Th2 cells, and lower CD8 T effector memory cells (Figure 2, volcano plot), compared to those without. CCL7 (MCP-3) levels were correlated with native T1 mapping (r=0.45, p&amp;lt;0.05) and these along with CD8 T effector memory cell counts were the strongest predictors of MRI abnormalities in a LASSO regression model incorporating all clinical, cell phenotyping and proteomic data (composite AUC 0.96, CI 0.88-1). Conclusion Here we show for the first time that COVID-19 related cardiac injury in patients with MRI abnormalities is associated with increased CCL7 (MCP-3), a chemokine known to be important in viral myocarditis1, as well as decreased CD8 T effector memory cells. These findings provide novel insights into the immune-mediated mechanisms underlying non-ischemic cardiac complications of COVID-19.Fig. 1.Post-COVID imaging abnormalitiesFig. 2.Immunophenotyping and proteomics

The Agreement Between Long-Term Monitoring by Electroencephalography and Magnetic Resonance Imaging in Paediatric Seizure

Background: Demonstration of high agreement between structural abnormalities identified on magnetic resonance imaging (MRI) and physiologic abnormalities identified on electroencephalography (EEG) could benefit the assessment of epileptic focus in childhood seizures. Objectives: The present study aimed to assess the agreement between abnormal findings on brain MRI and long-term monitoring (LTM) by EEG as the standard protocol in children with abnormal focal epileptic discharges in LTM. Methods: This cross-sectional study was performed on 95 children who suffered from seizures with evidence of focal epileptic discharges in LTM who were referred to the Children's Medical Center in 2017. All patients were also concurrently evaluated by MRI. All MRIs were evaluated twice, before and after receiving the EEG results. Results: In this study, 59 out of 95 patients with abnormal LTM had concurrently abnormal MRI findings. The diagnostic agreement between the MRI and LTM in discovering abnormal findings was found to be high (86.4%) with a kappa correlation coefficient equal to 0.79. Conclusions: About two-thirds of patients with abnormal LTM findings had concurrent abnormal MRI features with high agreement between the two. Thus, MRI and EEG can be valuable in predicting epileptic focus in drug-resistant patients who need surgery.

Efficient organisation of the contralateral hemisphere connectome is associated with improvement in intelligence quotient after paediatric epilepsy surgery

ObjectiveAims of epilepsy surgery in childhood include optimising seizure control and facilitating cognitive development. Predicting which children will improve cognitively is challenging. We investigated the association of the pre-operative structural connectome of the contralateral non-operated hemisphere with improvement in intelligence quotient (IQ) post-operatively. MethodsConsecutive children who had undergone unilateral resective procedures for epilepsy at a single centre were retrospectively identified. We included those with pre-operative volume T1-weighted non-contrast brain magnetic resonance imaging (MRI), no visible contralateral MRI abnormalities, and both pre-operative and two years post-operative IQ assessment. The MRI of the hemisphere contralateral to the side of resection was anatomically parcellated into 34 cortical regions and the covariance of cortical thickness between regions was used to create binary and weighted group connectomes. ResultsEleven patients with a post-operative IQ increase of at least 10 points at two years were compared with twenty-four patients with no change in IQ score. Children who gained at least 10 IQ points post-operatively had a more efficiently structured contralateral hemisphere connectome with higher global efficiency (0.74) compared to those whose IQ did not change at two years (0.58, p = 0.014). This was consistent across thresholds and both binary and weighted networks. There were no statistically significant group differences in age, sex, age at onset of epilepsy, pre-operative IQ, mean cortical thickness, side or site of procedure, two year post-operative Engel scores or use of anti-seizure medications between the two groups. ConclusionsSurgical procedures to reduce or stop seizures may allow children with an efficiently structured contralateral hemisphere to achieve their cognitive potential.

32 Prediction of Seizure Outcome with Presurgical IAT, MRI, and PET in Patients with Temporal Lobe Epilepsy Undergoing Surgery

Objective:Anterior temporal lobectomy is a common surgical approach for medication-resistant temporal lobe epilepsy (TLE). Prior studies have shown inconsistent findings regarding the utility of presurgical intracarotid sodium amobarbital testing (IAT; also known as Wada test) and neuroimaging in predicting postoperative seizure control. In the present study, we evaluated the predictive utility of IAT, as well as structural magnetic resonance imaging (MRI) and positron emission tomography (PET), on long-term (3-years) seizure outcome following surgery for TLE.Participants and Methods:Patients consisted of 107 adults (mean age=38.6, SD=12.2; mean education=13.3 years, SD=2.0; female=47.7%; White=100%) with TLE (mean epilepsy duration =23.0 years, SD=15.7; left TLE surgery=50.5%). We examined whether demographic, clinical (side of resection, resection type [selective vs. non-selective], hemisphere of language dominance, epilepsy duration), and presurgical studies (normal vs. abnormal MRI, normal vs. abnormal PET, correctly lateralizing vs. incorrectly lateralizing IAT) were associated with absolute (cross-sectional) seizure outcome (i.e., freedom vs. recurrence) with a series of chi-squared and t-tests. Additionally, we determined whether presurgical evaluations predicted time to seizure recurrence (longitudinal outcome) over a three-year period with univariate Cox regression models, and we compared survival curves with Mantel-Cox (log rank) tests.Results:Demographic and clinical variables (including type [selective vs. whole lobectomy] and side of resection) were not associated with seizure outcome. No associations were found among the presurgical variables. Presurgical MRI was not associated with cross-sectional (OR=1.5, p=.557, 95% CI=0.4-5.7) or longitudinal (HR=1.2, p=.641, 95% CI=0.4-3.9) seizure outcome. Normal PET scan (OR= 4.8, p=.045, 95% CI=1.0-24.3) and IAT incorrectly lateralizing to seizure focus (OR=3.9, p=.018, 95% CI=1.2-12.9) were associated with higher odds of seizure recurrence. Furthermore, normal PET scan (HR=3.6, p=.028, 95% CI =1.0-13.5) and incorrectly lateralized IAT (HR= 2.8, p=.012, 95% CI=1.2-7.0) were presurgical predictors of earlier seizure recurrence within three years of TLE surgery. Log rank tests indicated that survival functions were significantly different between patients with normal vs. abnormal PET and incorrectly vs. correctly lateralizing IAT such that these had seizure relapse five and seven months earlier on average (respectively).Conclusions:Presurgical normal PET scan and incorrectly lateralizing IAT were associated with increased risk of post-surgical seizure recurrence and shorter time-to-seizure relapse.

Magnetic Resonance Imaging Findings and Genetic Testing Results in Children With Congenital Corneal Opacities

This study investigates brain and globe abnormalities identified on magnetic resonance imaging (MRI) in children with congenital corneal opacities (CCO). Retrospective cohort study. Clinical notes, radiology records, and genetic testing results were reviewed for patients diagnosed with corneal opacification within the first 6 months of life at a tertiary referral academic center between August 2008 and January 2018. Ocular findings, systemic anomalies, neuroimaging, and genetic testing results were summarized. A total of 135 patients presenting at age 1 day to 12 years (mean age, 1 year) were identified. Children with bilateral CCO were more likely to have systemic disease (P = 0.018). Of the entire cohort, 43 (31.8%) patients received MRI, of whom 27 (62.8%) had abnormal brain findings and 30 (69.7%) had abnormal orbital findings. The most common abnormal brain findings were ventriculomegaly (n = 16, 59.2%) and corpus callosum abnormalities (n = 10, 37.0%) followed by brainstem/pons anomalies (n = 5, 18.5%), and cerebellar anomalies (n = 2, 7.4%). Abnormal brain MRI findings were associated with the presence of neurologic (P = .003) and craniofacial (P = .034) disease. A total of 44 (32.1%) patients underwent genetic testing, of whom 29 (65.9%) had pathogenic results. More than 60% of the children with CCO who underwent MRI had abnormal brain and orbit findings that were correlated with significant neurologic disease. Furthermore, almost two-thirds of patients with CCO who underwent genetic testing had pathogenic results. These data demonstrate the value of systemic workup in children with CCO, and highlight the role of ophthalmologists in facilitating the diagnosis of systemic comorbidities associated with CCO.

A comparison between neurological clinical signs, cerebrospinal fluid analysis, cross-sectional CNS imaging, and infectious disease testing in 168 dogs with infectious or immune-mediated meningoencephalomyelitis from Brazil.

This retrospective study evaluated canine patients with presumptively diagnosed meningoencephalomyelitis (ME) based on neurological clinical signs, cerebrospinal fluid (CSF) analysis, cross-sectional imaging, and infectious disease testing with a limited neurological-focused polymerase chain reaction (PCR) panel performed on blood and CSF. The first goal was to determine the proportion of dogs where the condition was caused by an infectious agent versus a probable immune-mediated etiology (i.e., meningoencephalomyelitis of unknown origin; MUO) in our geographic region. The secondary goals of this study were to examine and define associations between abnormal CSF test results and cross-sectional neuroimaging findings, in addition to defining the age and most common neurological clinical signs in each group of ME. A total of 168 dogs matched the inclusion criteria with magnetic resonance imaging (MRI) performed in 130 dogs and computed tomography (CT) performed in 38 dogs. Presumptive MUO was observed in 152/168 (90.5%) of dogs and infectious ME was identified in 16/168 (9.5%) of dogs (p < 0.0001). Canine distemper virus (CDV) was the most common cause of infectious ME in 10/16 dogs (62.5%). Of the total cases with a positive infectious disease result, 3/16 (18.7%) had normal CSF results and 13/16 (81.3%) had abnormal CSF results (p = 0.0078). MRI and CT abnormalities in the brain were detected in 74 and 39% of dogs with inflammatory CSF, respectively. MRI and CT abnormalities in the spinal cord were detected in 90 and 57% of dogs with inflammatory CSF results, respectively. Age was not significantly different between infectious ME and presumptive MUO groups (p = 0.15). Seizures were the most common clinical sign reported for both MUO (36.8% of cases) and infectious ME (31.2% of cases). In conclusion, presumptive MUO is significantly more common than infectious ME in this population of dogs. Furthermore, although normal CSF results were uncommon in dogs with infectious ME, this finding occurred in several patients (3/16), suggesting that infectious disease testing should be considered even in the face of normal CSF results. Finally, MRI was more sensitive than CT in the detection of abnormalities when dogs with ME had inflammatory CSF results but was not 100% sensitive, suggesting CSF analysis should be performed to rule out inflammation even when no abnormalities are detected on MRI or CT.