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Brain Pathology

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https://www.readbyqxmd.com/read/30020563/mri-of-cortical-lesions-and-its-use-in-studying-their-role-in-ms-pathogenesis-and-disease-course
#1
R Magliozzi, R Reynolds, M Calabrese
Cortical grey matter (GM) demyelination is present from the earliest stages of multiple sclerosis (MS) and is associated with physical deficits and cognitive impairment. In particular, the rate of disability progression in MS, both in the relapsing and progressive phases, appears to be strictly associated with degenerative GM demyelination and diffuse cortical atrophy. In the last decade, several histopathological studies and advanced radiological methodologies have contributed to better identify the exact involvement/load of cortical pathology in MS, even if the specific inflammatory features and the precise cell and molecular mechanisms of GM demyelination and neurodegeneration in MS remain still not fully understood...
July 18, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30020561/a-clinical-and-histopathological-study-of-malformations-observed-in-fetuses-infected-by-the-zika-virus
#2
A Beaufrère, B Bessières, M Bonnière, M Driessen, C Alfano, T Couderc, M Thiry, N Thelen, M Lecuit, T Attié-Bitach, M Vekemans, Y Ville, L Nguyen, M Leruez-Ville, F Encha-Razavi
BACKGROUND: The recent outbreak of Zika virus (ZIKV) infection and the associated increased prevalence of microcephaly in Brazil underline the impact of viral infections on embryo-fetal development. The aim of the present study is to provide a detailed clinical and histopathological study of the fetal disruption caused by the ZIKV, with a special focus on the associated neuropathological findings. METHODS: A detailed feto-placental examination, as well as neuropathological and neurobiological studies were performed on three fetuses collected after pregnancy termination between 22 to 25 weeks of gestation (WG), because brain malformations associated with a maternal and fetal ZIKV infection was diagnosed...
July 18, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30020560/exploring-the-heterogeneity-of-ms-lesions-using-positron-emission-tomography-a-reappraisal-of-their-contribution-to-disability
#3
Bruno Stankoff, Emilie Poirion, Matteo Tonietto, Benedetta Bodini
The biological mechanisms driving disability worsening in multiple sclerosis (MS) are only partly understood.. Monitoring changes in lesion load on MRI has a limited predictive value on the development of clinical disability, and there is an essential need for novel imaging markers specific for the main candidate mechanisms underlying neurodegeneration which include failing myelin repair, innate immune cell activation and grey matter neuronal damage. Positron Emission Tomography (PET) is an imaging technology based on the injection of radiotracers directed against specific molecular targets and has recently allowed the selective quantification in-vivo of the key biological mechanisms relevant to MS pathophysiology...
July 18, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30020556/iron-related-changes-in-ms-lesions-and-their-validity-to-characterize-ms-lesion-types-and-dynamics-with-ultra-high-field-magnetic-resonance-imaging
#4
Simon Hametner, Assunta Dal Bianco, Siegfried Trattnig, Hans Lassmann
Iron accumulates with age in the normal human brain. This process is altered at several levels in the brain of multiple sclerosis (MS) patients. Since iron is mainly stored in oligodendrocytes and myelin in the normal brain, its liberation in demyelinating lesions may amplify tissue damage in demyelinating lesions and its uptake in macrophages and microglia may help to more precisely define activity stages of the lesions. In addition, glia cells change their iron import, export and storage properties in MS lesions, which is reflected by alterations in the expression of iron transport molecules...
July 18, 2018: Brain Pathology
https://www.readbyqxmd.com/read/29757481/severe-white-matter-astrocytopathy-in-cadasil
#5
Yoshiki Hase, Aiqing Chen, Letitia L Bates, Lucinda J L Craggs, Yumi Yamamoto, Elizabeth Gemmell, Arthur E Oakley, Viktor I Korolchuk, Raj N Kalaria
Objectives Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is characterised by strategic white matter (WM) hyperintensities on MRI. Pathological features include WM degeneration, arteriolosclerosis, lacunar infarcts and the deposition of granular osmiophilic material. Based on the hypothesis that the gliovascular unit is compromised, we assessed the nature of astrocyte damage in the deep WM of CADASIL subjects. Methods We evaluated post-mortem brains from CADASIL, cerebral small vessel disease, similar age cognitively normal and older control subjects...
May 14, 2018: Brain Pathology
https://www.readbyqxmd.com/read/29722917/pathogenic-variants-in-the-abcc6-gene-are-associated-with-an-increased-risk-for-ischemic-stroke
#6
Eva Y G De Vilder, Stefanie Cardoen, Mohammad J Hosen, Olivier Le Saux, Julie De Zaeytijd, Bart P Leroy, Jacques De Reuck, Paul J Coucke, Anne De Paepe, Dimitri Hemelsoet, Olivier M Vanakker
Ischemic stroke causes a high mortality and morbidity worldwide. It results from a complex interplay of incompletely known environmental and genetic risk factors. We investigated the ABCC6 gene as a candidate risk factor for ischemic stroke because of the increased ischemic stroke incidence in the autosomal recessive disorder pseudoxanthoma elasticum, caused by biallelic pathogenic ABCC6 variants, the higher cardiovascular risk in heterozygous carriers and the established role of ABCC6 dysfunction in myocardial ischemia...
May 3, 2018: Brain Pathology
https://www.readbyqxmd.com/read/29740949/68-year-old-woman-with-multiple-sclerosis-cutaneous-t-cell-lymphoma-and-seizures
#7
Alireza Shams, Katherine Schwetye
No abstract text is available yet for this article.
May 2018: Brain Pathology
https://www.readbyqxmd.com/read/29740948/astrocytes-an-active-player-in-aicardi-gouti%C3%A3-res-syndrome
#8
Sunetra Sase, Asako Takanohashi, Adeline Vanderver, Akshata Almad
Aicardi-Goutières syndrome (AGS) is an early-onset, autoimmune and genetically heterogeneous disorder with severe neurologic injury. Molecular studies have established that autosomal recessive mutations in one of the following genes are causative: TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, ADAR1 and IFIH1/MDA5. The phenotypic presentation and pathophysiology of AGS is associated with over-production of the cytokine Interferon-alpha (IFN-α) and its downstream signaling, characterized as type I interferonopathy...
May 2018: Brain Pathology
https://www.readbyqxmd.com/read/29740947/a-25-year-old-male-with-a-subgaleal-mass
#9
Melanie H Hakar, Benjamin P Liu, Craig M Horbinski
No abstract text is available yet for this article.
May 2018: Brain Pathology
https://www.readbyqxmd.com/read/29740946/a-37-year-old-woman-with-progressive-right-side-ptosis-for-one-month
#10
Hui-Yuan Su, Yu-Feng Su
No abstract text is available yet for this article.
May 2018: Brain Pathology
https://www.readbyqxmd.com/read/29740945/alexander-disease-an-astrocytopathy-that-produces-a-leukodystrophy
#11
Alexander Sosunov, Markel Olabarria, James E Goldman
Alexander Disease (AxD) is a degenerative disorder caused by mutations in the GFAP gene, which encodes the major intermediate filament of astrocytes. As other cells in the CNS do not express GFAP, AxD is a primary astrocyte disease. Astrocytes acquire a large number of pathological features, including changes in morphology, the loss or diminution of a number of critical astrocyte functions and the activation of cell stress and inflammatory pathways. AxD is also characterized by white matter degeneration, a pathology that has led it to be included in the "leukodystrophies...
May 2018: Brain Pathology
https://www.readbyqxmd.com/read/29740944/corrigendum
#12
(no author information available yet)
No abstract text is available yet for this article.
May 2018: Brain Pathology
https://www.readbyqxmd.com/read/29740943/vanishing-white-matter-a-leukodystrophy-due-to-astrocytic-dysfunction
#13
Marianna Bugiani, Caroline Vuong, Marjolein Breur, Marjo S van der Knaap
VWM is one of the most prevalent leukodystrophies with unique clinical, pathological and molecular features. It mostly affects children, but may develop at all ages, from birth to senescence. It is dominated by cerebellar ataxia and susceptible to stresses that act as factors provoking disease onset or episodes of rapid neurological deterioration possibly leading to death. VWM is caused by mutations in any of the genes encoding the five subunits of the eukaryotic translation initiation factor 2B (eIF2B). Although eIF2B is ubiquitously expressed, VWM primarily manifests as a leukodystrophy with increasing white matter rarefaction and cystic degeneration, meager astrogliosis with no glial scarring and dysmorphic immature astrocytes and increased numbers of oligodendrocyte progenitor cells that are restrained from maturing into myelin-forming cells...
May 2018: Brain Pathology
https://www.readbyqxmd.com/read/29740942/genetic-defects-disrupting-glial-ion-and-water-homeostasis-in-the-brain
#14
Rogier Min, Marjo S van der Knaap
Electrical activity of neurons in the brain, caused by the movement of ions between intracellular and extracellular compartments, is the basis of all our thoughts and actions. Maintaining the correct ionic concentration gradients is therefore crucial for brain functioning. Ion fluxes are accompanied by the displacement of osmotically obliged water. Since even minor brain swelling leads to severe brain damage and even death, brain ion and water movement has to be tightly regulated. Glial cells, in particular astrocytes, play a key role in ion and water homeostasis...
May 2018: Brain Pathology
https://www.readbyqxmd.com/read/29740941/deposition-of-phosphorylated-amyloid-%C3%AE-in-brains-of-aged-nonhuman-primates-and-canines
#15
LETTER
Sathish Kumar, Jeffrey L Frost, Carl W Cotman, Elizabeth Head, Roberta Palmour, Cynthia A Lemere, Jochen Walter
No abstract text is available yet for this article.
May 2018: Brain Pathology
https://www.readbyqxmd.com/read/29740940/leukodystrophies-due-to-astroyctic-dysfunction
#16
Marianna Bugiani, Marjolein Breur
Leukodystrophies are genetically determined disorders due to defects in any structural components of the brain white matter. This mini-symposium presents a selection of leukodystrophies due to astrocytic dysfunction, the astrocytopathies. Examples are provided of astrocytopathies due to defects in astrocyte-specific proteins and in which astrocytes play a major role in the pathophysiology. Knowledge on the disease mechanisms underlying these leukodystrophies also provides information how loss of physiologic functions and gain of detrimental functions in astrocytes leads to degeneration of the white matter...
May 2018: Brain Pathology
https://www.readbyqxmd.com/read/29740938/four-repeat-tau-dominant-pathology-in-a-congenital-myotonic-dystrophy-type-1-patient-with-mental-retardation
#17
LETTER
Yuri Mizuno, Norihisa Maeda, Hideomi Hamasaki, Hajime Arahata, Naokazu Sasagasako, Hiroyuki Honda, Naoki Fujii, Toru Iwaki
No abstract text is available yet for this article.
May 2018: Brain Pathology
https://www.readbyqxmd.com/read/29740937/a-74-year-old-female-with-a-well-circumscribed-parietal-lobe-mass
#18
Alexandros Iliadis, Elsa Pazarli, Charalampos-Chrysovalantis Chytoudis-Peroudis, Stella Chondromatidou, Ioannis Tsitouridis, Ioannis Efstratiou, Dimitrios Kanakis
No abstract text is available yet for this article.
May 2018: Brain Pathology
https://www.readbyqxmd.com/read/29740936/corrigendum
#19
(no author information available yet)
No abstract text is available yet for this article.
May 2018: Brain Pathology
https://www.readbyqxmd.com/read/28960710/optineurin-pathology-in-the-spinal-cord-of-amyotrophic-lateral-sclerosis-parkinsonism-dementia-complex-patients-in-kii-peninsula-japan
#20
LETTER
Satoru Morimoto, Hiroyuki Hatsuta, Rie Motoyama, Yasumasa Kokubo, Hiroyuki Ishiura, Shoji Tsuji, Shigeki Kuzuhara, Shigeo Murayama
No abstract text is available yet for this article.
May 2018: Brain Pathology
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