Pediatric Acute Hemorrhagic Leukoencephalitis (Weston-Hurst syndrome): A Rare Entity

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S38-S38
Author(s):  
H Tomac Pavosevic ◽  
S Zaheer ◽  
G M Kleinman
Keyword(s):  
White Matter ◽  
Brain Mri ◽  
Brain Lesion ◽  
Brain Biopsy ◽  
Acute Disseminated Encephalomyelitis ◽  
Small Vessel Vasculitis ◽  
Hyperintense Lesion ◽  
Acute Hemorrhagic Leukoencephalitis ◽  
Brain White Matter ◽  
The Brain

Abstract Introduction/Objective Acute hemorrhagic leukoencephalitis (Weston-Hurst syndrome) is a rare, fulminant inflammatory hemorrhagic demyelination of the brain white matter, and it is considered to be the most severe form of acute disseminated encephalomyelitis (ADEM). Herein we describe a case of 6-year-old boy presenting with elevated intracranial pressure and uncal herniation. Brain MRI showed a large right temporoparietal T2 hyperintense lesion with edema, abnormal perivascular enhancement and midline shift. Emergent decompressive craniotomy with brain biopsy was performed. Results H&E sections of the brain lesion revealed neutrophilic small vessel vasculitis with extravasation of blood and fibrin. Predominantly in a perivascular distribution were infiltrates of neutrophils and in areas foamy macrophages. Additionally, the white matter was studded with hemorrhages of varying size. Sections stained with Luxol Fast Blue for myelin and with a neurofilament immunostain for axons were compared to reveal selective perivascular loss of myelin with relative sparing of axons. Conclusion The constellation of acute hemorrhages, neutrophilic infiltration and selective loss of myelin with relative sparing of axons is characteristic of this exceptional, hyperacute variant of acute disseminated encephalomyelitis. Histomorphologic diagnosis is characteristic and may be crucial for timely decision for treatment of this often fatal disease.

scholarly journals Diagnostic Significance of Hyperintense Lesion in the Pons on Brain Magnetic Resonance Imaging in Patients with Intravascular Lymphoma

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4214-4214
Author(s):  
Yoshiaki Abe ◽  
Kentaro Narita ◽  
Hiroki Kobayashi ◽  
Akihiro Kitadate ◽  
Masami Takeuchi ◽  
...  
Keyword(s):  
White Matter ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
White Matter Lesions ◽  
B Cell Lymphoma ◽  
Impaired Consciousness ◽  
Hyperintense Lesion ◽  
Random Skin Biopsy ◽  
The Brain

Abstract Introduction: Neurological symptoms related to the involvement of the central nervous system (CNS) have been commonly observed at diagnosis and at relapse in intravascular large B-cell lymphoma (IVLBCL). Although various patterns of abnormal findings on brain magnetic resonance imaging (MRI) in patients with IVLBCL have been reported, most of them were from case reports or small case series of selected patients.Hence, we aimed to investigate the prevalence and clinical value of abnormal findings detected using brain MRI in patients with IVLBCL regarding diagnosis and prognosis. Methods: A total of 33 consecutive patients diagnosed with IVLBCL who underwent treatment at Kameda Medical Center between 1998 and 2017 were available for data of routine pretreatment brain MRI.The diagnosis of IVLBCL was pathologically made by an expert hematopathologist (KT) in all patients. Brain MRI was performed as previously reported, and the abnormalities were classified into the following 4patterns by 2 neuroradiologists in consensus (Figure 1): (A) hyperintense lesion in the pons on T2-weighted imaging (T2WI), (B) nonspecific white matter lesions, (C) infarct-like lesions, and (D) meningeal thickening and/or enhancement. We subsequently identified 77 consecutive patients (52 patients at initial presentation and 25 patients at relapse) with pathologically diagnosed diffuse large B-cell lymphoma (DLBCL) without IVLBCL and 41 patients who received random-skin biopsy on the suspicion of IVLBCL but were found to be negative, as control groups for the presence or absence of hyperintense lesions in the pons. Results: Pretreatment brain MRI revealed abnormal findings in 29 (87.9%) patients. Hyperintense lesions in the pons on T2WI was the most common abnormal finding and was detected in 19 (65.5%) patients. Among them, 10 (52.6%) patients did not have impaired consciousness (Figure 2). Among the 7 patients in whom hyperintense lesions in the pons on T2WI was the sole abnormality, 5 patients (71.4%) did not have impaired consciousness. Infarct-like lesions were detected in 8 (27.6%) patients, and impaired consciousness was more frequent in patients with this pattern than in those without (87.5% vs. 28.0%; P=0.005). Nonspecific white matter lesions and meningeal thickening and/or enhancement were detected in 14 (48.3%) and 4 (13.8%) patients, respectively. No significant difference in overall survival (OS) was detected between patients with and without hyperintense lesions in the pons on T2WI (Figure 3). Patients with nonspecific white matter lesions had relatively shorter OS than those without the finding, although the difference was not statistically significant (median OS, 19.8 months vs. not achieved; P=0.057). Infarct-like lesions were associated with unfavorable survival (median OS, 12.5 months vs. not achieved; P=0.030). Follow-up brain MRI revealed improvements in abnormal findings in most of the patients who responded to chemotherapy (Figure 4). Furthermore, postmortem examinations revealed pathological changes in the brain related to the lymphoma lesions, indicating that these MRI findings might represent these lesions of the brain. Next, we reviewed findings on brain MRI in 77 control patients with DLBCL without IVLBCL. Among them, 16 (20.8%) patients had concomitant CNS involvement of lymphoma. No patients harbored hyperintense lesions in the pons, in contrast with the patients with IVLBCL (P<0.001). This finding was detected in no patient also among those who received random-skin biopsy on the suspicion of IVLBCL but were found to be negative (P<0.001). Conclusions: Our findings revealed that most patients with IVLBCL presented abnormal findings on pretreatment brain MRI, even if they exhibited no neurological symptoms. In particular, hyperintense lesions in the pons on T2WI were frequently observed in patients with IVLBCL, irrespective of the presence or absence of impaired consciousness, and were highly specific in IVLBCL compared to those in control groups, suggesting that this pattern may be pathognomonic and valuable for the timely diagnosis of IVLBCL. Improvements in all types of abnormal findings on follow-up brain MRI indicated that these findings might reflect structural changes associated with IVLBCL and might be useful for confirmation of the therapeutic effect. Further longitudinal studies are required to validate our findings and determine their clinical implications. Disclosures No relevant conflicts of interest to declare.

scholarly journals Transfer Learning for Task Adaptation of Brain Lesion Assessment and Prediction of Brain Abnormalities Progression/Regression using Irregularity Age Map in Brain MRI

2018 ◽  
Author(s):  
Muhammad Febrian Rachmadi ◽  
Maria del C. Valdés-Hernández ◽  
Taku Komura
Keyword(s):  
Machine Learning ◽  
White Matter ◽  
Transfer Learning ◽  
White Matter Hyperintensities ◽  
Brain Mri ◽  
Brain Lesion ◽  
Transfer Task ◽  
Mri Brain ◽  
Brain White Matter ◽  
Task Adaptation

AbstractThe Irregularity Age Map (IAM) for the unsupervised assessment of brain white matter hyperintensities (WMH) opens several opportunities in machine learning-based brain MRI analysis, including transfer task adaptation learning in the MRI brain lesion’s segmentation and prediction of lesion progression and regression. The lack of need for manual labels is useful for transfer learning. Whereas, the nature of IAM itself can be exploited for predicting lesion progression/regression. In this study, we propose the use of task adaptation transfer learning for WMH segmentation using CNN through weakly-training UNet and UResNet using the output from IAM and the use of IAM for predicting patterns of WMH progression and regression.

scholarly journals A Case of Acute Disseminated Encephalomyelitis in a Middle-Aged Adult

2015 ◽  
Vol 2015 ◽  
pp. 1-4
Author(s):  
Nicole Mahdi ◽  
Peter A. Abdelmalik ◽  
Mark Curtis ◽  
Barak Bar
Keyword(s):  
White Matter ◽  
Brain Mri ◽  
Brain Biopsy ◽  
Acute Disseminated Encephalomyelitis ◽  
Steroid Treatment ◽  
Neurological Deficits ◽  
High Dose ◽  
Middle Aged ◽  
Neurological Exam ◽  
Middle Aged Adult

Objectives. Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disorder that is often preceded by infection or recent vaccination. Encephalopathy and focal neurological deficits are usually manifest several weeks after a prodromal illness with rapidly progressive neurologic decline. ADEM is most commonly seen in children and young adults, in which prognosis is favorable, but very few cases have been reported of older adults with ADEM and thus their clinical course is unknown.Methods. Here we present a case of ADEM in a middle-aged adult that recovered well after treatment.Results. A 62-year-old man presented with encephalopathy and rapid neurological decline following a gastrointestinal illness. A brain MRI revealed extensive supratentorial white matter hyperintensities consistent with ADEM and thus he was started on high dose intravenous methylprednisolone. He underwent a brain biopsy showing widespread white matter inflammation secondary to demyelination. At discharge, his neurological exam had significantly improved with continued steroid treatment and four months later, he was able to perform his ADLs.Conclusions. This case of ADEM in a middle-aged adult represents an excellent response to high dose steroid treatment with a remarkable neurological recovery. Thus it behooves one to treat suspected cases of ADEM in an adult patient aggressively, as outcome can be favorable.

On the microstructural origin of brain white matter hydraulic permeability

2021 ◽  
Vol 118 (36) ◽  
pp. e2105328118
Author(s):  
Marco Vidotto ◽  
Andrea Bernardini ◽  
Marco Trovatelli ◽  
Elena De Momi ◽  
Daniele Dini
Keyword(s):  
White Matter ◽  
Corpus Callosum ◽  
Drug Transport ◽  
Three Dimensional ◽  
Principal Component ◽  
Hydraulic Permeability ◽  
Convection Enhanced Delivery ◽  
Brain White Matter ◽  
Significant Difference ◽  
The Brain

Brain microstructure plays a key role in driving the transport of drug molecules directly administered to the brain tissue, as in Convection-Enhanced Delivery procedures. The proposed research analyzes the hydraulic permeability of two white matter (WM) areas (corpus callosum and fornix) whose three-dimensional microstructure was reconstructed starting from the acquisition of electron microscopy images. We cut the two volumes with 20 equally spaced planes distributed along two perpendicular directions, and, on each plane, we computed the corresponding permeability vector. Then, we considered that the WM structure is mainly composed of elongated and parallel axons, and, using a principal component analysis, we defined two principal directions, parallel and perpendicular, with respect to the axons’ main direction. The latter were used to define a reference frame onto which the permeability vectors were projected to finally obtain the permeability along the parallel and perpendicular directions. The results show a statistically significant difference between parallel and perpendicular permeability, with a ratio of about two in both the WM structures analyzed, thus demonstrating their anisotropic behavior. Moreover, we find a significant difference between permeability in corpus callosum and fornix, which suggests that the WM heterogeneity should also be considered when modeling drug transport in the brain. Our findings, which demonstrate and quantify the anisotropic and heterogeneous character of the WM, represent a fundamental contribution not only for drug-delivery modeling, but also for shedding light on the interstitial transport mechanisms in the extracellular space.

6. What do more intelligent brains look like?

2020 ◽  
pp. 83-90
Author(s):  
Ian J. Deary
Keyword(s):  
White Matter ◽  
General Intelligence ◽  
Total Brain Volume ◽  
Brain White Matter ◽  
Total Brain ◽  
Lothian Birth Cohort ◽  
Intelligence Test Scores ◽  
Intelligence Scores ◽  
The Brain ◽  
Brain Connections

‘What do more intelligent brains look like?’ considers a study that used data from the Lothian Birth Cohort 1936 to test the strength of the correlation between the general intelligence scores of the participants and different measures of their brain’s structure. Magnetic resonance imaging was used to measure total brain volume, brain cortical thickness, brain white matter integrity (or health), and brain white matter hyperintensities. The study showed that people who have higher general intelligence tend to have larger brains, thicker grey matter on the surface of the brain, and healthier white matter brain connections. The associations are not strong, but some aspects of brain structure do relate to intelligence test scores.

Microstructural Hyperelastic Characterization of Brain White Matter in Tension

2019 ◽  
Author(s):  
Mohammadreza Ramzanpour ◽  
Mohammad Hosseini-Farid ◽  
Mariusz Ziejewski ◽  
Ghodrat Karami
Keyword(s):  
Finite Element ◽  
White Matter ◽  
Human Brain ◽  
Volume Fraction ◽  
Fibrous Composite ◽  
Diffuse Axonal Injury ◽  
Stiffness Ratio ◽  
Fibrous Composite Material ◽  
Brain White Matter ◽  
The Brain

Abstract Axons as microstructural constituent elements of brain white matter are highly oriented in extracellular matrix (ECM) in one direction. Therefore, it is possible to model the human brain white matter as a unidirectional fibrous composite material. A micromechanical finite element model of the brain white matter is developed to indirectly measure the brain white matter constituents’ properties including axon and ECM under tensile loading. Experimental tension test on corona radiata conducted by Budday et al. 2017 [1] is used in this study and one-term Ogden hyperelastic constitutive model is applied to characterize its behavior. By the application of genetic algorithm (GA) as a black box optimization method, the Ogden hyperelastic parameters of axon and ECM minimizing the error between numerical finite element simulation and experimental results are measured. Inverse analysis is conducted on the resultant optimized parameters shows high correlation of coefficient (&gt;99%) between the numerical and experimental data which verifies the accuracy of the optimization procedure. The volume fraction of axons in porcine brain white matter is taken to be 52.7% and the stiffness ratio of axon to ECM is perceived to be 3.0. As these values are not accurately known for human brain white matter, we study the material properties of axon and ECM for different stiffness ratio and axon volume fraction values. The results of this study helps to better understand the micromechanical structure of the brain and micro-level injuries such as diffuse axonal injury.

Brain Microvasculature Involvement in ANCA Positive Vasculitis

2016 ◽  
Vol 41 (5-6) ◽  
pp. 313-321 ◽  
Author(s):  
Inés González-Suárez ◽  
Javier Arpa ◽  
Juan José Ríos-Blanco
Keyword(s):  
Blood Flow ◽  
White Matter ◽  
Endothelial Dysfunction ◽  
Arterial Stiffness ◽  
Linear Regression Analysis ◽  
Brain Mri ◽  
Mean Flow ◽  
Brain Involvement ◽  
Brain Microvasculature ◽  
The Brain

Objective: Endothelial dysfunction is associated with arterial stiffness, a factor that is increasingly recognised as an important determinant of cardiovascular risk. High-flow organs such as the brain and kidneys are particularly sensitive to excessive pressure and flow pulsatility. High, local blood flow is associated with low microvascular impedance, which facilitates the penetration of excessive pulsatile energy into the microvascular bed leading to tissue damage. Systemic endothelial dysfunction and arterial stiffness have been demonstrated in peripheral vessels in associated vasculitis (AAV). Although, the brain involvement is not infrequent in AAV, it has not been evaluated previously. Our aim is to evaluate the involvement of the brain microvasculature in AAV. Methods: Twenty-three patients with inactive AAV were studied. Brain blood flow was assessed by transcranial Doppler (TCD) and single-photon positron emission tomography (SPECT), structural brain involvement by brain MRI and cognitive scores by Montreal Cognitive Assessment (MoCA) test. Results: Lower mean flow velocity (MFV) was associated to altered SPECT perfusion, higher white matter changes (WMC), lower MoCA scores and younger age (p < 0.05). Middle cerebral artery pulsatility index (MCA-PI) was related to hypertension, diabetes, lower scores on MoCA, increased vasculitis damage index (VDI) and perfusion impairment in SPECT (p < 0.05). These data were reproduced for all intracranial arteries. Up to 88.9% of patients had WMC on MRI. A higher lesion load was associated with age, decreased MoCA and fewer MFV with higher PI. The multivariable linear regression analysis showed that the greater the lesion loads, greater the bifrontal atrophy, MCA-PI and lower MoCA scores. Up to 60.9% of patients presented a decreased MoCA score (p = 0.012). It appeared to be related to VDI (p = 0.04), WMC (p = 0.004) and altered SPECT (p = 0.05). Conclusions: The alterations in brain perfusion SPECT, the presence of white matter lesions on MRI, as well as increased PI and RI with lower MFV of the cerebral vessels in TCD suggest the presence of microangiopathy in asymptomatic AAV that could lead to cognitive impairment.

White matter hyperintensities and cognitive performance in adult patients with bipolar I, bipolar II, and major depressive disorders

2014 ◽  
Vol 29 (4) ◽  
pp. 226-232 ◽  
Author(s):  
T. Kieseppä ◽  
R. Mäntylä ◽  
A. Tuulio-Henriksson ◽  
K. Luoma ◽  
O. Mantere ◽  
...  
Keyword(s):  
White Matter ◽  
White Matter Hyperintensities ◽  
Structural Equation Models ◽  
Brain Mri ◽  
Path Model ◽  
Control Group ◽  
Coefficient Estimate ◽  
Major Depressive ◽  
Brain White Matter ◽  
Increased Risk

AbstractPurpose:We evaluate for the first time the associations of brain white matter hyperintensities (WMHs) on magnetic resonance imaging (MRI) with neuropsychological variables among middle-aged bipolar I (BPI), II (BPII) and major depressive disorder (MDD) patients and controls using a path model.Methods:Thirteen BPI, 15 BPII, 16 MDD patients, and 21 controls underwent brain MRI and a neuropsychological examination. Two experienced neuroradiologists evaluated WMHs on the MRI scans. We constructed structural equation models to test the strength of the associations between deep WMH (DWMH) grade, neuropsychological performance and diagnostic group.Results:Belonging in the BPI group as opposed to the control group predicted higher DWMH grade (coefficient estimate 1.13, P = 0.012). The DWMH grade independently predicted worse performance on the Visual Span Forward test (coefficient estimate −0.48, P = 0.002). Group effects of BPI and MDD were significant in predicting poorer performance on the Digit Symbol test (coefficient estimate −5.57, P = 0.016 and coefficient estimate −5.66, P = 0.034, respectively).Limitations:Because of the small number of study subjects in groups, the negative results must be considered with caution.Conclusions:Only BPI patients had an increased risk for DWMHs. DWMHs were independently associated with deficits in visual attention.

scholarly journals Genome-wide scan in Hispanics highlights candidate loci for brain white matter hyperintensities

2017 ◽  
Vol 3 (5) ◽  
pp. e185 ◽  
Author(s):  
Ashley Beecham ◽  
Chuanhui Dong ◽  
Clinton B. Wright ◽  
Nicole Dueker ◽  
Adam M. Brickman ◽  
...  
Keyword(s):  
White Matter ◽  
Genetic Variants ◽  
White Matter Hyperintensities ◽  
Brain Mri ◽  
Intracranial Volume ◽  
Snp Analysis ◽  
Community Based ◽  
Brain White Matter ◽  
Genome Wide ◽  
A Genome

Objective:To investigate genetic variants influencing white matter hyperintensities (WMHs) in the understudied Hispanic population.Methods:Using 6.8 million single nucleotide polymorphisms (SNPs), we conducted a genome-wide association study (GWAS) to identify SNPs associated with WMH volume (WMHV) in 922 Hispanics who underwent brain MRI as a cross-section of 2 community-based cohorts in the Northern Manhattan Study and the Washington Heights–Inwood Columbia Aging Project. Multiple linear modeling with PLINK was performed to examine the additive genetic effects on ln(WMHV) after controlling for age, sex, total intracranial volume, and principal components of ancestry. Gene-based tests of association were performed using VEGAS. Replication was performed in independent samples of Europeans, African Americans, and Asians.Results:From the SNP analysis, a total of 17 independent SNPs in 7 genes had suggestive evidence of association with WMHV in Hispanics (p < 1 × 10−5) and 5 genes from the gene-based analysis with p < 1 × 10−3. One SNP (rs9957475 in GATA6) and 1 gene (UBE2C) demonstrated evidence of association (p < 0.05) in the African American sample. Four SNPs with p < 1 × 10−5 were shown to affect binding of SPI1 using RegulomeDB.Conclusions:This GWAS of 2 community-based Hispanic cohorts revealed several novel WMH-associated genetic variants. Further replication is needed in independent Hispanic samples to validate these suggestive associations, and fine mapping is needed to pinpoint causal variants.

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