Brain Magnetic Resonance Imaging Research Articles

Worsening abdominal pain leading to false laparotomy: a case of acute intermittent porphyria

Acute intermittent porphyria (AIP), one of the most severe types of acute hepatic porphyria, is an autosomal dominant inherited disorder of heme biosynthesis. We present a case of a 16-year-old girl who presented with severe abdominal pain, subjected to a laparotomy and later developed seizures and other neurological manifestations. Initial investigations showed hyponatraemia. Magnetic Resonance Imaging of brain showed cerebritis. She underwent several investigations including an ultrasound of abdomen, a computed tomography scan of abdomen and pelvis, cerebrospinal fluid routine examination and culture, and autoimmune investigation which were found to be normal. HM urine porphobilinogen levels were found to be raised. The diagnosis was made based on investigation and clinical symptoms. AIP should be suspected when a patient presents with chronic abdominal pain and neurological symptoms. Keywords: Acute Intermittent Porphyria, false laparotomy, appendicitis, acute abdomen, hyponatremia, tonic-clonic seizures.

Sex Differences in Frequency, Severity, and Distribution of Cerebral Microbleeds

Cerebral small vessel disease (SVD) is associated with various cerebrovascular outcomes, but data on sex differences in SVD are scarce. To investigate whether the frequency, severity, and distribution of cerebral microbleeds (CMB), other SVD markers on magnetic resonance imaging (MRI), and outcomes differ by sex. This cohort study used pooled individual patient data from the Microbleeds International Collaborative Network, including patients from 38 prospective cohort studies in 18 countries between 2000 and 2018, with clinical follow-up of at least 3 months (up to 5 years). Participants included patients with acute ischemic stroke or transient ischemic attack with available brain MRI. Data were analyzed from April to December 2023. Outcomes of interest were presence of CMB, lacunes, and severe white matter hyperintensities determined on MRI. Additionally, mortality, recurrent ischemic stroke, and intracranial hemorrhage during follow-up were assessed. Multivariable random-effects logistic regression models, Cox regression, and competing risk regression models were used to investigate sex differences in individual SVD markers, risk of recurrent cerebrovascular events, and death. A total of 20 314 patients (mean [SD] age, 70.1 [12.7] years; 11 721 [57.7%] male) were included, of whom 5649 (27.8%) had CMB. CMB were more frequent in male patients, and this was consistent throughout different age groups, locations, and in multivariable models (female vs male adjusted odds ratio [aOR], 0.86; 95% CI, 0.80-0.92; P < .001). Female patients had fewer lacunes (aOR, 0.82; 95% CI, 0.74-0.90; P < .001) but a higher prevalence of severe white matter hyperintensities (aOR, 1.10; 95% CI, 1.01-1.20; P = .04) compared with male patients. A total of 2419 patients (11.9%) died during a median (IQR) follow-up of 1.4 (0.7-2.5) years. CMB presence was associated with a higher risk of mortality in female patients (hazard ratio, 1.15; 95% CI, 1.02-1.31), but not male patients (hazard ratio, 0.95; 95% CI, 0.84-1.07) (P for interaction = .01). A total of 1113 patients (5.5%) had recurrent ischemic stroke, and 189 patients (0.9%) had recurrent intracranial hemorrhage, with no sex differences. This cohort study using pooled individual patient data found varying frequencies of individual SVD markers between female and male patients, indicating potential pathophysiological differences in manifestation and severity of SVD. Further research addressing differences in pathomechanisms and outcomes of SVD between female and male patients is required.

Functional connectivity in resting-state networks relates to short-term global cognitive functioning in cardiac arrest survivors.

Long-term cognitive impairment is common in cardiac arrest survivors. Screening to identify patients at risk is recommended. Functional magnetic resonance brain imaging (fMRI) holds potential to contribute to prediction of cognitive outcomes. In this study, we investigated the possible value of early changes in resting-state networks for predicting short and long-term cognitive functioning of cardiac arrest survivors. We performed a prospective multicenter cohort study in cardiac arrest survivors in three Dutch hospitals. Resting-state fMRI scans were acquired within a month after cardiac arrest. We primarily focused on functional connectivity within the default-mode network (DMN) and salience network (SN), and additionally explored functional connectivity in seven other networks. Cognitive outcome was measured using the Montreal Cognitive Assessment (MoCA) during hospital admission and at 3 and 12 months, and by neuropsychological examination (NPE) at 12 months. We tested mixed effects models to evaluate the value of connectivity within the networks for predicting global cognitive outcomes at the three time points, and long-term cognitive outcomes in the memory, attention, and executive functioning domains. We included 80 patients (age 60 ± 11 years, 72 (90%) male). MoCA scores increased significantly between hospital admission and 3 months (ΔMoCAhospital-3M = 2.89, p < 0.01), but not between 3 and 12 months (ΔMoCA3M-12M = 0.38, p = 0.52). Connectivity within the DMN, SN, and dorsal attention network (DAN) was positively related to global cognitive functioning during hospital admission (βDMN = 0.85, p = 0.03; βSN = 1.48, p < 0.01; βDAN = 0.96, p = 0.01), but not at 3 and 12 months. Network connectivity was also unrelated to long-term memory, attention, or executive functioning. Resting-state functional connectivity in the DMN, SN, and DAN measured in the first month after cardiac arrest is related to short-term global, but not long-term global or domain-specific cognitive performance of survivors. These results do not support the value of functional connectivity within these RSNs for prediction of long-term cognitive performance after cardiac arrest.

Synthetic data in generalizable, learning-based neuroimaging

Abstract Synthetic data has emerged as an attractive option for developing machine learning methods in human neuroimaging, particularly in magnetic resonance imaging (MRI)— a modality where image contrast depends enormously on acquisition hardware and parameters. This retrospective paper reviews a family of recently proposed methods, based on synthetic data, for generalizable machine learning in brain MRI analysis. Central to this framework is the concept of domain randomization, which involves training neural networks on a vastly diverse array of synthetically generated images with random contrast properties. This technique has enabled robust, adaptable models that are capable of handling diverse MRI contrasts, resolutions, and pathologies, while working out-of-the-box, without retraining. We have successfully applied this method to tasks such as whole brain segmentation (SynthSeg), skull-stripping (SynthStrip), registration (SynthMorph, EasyReg), super-resolution and MR contrast transfer (SynthSR). Beyond these applications, the paper discusses other possible use cases and future work in our methodology. Neural networks trained with synthetic data enable the analysis of clinical MRI, including large retrospective datasets, while greatly alleviating (and sometimes eliminating) the need for substantial labeled datasets, and offer enormous potential as robust tools to address various research goals.

A novel classification framework for genome-wide association study of whole brain MRI images using deep learning.

Genome-wide association studies (GWASs) have been widely applied in the neuroimaging field to discover genetic variants associated with brain-related traits. So far, almost all GWASs conducted in neuroimaging genetics are performed on univariate quantitative features summarized from brain images. On the other hand, powerful deep learning technologies have dramatically improved our ability to classify images. In this study, we proposed and implemented a novel machine learning strategy for systematically identifying genetic variants that lead to detectable nuances on Magnetic Resonance Images (MRI). For a specific single nucleotide polymorphism (SNP), if MRI images labeled by genotypes of this SNP can be reliably distinguished using machine learning, we then hypothesized that this SNP is likely to be associated with brain anatomy or function which is manifested in MRI brain images. We applied this strategy to a catalog of MRI image and genotype data collected by the Alzheimer's Disease Neuroimaging Initiative (ADNI) consortium. From the results, we identified novel variants that show strong association to brain phenotypes.

Bihemispheric IDH-Wildtype Glioblastoma with Unilateral FGFR3-TACC3 Fusion in Patient with Breast Cancer History: A Case Report and Literature Review

Abstract Introduction/Objective Adult IDH-wild type glioblastomas comprise a heterogeneous spectrum of neoplasms, characterized by poor prognosis and resistance to the chemoradiotherapy. For WHO integrated diagnosis classification and to establish treatment options, molecular analysis is necessary. Herein, we present a case of a glioblastoma, IDH-wildtype with involvement of both cerebral hemispheres and an uncharacteristic in-frame fusion limited to one hemisphere. Methods/Case Report The patient is a 52-year-old woman with past medical history of metastatic breast cancer who presented with headache and worsening facial pain. Brain magnetic resonance imaging brain demonstrated a right frontal lobe lesion with vasogenic edema. The patient was treated with Gamma Knife radiosurgery. Scans revealed enlargement and a new left frontal lobe lesion. Bihemispheric tumor resection and GammaTile placement was undertaken. Results (if a Case Study enter NA) Histologically, the tumor was composed of hypercellular astrocytes, microvascular proliferation, and necrosis with no evidence of metastatic disease. Immunohistochemistically, neoplastic cells showed glial fibrillary acidic protein expression and elevated Ki-67 proliferation index. Molecular examination revealed in the right hemisphere lesion post-radiation (1) FGFR3::TACC3 (exon 17::exon 11) fusion and (2) TERT promoter, while the left hemisphere lesion which did not receive radiation showed clinically relevant variants in (1) TERT promoter, (2) TP53, (3) NF1, and (3) PIK3CA without identification of a fusion. An integrated diagnosis of glioblastoma, IDH- wildtype, CNS WHO grade 4 was established bilaterally. Conclusion The fibroblast growth factor receptor 3 (FGFR3)-transforming acidic coiled-coil 3 (TACC3) fusion is identified as a rare molecular feature in glioblastomas with an estimated prevalence of 4%. Clinical trials suggest that F3T3-positive tumors have a better prognosis than those without the translocation. The identification of the oncogenic FGFR3-TACC3 fusion highlights the possibility of identifying patients potentially responsive to targeted therapy with FGFR kinase inhibitors. The significance of the molecular differences in the right and left hemisphere lesions is uncertain; glioblastomas are heterogeneous tumors with intra-and intertumoral heterogeneity but it may suggest independent origins.

Mild Encephalopathy/Encephalitis with Reversible Splenial Lesions after Amphetamine and Opioid Use.

A 47-year-old woman with a history of substance abuse, depression, and insomnia experienced a collapse. Upon arrival at the hospital, the patient displayed low systolic blood pressure, confusion, dehydration, and renal failure. Urine tests confirmed an amphetamine and opioid overdose. Her condition fluctuated with reduced consciousness, myoclonic movements, fever, and suspected psychogenic seizures. Brain magnetic resonance imaging revealed abnormalities in the splenium of the corpus callosum, which later resolved. The patient's condition gradually improved without any specific treatment. This unique case represents the first report of mild encephalopathy/encephalitis with reversible splenial lesions associated with amphetamine and opioid use.

Deciphering the Neuropsychiatric Landscape of Huntington’s Disease: Insights and Implications—A Case Report

Huntington’s disease (HD) is an autosomal dominant neurodegenerative illness with a specific phenomenology that includes chorea, incoordination, dystonia, behavioral difficulties, and cognitive decline. The most frequent cause of inherited chorea manifesting in adults is HD, which affects approximately 5 per 100,000 to 12 per 100,000 people. This case report describes the case of a 42-year-old woman from Assam who had been experiencing progressive and gradual onset choreiform movements of both upper limbs, affecting the fingers and face for the last two years, along with an unsteady gait and slurred speech and lack of personal care and hygiene. She also developed psychotic manifestations in the form of erotomanic delusions along with decreased sleep, restlessness, and disorganized behavior. A positive family history of dementia in their maternal grandmother and movement disorder in their paternal grandmother, along with the history of the death of her elder brother at birth, was present. The patient was admitted considering HD as a provisional diagnosis, and treatment with tablets tetrabenazine, olanzapine, risperidone, and lorazepam for her behavioral and movement symptoms was initiated. Her magnetic resonance imaging (MRI) brain report showed caudate atrophy. HD repeat expansion analysis that revealed full penetrance expansion of approximately 46 coronary angiogram (CAG) repeats in either allele was confirmatory of HD. There was an improvement in her symptoms seen in two follow-up visits to the outpatient department (OPD) after discharge except for reduced sleep; hence, melatonin 10 mg was added for it. Middle-aged patients presenting to psychiatrists with choreiform movement and psychiatric symptoms along with a positive family history should raise the suspicion of HD.

Safety of 177Lu-DOTATATE administration in a patient with recurrent meningioma complicated by radiation necrosis: a case report

Radiation-associated meningiomas are characterized by high rates of recurrence. 177Lu-DOTATATE may represent a treatment option for these tumors, but safety of this agent for patients with a history of radiation necrosis is unknown. In this case report, we describe the preliminary safety of 177Lu-DOTATATE administration for a patient with prior history of radiation necrosis. The patient was diagnosed with central nervous system (CNS) germinoma at age 17, managed with chemoradiation. Twenty years later, a left-parietal extra-axial mass was identified within the prior radiation field, with diagnosis of atypical meningioma established on subtotal resection. He received additional radiation therapy (RT, 60 Gy, 30 fractions). Seventeen months later, tumor progression was treated with gamma knife radiosurgery (GKRS, 15 Gy, single fraction). Seven months after GKRS, seizures recurred, with imaging consistent with radiation necrosis. The patient was treated with bevacizumab (7.5 mg/kg, every 3 weeks, 4 doses). One year later, magnetic resonance imaging (MRI) brain revealed tumor progression within the treatment field. 68 Ga-DOTATATE positron emission tomography demonstrated that the meningioma was DOTATATE-avid with no evidence of metastasis. The patient was treated with 177Lu-DOTATATE (two infusions, two months apart), which was well tolerated with no significant toxicities and no recurrence of radiation necrosis. Two months after the second infusion, the patient experienced disease progression and was transitioned to bevacizumab. In this patient with three prior courses of intracranial radiation and subsequent radiation necrosis, 177Lu-DOTATATE was well-tolerated. 177Lu-DOTATATE administration may be feasible for patients with a history of radiation necrosis.

Choroid plexus enlargement in patients with end-stage renal disease: implications for glymphatic system dysfunction.

The choroid plexus plays a role in eliminating detrimental metabolites from the brain as an integral component of the glymphatic system. This study aimed to investigate alterations in choroid plexus volume in patients with end-stage renal disease (ESRD) compared with healthy controls. We enrolled 40 patients with ESRD and 42 healthy controls. They underwent brain magnetic resonance imaging (MRI), specifically using three dimensional T1-weighted imaging. We analyzed choroid plexus volumes and compared them between patients with ESRD and healthy controls. The diffusion tensor image analysis along the perivascular space (DTI-ALPS) index was calculated. We compared the DTI-ALPS index between the ESRD patients and healthy controls. Additionally, we evaluated the association between choroid plexus volume and neuropsychological tests results in patients with ESRD. There were significant differences in choroid plexus volumes between patients with ESRD and healthy controls. The choroid plexus volumes in patients with ESRD were higher than those in healthy controls (1.392 vs. 1.138%, p < 0.001). The DTI-ALPS index in patients with ESRD was lower than that in healthy controls (1.470 ± 0.239 vs. 1.641 ± 0.266, p = 0.005). There were no differences in choroid plexus volumes between patients with ESRD, regardless of the presence of cognitive impairment. However, among the neuropsychological tests, the scores for word-list recognition in verbal memory were negatively correlated with the choroid plexus volume (r = -0.428, p = 0.006). We demonstrated a significant enlargement of the choroid plexus volume in patients with ESRD compared to healthy controls. This finding suggests that patients with ESRD have glymphatic system dysfunction, which may be related to cognitive impairment.

Isolated central nervous system lymphomatoid granulomatosis in an older adult patient with systematic lupus erythematosus: A case report.

Lymphomatoid granulomatosis (LYG) is a rare, T-cell-rich Epstein-Barr virus (EBV)-positive B-cell lymphoproliferative systemic disease. Only a few cases of LYG have been described in patients with autoimmune disorders, with only one case described in a patient with systemic lupus erythematosus (SLE). However, no cases of isolated central nervous system (CNS)-LYG have been reported in patients with autoimmune diseases. Since isolated CNS involvement is rare, its clinicopathological features remain incompletely understood. Herein, we report about an 85-year-old Japanese woman who was diagnosed with SLE 26 years ago and was stable and maintained on prednisone (5 mg/day) for 20 years. Twenty-six days before admission, she developed cognitive dysfunction. Brain magnetic resonance imaging (MRI) revealed multiple nodular lesions with ring-like enhancements seen on gadolinium-enhanced T1-weighted imaging. A brain biopsy was performed on the right frontal lobe lesion. Pathological findings revealed T-cell infiltration surrounding the blood vessels with fibrin deposition, a few multinucleated cells, and large atypical cells with prominent nucleoli. Large atypical cells positive for CD20 and EBV-encoded RNA (EBER) were seen at a density of >100 cells per high-power field. Based on laboratory testing, imaging, and pathology findings, the patient was diagnosed with grade III LYG. Treatment with tirabrutinib (480 mg once daily) was started. The patient achieved clinical response to treatment, as evidenced by improved mental status. In patients with SLE who present with multiple nodular or ring-like lesions on brain MRI, brain biopsy with histological diagnosis is crucial for the exclusion of CNS-LYG.

Case report: Encephalitis with Guillain-Barré Syndrome secondary to extensively multi-drug resistant Salmonella infection

Abstract Background This case report presents a highly unusual and challenging medical case involving a 16-month-old previously healthy boy who developed encephalitis followed by Guillain-Barré Syndrome (GBS) as complications of multi-drug resistant Salmonella infection, despite an initial misdiagnosis of gastroenteritis and outpatient treatment with Cefixime. Methods The patient initially experienced an increase in stool frequency, accompanied by low-grade fevers, which was initially attributed to gastroenteritis. Outpatient treatment with oral Cefixime was started, but the child’s symptoms worsened, ultimately leading to high-grade fevers and an increase in vomiting and loose stools. In light of his clinical deterioration, he was admitted to our hospital with signs of mild metabolic acidosis and elevated inflammatory markers. During his hospitalization, the patient’s condition worsened, with drowsiness, irritability, and a generalized tonic-clonic seizure, prompting concern for brain involvement. Consequently, he underwent lumbar puncture, and neuroimaging, and received high-dose intravenous steroids. Further medical investigations including lumbar puncture and brain Magnetic Resonance Imaging (MRI), which revealed normal cerebrospinal fluid (CSF) analysis and non-enhancing white matter changes in the occipital region, respectively. In addition, a collected blood culture confirmed the presence of extensively multi-drug resistant Salmonella typhi and prompted an adjustment of antibiotics to intravenous Meropenem. Due to autonomic dysfunction, the patient was transferred to the Pediatric Intensive Care Unit (PICU) for close monitoring and specialized care. While in the PICU, he developed flaccid limb weakness and axial hypotonia. A repeat lumbar puncture was normal, but nerve conduction studies showed features of axonal polyneuropathy diagnosis, consistent with the diagnosis of GBS. To treat the encephalopathy and polyneuropathy, high-dose dexamethasone and Intravenous Immunoglobulin (IVIG) treatments were initiated, without necessitating respiratory or inotropic support. After receiving these treatments, the patient’s autonomic instability and motor strength improved, prompting his transfer back to the general pediatrics wards. He was treated with a 21-day course of IV Meropenem. Upon discharge, his motor power had returned to normal, and he displayed progress in movement transitions, including the ability to stand and walk independently, albeit with mild gait abnormalities. Results The co-occurrence of encephalitis and Guillain-Barré Syndrome due to Typhoid fever is exceedingly rare. The patient’s origin, with recent travel to Pakistan where extensively drug-resistant Salmonella typhi prevails, necessitates heightened awareness among healthcare providers. Initial misdiagnosis and inadequate antibiotics treatment may have contributed to encephalitis and polyneuropathy. High-dose dexamethasone and IVIG were pivotal in the patient’s treatment. While Typhoid fever-related neurological complications are infrequent, high-dose dexamethasone offers promise in reducing mortality and mitigating typhoid-induced encephalopathy. Importantly, IVIG is not conventionally employed in Salmonella encephalopalitis but was used due to polyneuropathy and its role in GBS management. Conclusion This exceptional pediatric case serves as a striking example of the possibility simultaneous encephalitis and Guillain-Barré Syndrome as rare complications of Typhoid fever. This case report underscores the importance of early diagnosis, prompt management with appropriate antibiotics and caution of possible neurological complications. Furthermore, it calls for further research into the role of high-dose glucocorticoids and IVIG in the management of extensively multi-drug resistant Salmonella infection and its neurological complications.

ADVANCEMENTS IN ALZHEIMER’S DIAGNOSIS THROUGH MRI USING BAYESIAN CONVOLUTIONAL NEURAL NETWORKS AND VARIATIONAL INFERENCE

Alzheimer’s disease is one of the brain disorders that can be deadly in older. The disease is less treated and less recognized, but Alzheimer’s disease is now a significant public health problem. Early detection of the disease can significantly reduce symptoms. However, the lack of medical personnel makes handling this disease complex. Therefore, an automatic diagnosis of Alzheimer’s disease is needed with a Magnetic Resonance Imaging (MRI) examination to get an accurate diagnosis of the disease. This study classified the type of Alzheimer’s disease with deep learning methods using the Bayesian Convolutional Neural Network (BCNN) and the Variational Inference (VI) technique. It aims to determine image classification and accuracy level at the level of Alzheimer’s disease by using 2,400 brain MRI images, divided into three classes (non-demented, very mild demented, and mild demented) based on severity. The data was acquired from the kaggle.com website. We use a dataset scenario of 80% for training and 20% for testing, 100x100 pixels, kernel size 3x3, and optimizer Adam with epoch 200. The accuracy of the image classification process is 80%. The non-demented label predicts that the uncertainty is 0.371, and the other uncertainty prediction is 0.002. The ability to anticipate uncertainty enables clinicians to make informed decisions regarding the reliability of the model’s output and the need for additional validation or confirmation.

Silent brain ischemia within the TAXINOMISIS framework: association with clinical and advanced ultrasound metrics.

IntroductionThe relationship between carotid artery stenosis (CAS) and ipsilateral silent brain ischemia (SBI) remains unclear, with uncertain therapeutic implications. The present study, part of the TAXINOMISIS project (nr. 755,320), aimed to investigate SBIs in patients with asymptomatic CAS, correlating them with clinical, carotid ultrasonographic data, and CFD analyses.MethodsThe TAXINOMISIS clinical trial study (nr. NCT03495830) involved six vascular surgery centers across Europe, enrolling patients with asymptomatic and symptomatic CAS ranging from 50 to 99%. Patients underwent carotid ultrasound and magnetic resonance imaging (MRI), including brain diffusion-weighted, T2-weighted/FLAIR, and T1-weighted sequences. Brain MRI scans were analyzed for the presence of SBI according to established definitions. Ultrasound assessments included Doppler and CFD analysis. Only asymptomatic patients were included in this substudy.ResultsAmong 195 asymptomatic patients, the mean stenosis (NASCET) was 64.1%. Of these, a total of 33 patients (16.9%) had at least one SBI detected on a brain MRI scan. Specifically, 19 out of 33 patients (57.6%) had cortical infarcts, 4 out of 33 patients (12.1%) had ipsilateral lacunar infarcts, 6 out of 33 patients had (18.2%) subcortical infarcts, 1 out of 33 patients (3.0%) had both cortical and lacunar infarcts, and 3 out of 33 patients (9.1%) both cortical and subcortical infarcts. Patients with SBIs exhibited significantly higher risk factors, including a higher body mass index (28.52 ± 9.38 vs. 26.39 ± 3.35, p = 0.02), diastolic blood pressure (80.87 ± 15.73 mmHg vs. 80.06 ± 8.49 mmHg, p = 0.02), creatinine levels (93.66 ± 34.61 μmol/L vs. 84.69 ± 23.67 μmol/L, p = 0.02), and blood triglycerides (1.8 ± 1.06 mmol/L vs. 1.48 ± 0.78 mmol/L, p = 0.03). They also had a higher prevalence of cardiovascular interventions (29.6% vs. 13.8%, p = 0.04), greater usage of third/fourth-line antihypertensive treatment (50%vs16%, p = 0.03), and anticoagulant medications (60% vs. 16%, p = 0.01). Additionally, the number of contralateral cerebral infarcts was higher in patients with SBIs (35.5% vs. 13.4%, p &amp;lt; 0.01). Moreover, carotid ultrasound revealed higher Saint Mary’s ratios (15.33 ± 12.45 vs. 12.96 ± 7.99, p = 0.02), and CFD analysis demonstrated larger areas of low wall shear stress (WSS) (0.0004 ± 0.0004 m2 vs. 0.0002 ± 0.0002 m2, p &amp;lt; 0.01).ConclusionThe TAXINOMISIS clinical trial provides valuable insights into the prevalence and risk factors associated with SBIs in patients with moderate asymptomatic carotid stenosis. The findings suggest that specific hemodynamic and arterial wall characteristics may contribute to the development of silent brain infarcts.

Intravenous anakinra for treating macrophage activation syndrome in adult onset still’s disease

Background: Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disease characterized by fever, rash, arthritis, and multi-organ involvement. Macrophage activation syndrome (MAS), a serious complication of AOSD, poses significant diagnostic and therapeutic challenges. Case Presentation: A 32-year-old male was diagnosed with AOSD in 2020 after being hospitalized for a fever of unknown origin and elevated liver enzymes. The patient was initially treated with corticosteroids and methotrexate but subsequently discontinued both treatment and follow-up. In September 2023, he presented with fever, sore throat, and elevated inflammatory markers. After screening for infections, methylprednisolone (MP) treatment was initiated because of AOSD activation. The following day, the patient was admitted to the intensive care unit due to an altered state of consciousness. Brain magnetic resonance imaging revealed brainstem involvement. Empirical treatments were initiated, including intravenous MP, and immunoglobulin therapy. Due to suspected macrophage activation syndrome (MAS), anakinra (ANA) infusion was initiated. Significant improvement was observed after the ANA infusion. Conclusion: This case highlights the complex management of severe AOSD complications, emphasizing the role of early recognition, aggressive therapy, and multidisciplinary care in improving outcomes.

Comparison of brief olfactory and cognitive assessments to neuroimaging biomarkers in the prediction of cognitive decline and dementia in the MCSA cohort.

We evaluated impaired odor identification and global cognition as simple, cost-effective alternatives to neuroimaging biomarkers to predict cognitive decline and dementia in the Mayo Clinic Study of Aging. Six hundred forty-seven participants (mean 8.1, standard deviation 3.4 years' follow-up) had the following baseline procedures: modified Blessed Information Memory Concentration Test (BIMCT), 12-item Brief Smell Identification Test (BSIT), structural brain magnetic resonance imaging (MRI), and positron emission tomography (PET) imaging with 11C-Pittsburgh compound B (11C-PiB) and fluorodeoxyglucose (FDG; subset). Cognitive decline developed in 102 participants and dementia in 34 participants. In survival analyses, PiB PET showed robust prediction for cognitive decline. Impaired BSIT, impaired BIMCT, MRI, and FDG measures were also significant predictors. The combination of demographics + BSIT + BIMCT showed strong predictive utility (C-index 0.81), similar to demographics + PiB PET (C-index 0.80). Similar but stronger results were obtained for prediction of dementia. Impairment in both odor identification test and global cognition was comparable to PiB PET for predicting cognitive decline and dementia. In 647 participants in the population-based Mayo Clinic Study of Aging, several clinical markers and biomarkers each predicted cognitive decline or dementia during an average 8 years of follow-up. The combination of the demographic variables of age, sex, and education with a brief odor identification test (BSIT) and a global cognitive test (Blessed Information Memory Concentration Test) showed strong predictive utility (C-index 0.81) for cognitive decline that was similar to the demographic variables combined with Pittsburgh Compound B amyloid imaging (C-index 0.80). Combining a brief odor identification test with a brief cognitive test needs consideration as a simple, cost-effective option in the clinical assessment of individuals at risk of cognitive decline and dementia, as well as a potential tool to identify individuals who may benefit from disease-modifying treatments and to screen participants for prevention trials.

Rocky Mountain Spotted Fever Encephalitis and "Starry Sky" Pattern on MRI: A Case Report.

Rocky Mountain Spotted Fever (RMSF) is a tick-borne disease caused by Rickettsia rickettsii (R. rickettsii). RMSF presents after a tick bite with fever, rash, and headache but can also cause more serious neurological manifestations. We report a case of RMSF encephalitis presenting with altered sensorium and rapid progression to coma, fever, and petechial rash, and an magnetic resonance imaging (MRI) brain notable for a "starry sky" pattern. A 61-year-old woman presented with confusion and fever and was diagnosed with a urinary tract infection. Two days later, she became comatose. MRI brain revealed lacunar infarcts in the right centrum semiovale and splenium of the corpus callosum. Lumbar puncture was notable for neutrophilic pleocytosis and elevated protein with negative bacterial and viral cultures. Empiric meningitis therapy was initiated, and she was transferred to our institution. On transfer, she was febrile, comatose, and had a diffuse petechial rash. Repeat MRI brain demonstrated diffuse, innumerable punctate foci of diffusion restriction with susceptibility-weighted signal attenuation throughout cerebral hemispheres in a "starry sky" pattern. Skin biopsy revealed perivascular lymphocytic infiltrates. Serologic RSMF antibody titers were obtained, and doxycycline was initiated for presumed RMSF encephalitis. The family opted to pursue palliative measures, given no clinical improvement. RSMF titers and postmortem PCR from brain tissue were positive for R. rickettsii. This case report highlights the clinical presentation of RMSF encephalitis. RMSF encephalitis should be suspected in a patient presenting with encephalopathy, fever, petechial rash, and MRI brain findings of diffuse punctate foci of diffusion restriction and susceptibility-weighted signal attenuation in a "starry-sky" pattern.

Adjuvant High-Dose Erythropoietin With Delayed Therapeutic Hypothermia in Neonatal Hypoxic-Ischemic Encephalopathy

BackgroundTo evaluate the benefits of high-dose erythropoietin (EPO) combined with therapeutic hypothermia (TH) on brain magnetic resonance imaging (MRI) scores and neurodevelopmental outcomes in neonates with moderate to severe hypoxic-ischemic-ecephalopathy (HIE), especially in neonates who received TH between six to 12 hours of birth. MethodsThis prospective, single-blind, randomized, placebo-controlled trial enrolled term newborns with moderate to severe HIE admitted to neonatal intensive care unit between April 2018 and April 2022. Hypothermia was started within 12 hours of birth. Infants were randomized to receive EPO 1000 U/kg or an equal volume of normal saline (placebo) on days 1, 2, 3, 5, and 7 of age in combination with hypothermia. ResultsFifty-seven neonates with moderate to severe HIE were recruited; 10 were excluded. Forty-seven patients were included: 32 received TH within six hours (group I) and in 15 TH was started within six to 12 hours of birth (group II). The clinical characteristics of mothers and infants, maternal complications, and resuscitations performed during the perinatal period showed no statistical differences between EPO group and placebo groups I and II. The MRI scores and brain injury patterns did not differ between the EPO and placebo groups. There is no statistical significance in either group's seizure and severe electroencephalography background (initial and after rewarming) between EPO and placebo in each group. There were no differences in developmental outcomes (abnormal Denver II > 2 area, Gross Motor Function Classification Score >1); Bayley Scales of Infant and Toddler Development, third edition (BSID-III) score (cognitive, language, and motor); or disability (hearing impairment and impaired vision) between the EPO and placebo groups I and II at 12 and 18 months. ConclusionsAmong term infants with moderate to severe HIE, TH with EPO administration, compared with TH alone, did not reduce brain injury on MRI or the risk of neurological sequelae both in patients who received TH within six hours and in those who received TH later (six to 12 hours). Further studies on the benefit of EPO injection alone or before TH in situations where TH cannot be performed are required.

Algorithm development and metal oxide nanoparticle analysis in magnetic resonance imaging: Advancing neurodegenerative disease diagnostics

Magnetic resonance imaging (MRI) is critical in the diagnosis of neurodegenerative diseases, enabling the detection of brain lesions. Recent research has examined metallic nanoparticles (NPs) as MRI contrast agents (CAs) that can enhance lesion visibility by altering relaxation times. This study investigates the effects of metal oxide NPs on MRI relaxation times and brain lesion signals and proposes an algorithm for automated relaxation time determination using these NPs. The utilized NPs were synthesized using the sol‒gel method and characterized using Fourier-transform infrared spectroscopy and X-ray diffraction. MRI scans were performed on a phantom infused with varying concentrations of each metal oxide NP to assess changes in pixel signal intensities and relaxation rates. Our analysis involved segmenting the MRI images to focus on regions with different NP concentrations. The algorithm computed the longitudinal relaxation time for each region, revealing that Fe2O3 NPs exhibited the most substantial effect on signal intensity and relaxation time. The results indicated a high correlation (r = 0.9977), demonstrating strong agreement and confirming the reliability of our method. Our findings suggest that metallic oxide NPs, particularly Fe2O3, can considerably alter magnetization and act as effective negative CAs in MRI. These capabilities can improve the monitoring and treatment efficacy of neurodegenerative diseases. Our method for quantifying longitudinal relaxation times can potentially enhance routine clinical MRI assessments, offering a promising tool for future clinical applications.

Accelerating multi-coil MR image reconstruction using weak supervision.

Deep-learning-based MR image reconstruction in settings where large fully sampled dataset collection is infeasible requires methods that effectively use both under-sampled and fully sampled datasets. This paper evaluates a weakly supervised, multi-coil, physics-guided approach to MR image reconstruction, leveraging both dataset types, to improve both the quality and robustness of reconstruction. A physics-guided end-to-end variational network (VarNet) is pretrained in a self-supervised manner using a 4 under-sampled dataset following the self-supervised learning via data undersampling (SSDU) methodology. The pre-trained weights are transferred to another VarNet, which is fine-tuned using a smaller, fully sampled dataset by optimizing multi-scale structural similarity (MS-SSIM) loss in image space. The proposed methodology is compared with fully self-supervised and fully supervised training. Reconstruction quality improvements in SSIM, PSNR, and NRMSE when abundant training data is available (the high-data regime), and enhanced robustness when training data is scarce (the low-data regime) are demonstrated using weak supervision for knee and brain MR image reconstructions at 8 and 10 acceleration, respectively. Multi-coil physics-guided MR image reconstruction using both under-sampled and fully sampled datasets is achievable with transfer learning and fine-tuning. This methodology can provide improved reconstruction quality in the high-data regime and improved robustness in the low-data regime at high acceleration rates.