5,177 results match your criteria Journal of neuropathology and experimental neurology[Journal]


Expression of GLUT1 in Pseudopalisaded and Perivascular Tumor Cells Is an Independent Prognostic Factor for Patients With Glioblastomas.

J Neuropathol Exp Neurol 2019 May;78(5):389-397

Neurosurgery, Kurume University School of Medicine, Kurume, Fukuoka, Japan.

Glioblastomas are highly aggressive brain tumors with a particularly poor prognosis. Glucose transporter-1 (GLUT1/SLC2A1), a uniporter that is expressed by various carcinomas and may be involved in malignant neoplasm glycometabolism, may also be related to prognosis in glioblastomas. GLUT1 is essential to central nervous system glycometabolism. Read More

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http://dx.doi.org/10.1093/jnen/nly124DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6467190PMC
May 2019
1 Read

Peri-Infarct Upregulation of the Oxytocin Receptor in Vascular Dementia.

J Neuropathol Exp Neurol 2019 May;78(5):436-452

Department of Translational Science and Molecular Medicine, Michigan State University, Grand Rapids, Michigan.

Vascular dementia (VaD) is cognitive decline linked to reduced cerebral blood perfusion, yet there are few therapeutic options to protect cognitive function following cerebrovascular accidents. The purpose of this study was to profile gene expression changes unique to VaD to identify and characterize disease relevant changes that could offer clues for future therapeutic direction. Microarray-based profiling and validation studies of postmortem frontal cortex samples from VaD, Alzheimer disease, and age-matched control subjects revealed that the oxytocin receptor (OXTR) was strongly and differentially upregulated in VaD. Read More

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http://dx.doi.org/10.1093/jnen/nlz023DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6467199PMC
May 2019
1 Read

Differential Expression of Wilms' Tumor Protein in Diffuse Intrinsic Pontine Glioma.

J Neuropathol Exp Neurol 2019 May;78(5):380-388

Children's National Health System, Center for Genetic Medicine Research, Washington, District of Columbia.

Diffuse intrinsic pontine gliomas (DIPGs) are deadly tumors comprising 10%-15% of all childhood CNS cancers. Standard treatment is considered palliative and prognosis is near universal mortality. DIPGs have been classified into genomic subtypes based on histone variants with the lysine to methionine mutation on position 27 of histone tails (K27M). Read More

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http://dx.doi.org/10.1093/jnen/nlz021DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6467196PMC
May 2019
2 Reads
3.797 Impact Factor

X-Linked Lymphoproliferative Syndrome Presenting as Adult-Onset Multi-Infarct Dementia.

J Neuropathol Exp Neurol 2019 May;78(5):460-466

Department of Neurology, Mayo Clinic, Jacksonville, Florida.

Pathogenic hemizygous variants in the SH2D1A gene cause X-linked lymphoproliferative (XLP) syndrome, a rare primary immunodeficiency usually associated with fatal Epstein-Barr virus infection. Disease onset is typically in early childhood, and the average life expectancy of affected males is ∼11 years. We describe clinical, radiographic, neuropathologic, and genetic features of a 49-year-old man presenting with central nervous system vasculitis that was reminiscent of adult primary angiitis but which was unresponsive to treatment. Read More

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https://academic.oup.com/jnen/article/78/5/460/5445919
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http://dx.doi.org/10.1093/jnen/nlz018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6467195PMC
May 2019
3 Reads

Chronic Traumatic Encephalopathy (CTE) Is Absent From a European Community-Based Aging Cohort While Cortical Aging-Related Tau Astrogliopathy (ARTAG) Is Highly Prevalent.

J Neuropathol Exp Neurol 2019 May;78(5):398-405

Institute of Neurology, Medical University of Vienna, Vienna, Austria.

This study determined the prevalence of chronic traumatic encephalopathy (CTE) and cortical aging-related tau astrogliopathy (ARTAG) in a European community-based population (n = 310). The frontal, parietal, and temporal cortices, representing initial stages of CTE were assessed. No case fulfilling CTE consensus criteria was found. Read More

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https://academic.oup.com/jnen/advance-article/doi/10.1093/jn
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http://dx.doi.org/10.1093/jnen/nlz017DOI Listing
May 2019
8 Reads

A Highly Sensitive Sandwich ELISA to Detect CSF Progranulin: A Potential Biomarker for CNS Disorders.

J Neuropathol Exp Neurol 2019 May;78(5):406-415

Department of Pathology, Northwestern University Feinberg School of Medicine, Chicago, Illinois.

Progranulin (PGRN) plays critical roles in inflammation, tumorigenesis, and neurodegeneration. PGRN levels in blood and cerebrospinal fluid (CSF) are being increasingly investigated as potential biomarkers for these disorders. However, the value of CSF PGRN as a biomarker has been limited because currently available commercial enzyme-linked immunosorbent assay (ELISA) kits have suboptimal sensitivity for detecting CSF PGRN. Read More

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http://dx.doi.org/10.1093/jnen/nlz022DOI Listing
May 2019
2 Reads

Altered Dynein Axonemal Assembly Factor 1 Expression in C-Boutons in Bulbar and Spinal Cord Motor-Neurons in Sporadic Amyotrophic Lateral Sclerosis.

J Neuropathol Exp Neurol 2019 May;78(5):416-425

Department of Pathology and Experimental Therapeutics, University of Barcelona, Spain.

Dyneins are major components of microtubules. Dynein assembly is modulated by a heterogeneous group of dynein axonemal assembly factors (DNAAFs). The present study analyzes dynein axonemal assembly factor 1 (DNAAF1) and leucine-rich repeat-containing protein 50 (LRRC50), the corresponding encoded protein, in lower motor neurons in spinal cord of sALS postmortem samples and hSOD1-G93A transgenic mice compared with controls. Read More

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https://academic.oup.com/jnen/advance-article/doi/10.1093/jn
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http://dx.doi.org/10.1093/jnen/nlz019DOI Listing
May 2019
6 Reads

Poly-Arginine Peptides R18 and R18D Improve Functional Outcomes After Endothelin-1-Induced Stroke in the Sprague Dawley Rat.

J Neuropathol Exp Neurol 2019 May;78(5):426-435

Perron Institute for Neurological and Translational Science, Nedlands, Western Australia, Australia.

We have previously demonstrated that R18 and its d-enantiomer, R18D, are neuroprotective at 24 hours following intraluminal filament occlusion of the middle cerebral artery (MCAO) in the rat. This study examined R18 and R18D effectiveness in improving functional outcomes at up to 56 days poststroke following endothelin-1-induced MCAO. Peptides were administered intravenously at doses of 100, 300, or 1000 nmol/kg, 60 minutes after MCAO. Read More

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http://dx.doi.org/10.1093/jnen/nlz014DOI Listing
May 2019
1 Read

Axotomy Induces Phasic Alterations in Luman/CREB3 Expression and Nuclear Localization in Injured and Contralateral Uninjured Sensory Neurons: Correlation With Intrinsic Axon Growth Capacity.

J Neuropathol Exp Neurol 2019 Apr;78(4):348-364

Department of Anatomy, Physiology and Pharmacology.

Luman/CREB3 is an important early retrograde axotomy signal regulating acute axon outgrowth in sensory neurons through the adaptive unfolded protein response. As the injury response is transcriptionally multiphasic, a spatiotemporal analysis of Luman/CREB3 localization in rat dorsal root ganglion (DRG) with unilateral L4-L6 spinal nerve injury was conducted to determine if Luman/CREB3 expression was similarly regulated. Biphasic alterations in Luman/CREB3 immunofluorescence and nuclear localization occurred in neurons ipsilateral to 1-hour, 1-day, 2-day, 4-day, and 1-week injury, with a largely parallel, but less avid response contralaterally. Read More

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http://dx.doi.org/10.1093/jnen/nlz008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6426154PMC
April 2019
1 Read

Transforming Growth Factor-β Promotes Homing and Therapeutic Efficacy of Human Mesenchymal Stem Cells to Glioblastoma.

J Neuropathol Exp Neurol 2019 Apr;78(4):315-325

Department of Neurosurgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science & Technology, Wuhan, Hubei, People's Republic of China.

Human mesenchymal stem cell-based tumor therapeutic gene delivery is regarded as a promising strategy for the treatment of glioblastoma (GBM). However, the efficiency of these stem cells to home to the target sites limits their potential curative effect and clinical application. In this work, we provide a novel pretreatment approach for enhancing the homing capacity of human adipose-derived mesenchymal stem cells (hAMSCs) for stem cell-based tumor gene delivery for GBM therapy. Read More

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http://dx.doi.org/10.1093/jnen/nlz016DOI Listing

Refined Quantitation of Sweat Gland Innervation.

J Neuropathol Exp Neurol 2019 May;78(5):453-459

Department of Neurology, Mayo Clinic, Rochester, Minnesota (KM, AMS, JAG, JNM, PAL, WS).

Skin biopsies have gained increasing popularity as a tool to evaluate disorders affecting small nerve fibers. While reports on sweat gland nerve fiber density (SGNFD) to quantitate sudomotor innervation have been promising, methodologies vary significantly. Although conventional stereology is commonly used, no standard technique has been established. Read More

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http://dx.doi.org/10.1093/jnen/nlz015DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6467193PMC

Sporadic Creutzfeldt-Jakob Disease in a Young Girl With Unusually Long Survival.

J Neuropathol Exp Neurol 2019 Apr;78(4):373-378

Department of Pathology and Laboratory Medicine, University of Ottawa, Ottawa, Canada.

Creutzfeldt-Jakob disease (CJD) is a rapidly progressive, fatal degenerative encephalopathy caused by a pathologically altered form of the prion protein (PrP). CJD is rare, with 1 and 2 cases per million per year reported in the general population, mostly in individuals over 50 years of age. It is almost unknown in the pediatric population. Read More

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http://dx.doi.org/10.1093/jnen/nlz013DOI Listing
April 2019
2 Reads

Complex Patterns of GABAergic Neuronal Deficiency and Type 2 Potassium-Chloride Cotransporter Immaturity in Human Focal Cortical Dysplasia.

J Neuropathol Exp Neurol 2019 Apr;78(4):365-372

Department of Pathology and Laboratory Medicine.

Focal cortical dysplasia (FCD) is a common histopathologic finding in cortical specimens resected for refractory epilepsy. GABAergic neuronal abnormalities and K-Cl cotransporter type 2 (KCC2) immaturity may be contributing factors for FCD-related epilepsy. We examined surgical specimens from 12 cases diagnosed with FCD, and brain tissues without developmental abnormality obtained from 6 autopsy cases. Read More

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http://dx.doi.org/10.1093/jnen/nlz009DOI Listing

Clinical and Molecular Characterization of Adult Glioblastomas in Southern Brazil.

J Neuropathol Exp Neurol 2019 Apr;78(4):297-304

Graduate Program in Pathology.

We investigated 113 adult Brazilian patients with glioblastoma (GBM) for comparison with patients from distinct geographical areas and evaluation of suitability for novel targeted therapies. Patients were assessed for clinical features and tumor genomic characteristics such as ROS1 and NTRK1 rearrangements, KIT, PDGFRA, and KDR amplification, and RB1 deletion using multicolor fluorescence in situ hybridization. The majority of patients were male (53%), over 40 years (94%), with tumor located in single site (64%), in the right cerebral hemisphere (60%), and underwent partial resection (71%); 14% presented complications after surgery. Read More

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http://dx.doi.org/10.1093/jnen/nlz006DOI Listing
April 2019
5 Reads
3.797 Impact Factor

What Every Neuropathologist Needs to Know: Update on cIMPACT-NOW.

J Neuropathol Exp Neurol 2019 Apr;78(4):294-296

Department of Pathology, Northwestern Feinberg School of Medicine, Chicago, Illinois.

World Health Organization (WHO) central nervous system tumor classification represents the primary source of updates on diagnostic classes, grades, and criteria. However, recent and ongoing advances in molecular pathogenesis warrant more rapid integration into clinical practice between WHO updates. To accomplish this, the consortium to inform molecular and practical approaches to CNS tumor taxonomy-not official WHO (cIMPACT-NOW) was established in 2016. Read More

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http://dx.doi.org/10.1093/jnen/nlz012DOI Listing
April 2019
1 Read

Origin and Distribution of Connective Tissue and Pericytes Impacting Vascularization in Brain Metastases With Different Growth Patterns.

J Neuropathol Exp Neurol 2019 Apr;78(4):326-339

1st Department of Pathology and Experimental Cancer Research.

The impact of growth pattern on the distribution of connective tissue and on the vascularization of brain metastases (40 colon, lung and breast carcinoma samples) was analyzed. Most of the cases showed either a "pushing-type" (18/40, mostly colon and lung carcinomas) or a "papillary-type" (19/40, mostly breast carcinomas) growth pattern. There was a striking difference in the growth pattern and vascularization of colon/lung versus breast carcinoma metastases. Read More

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http://dx.doi.org/10.1093/jnen/nlz007DOI Listing
April 2019
5 Reads

Recurrent EP300-BCOR Fusions in Pediatric Gliomas With Distinct Clinicopathologic Features.

J Neuropathol Exp Neurol 2019 Apr;78(4):305-314

Department of Pathology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts.

BCOR is an epigenetic regulator and is genetically altered by mutation, deletion, or gene fusion in a range of cancers. "Central nervous system high-grade neuroepithelial tumor with BCOR alteration" is a recently described entity with characteristic internal tandem duplications within exon 15 of the BCOR gene (hereafter: CNS HGNET-BCOR ex15 ITD). In this case series of 3 patients, we report the clinicopathologic, molecular, and methylome features of gliomas with novel EP300-BCOR in-frame gene fusions, thus expanding the spectrum of BCOR alterations seen in CNS tumors. Read More

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https://academic.oup.com/jnen/advance-article/doi/10.1093/jn
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http://dx.doi.org/10.1093/jnen/nlz011DOI Listing
April 2019
5 Reads
3.797 Impact Factor

Multifocal Necrotizing Leukoencephalopathy: Expanding the Clinicopathologic Spectrum.

J Neuropathol Exp Neurol 2019 Apr;78(4):340-347

Department of Pathology, Medical College of Georgia at Augusta University, Augusta, Georgia.

Multifocal necrotizing leukoencephalopathy (MNL) is a rare condition typically described in patients undergoing chemotherapy or with HIV/AIDS. As a pathologic entity, it is characterized by multiple small foci of necrosis typically within white matter of the pons and occasionally in other areas. Herein we describe findings in 6 patients with MNL, 5 diagnosed at postmortem examination and 1 by surgical biopsy. Read More

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http://dx.doi.org/10.1093/jnen/nlz003DOI Listing
April 2019
1 Read

Morphology and Distribution of TDP-43 Pre-inclusions in Primary Progressive Aphasia.

J Neuropathol Exp Neurol 2019 Feb 8. Epub 2019 Feb 8.

Feinberg School of Medicine, Mesulam Center for Cognitive Neurology and Alzheimer's Disease, Northwestern University, Chicago, Illinois.

Diffusely stained phosphorylated 43-kDa TAR DNA-binding protein (TDP-43)-positive "pre-inclusions" have been described. This experiment investigated morphological subtypes of pre-inclusions and their relationship with TDP-43 inclusions in primary progressive aphasia (PPA), a dementia characterized by gradual dissolution of language. Brain sections from 5 PPA participants with postmortem diagnoses of frontotemporal lobar degeneration with TDP-43 pathology (FTLD-TDP) were immunohistochemically stained using an antibody to phosphorylated TDP-43 and quantitatively examined for regional and hemispheric distribution using unbiased stereology. Read More

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http://dx.doi.org/10.1093/jnen/nlz005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6380320PMC
February 2019
2 Reads

27-Hydroxycholesterol Alters Synaptic Structural and Functional Plasticity in Hippocampal Neuronal Cultures.

J Neuropathol Exp Neurol 2019 Feb 11. Epub 2019 Feb 11.

School of Public Health, Capital Medical University, Beijing, China.

This study aimed to explore the neurotoxic effects of 27-hydroxycholesterol (27-OHC), a major circulating cholesterol active derivative in brain on synaptic structural and functional plasticity in primary hippocampal neurons. Newborn SD rat primary hippocampal neurons were treated with 0, 1, 3, 10, and 30 μM 27-OHC for 24 hours. MTT and CCK-8 assays were used to monitor the cell viability of neurons with different treatments. Read More

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http://dx.doi.org/10.1093/jnen/nlz002DOI Listing
February 2019
6 Reads

What Every Neuropathologist Needs to Know: Neuropathology and the US Legal System.

J Neuropathol Exp Neurol 2019 Apr;78(4):291-293

Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota.

The legal system of the United States is complex, with nuances that are particular to its many jurisdictions. The neuropathologist may professionally interact with the legal system in both criminal and civil proceedings as either a fact or expert witness, and in rare instances as a defendant. The nature of the legal issue at hand will define the pathologist's role and determine what actions are required or requested. Read More

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http://dx.doi.org/10.1093/jnen/nly131DOI Listing

A Review of Neuropathological Features of Familial and Adult Hemophagocytic Lymphohistiocytosis.

J Neuropathol Exp Neurol 2019 Jan 14. Epub 2019 Jan 14.

Department of Pathology, University of Colorado School of Medicine, Aurora, Colorado.

Hemophagocytic lymphohistiocytosis (HLH) is a hematological disorder that can be due to genetic (primary HLH) causes or excessive activation of the immune system in association with infection, malignancy, rheumatologic disorders, or immune suppression (secondary HLH). Hemophagocytic lymphohistiocytosis remains an under-recognized condition among neuropathologists, especially the secondary forms, where it may be diagnosed only at brain biopsy or autopsy due to confounding comorbidities. The CNS is frequently affected, but neuropathological features are underappreciated. Read More

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https://academic.oup.com/jnen/advance-article/doi/10.1093/jn
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http://dx.doi.org/10.1093/jnen/nlz001DOI Listing
January 2019
8 Reads

Severe Neonatal RYR1 Myopathy With Pathological Features of Congenital Muscular Dystrophy.

J Neuropathol Exp Neurol 2019 Feb 4. Epub 2019 Feb 4.

Department of Pathology and Laboratory Medicine, Medical College of Wisconsin, Milwaukee, Wisconsin.

The phenotypes associated with pathogenic variants in the ryanodine receptor 1 gene (RYR1, OMIM# 180901) have greatly expanded over the last few decades as genetic testing for RYR1 variants has become more common. Initially described in association with malignant hyperthermia, pathogenic variants in RYR1 are typically associated with core pathology in muscle biopsies (central core disease or multiminicore disease) and symptomatic myopathies with symptoms ranging from mild weakness to perinatal lethality. We describe a 2-week-old male patient with multiple congenital dysmorphisms, severe perinatal weakness, and subsequent demise, whose histopathology on autopsy was consistent with congenital muscular dystrophy. Read More

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http://dx.doi.org/10.1093/jnen/nlz004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6380315PMC
February 2019
1 Read

Downregulation of LncRNA TUG1 Inhibited TLR4 Signaling Pathway-Mediated Inflammatory Damage After Spinal Cord Ischemia Reperfusion in Rats via Suppressing TRIL Expression.

J Neuropathol Exp Neurol 2019 Feb 1. Epub 2019 Feb 1.

Department of Anesthesiology, First Hospital of China Medical University, Shenyang, Liaoning, China.

Toll-like receptor 4 (TLR4) and TLR4 interactor with leucine-rich repeats (TRIL) play a crucial role in the inflammatory response. This study investigated the role of long noncoding RNA taurine-upregulated gene 1 (lncRNA TUG1) in TRIL/TLR4 signaling in spinal cord ischemia reperfusion (IR) injury. IR injury was induced in experimental rats; knockdown of TUG1 and TRIL was induced by intrathecal injection of siRNAs and overexpression of TRIL was induced by pcDNA3. Read More

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http://dx.doi.org/10.1093/jnen/nly126DOI Listing
February 2019

Differences in Cognitive Impairment in Primary Age-Related Tauopathy Versus Alzheimer Disease.

J Neuropathol Exp Neurol 2019 Feb 1. Epub 2019 Feb 1.

Department of Pathology, Fishberg Department of Neuroscience, Friedman Brain Institute, Ronald M. Loeb Center for Alzheimer's Disease, Icahn School of Medicine at Mount Sinai, New York, New York.

This study examined differences in neuropsychological test scores between individuals with primary age-related tauopathy (PART) and Alzheimer disease (AD) using cross-sectional data from the National Alzheimer's Coordinating Center. Linear regression tested for differences in 4 cognitive domains stratified by cognitive status (global Clinical Dementia Rating [CDR]). The sample included 240 participants with no neuritic plaques (NP) (definite PART), 186 with sparse NP (possible PART), and 510 with moderate/frequent NP (AD). Read More

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http://dx.doi.org/10.1093/jnen/nly132DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6380319PMC
February 2019
10 Reads

Integrating a Large Next-Generation Sequencing Panel into the Clinical Diagnosis of Gliomas Provides a Comprehensive Platform for Classification from FFPE Tissue or Smear Preparations.

J Neuropathol Exp Neurol 2019 Jan 28. Epub 2019 Jan 28.

Department of Pathology, The University of Chicago, Chicago, Illinois.

The 2016 WHO classification of brain tumors represents a major step towards the integration of molecular data into pathologic diagnoses. Our institution has included massively parallel sequencing technology in the diagnostic work-up of all gliomas since January 2016. The utilized platform successfully identifies copy number variations, individual gene mutations, small insertions and deletions, and selected gene fusions. Read More

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http://dx.doi.org/10.1093/jnen/nly130DOI Listing
January 2019

Prognostic Characterization of Higher-Grade Meningiomas: A Histopathological Score to Predict Progression and Outcome.

J Neuropathol Exp Neurol 2019 Jan 25. Epub 2019 Jan 25.

Pathology Unit, Department of Medical Sciences, University of Turin, Torino, Italy.

Higher-grade meningiomas (WHO grade II and III) represent a diagnostic and prognostic challenge. We assessed the pathological and molecular characteristics of 94 higher-grade meningiomas (85 grade II, 9 grade III) to identify novel prognostic parameters. Higher mitotic count (p = 0. Read More

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http://dx.doi.org/10.1093/jnen/nly127DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6380327PMC
January 2019
2 Reads

Increased ISGylation in Cases of TBI-Exposed ALS Veterans.

J Neuropathol Exp Neurol 2018 Dec 28. Epub 2018 Dec 28.

Department of Biochemistry and Molecular Biology, LSUHSC-School of Medicine, New Orleans, Louisiana.

Veterans who have served in the military are at a nearly 60% greater risk of being diagnosed with amyotrophic lateral sclerosis (ALS). Literature reports suggest that a history of traumatic brain injury (TBI) may be a risk factor for ALS in veterans. However, no diagnostic biomarkers are available for identifying ALS risk/development in TBI-exposed veterans. Read More

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https://academic.oup.com/jnen/advance-article/doi/10.1093/jn
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http://dx.doi.org/10.1093/jnen/nly129DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6380302PMC
December 2018
18 Reads

Age-Related Changes of the Neurovascular Unit in the Cerebral Cortex of Alzheimer Disease Mouse Models: A Neuroanatomical and Molecular Study.

J Neuropathol Exp Neurol 2019 Feb;78(2):101-112

Department of Veterinary Medical Sciences (DIMEVET), University of Bologna, Bologna, Italy.

We describe age-related histological structure and molecular changes of the neurovascular unit (NVU) in the cerebral cortex of Tg2576 and age-matched wild-type (WT) mice. Major results can be summarized as follows: (i) β-amyloid (6E10)-immunoreactivity progressively increases in neurons and astrocytes of Tg2576 mice, reaching the highest concentration at 5 months and then decreasing as soon as extracellular plaque deposition begins; (ii) the synaptic puncta density of glutamatergic and GABAergic neurons in Tg2576 mice is unbalanced versus WT at all investigated ages, with a decrease in synaptophysin and VGLUT1; density of VGAT contacts is higher in 27-month-old Tg2576 versus WT mice; (iii) capillary density is higher in 5-month-old Tg2576 versus WT mice, then decreases to a lower density at 27 months, when the capillary-astrocyte interface is lower; and (iv) mRNA expression of genes involved in microvessel dynamics indicates age- and genotype-dependent changes in the expression levels of hypoxia-related genes, i.e. Read More

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https://academic.oup.com/jnen/advance-article/doi/10.1093/jn
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http://dx.doi.org/10.1093/jnen/nly125DOI Listing
February 2019
20 Reads

TAZ Expression on Endothelial Cells Is Closely Related to Blood Vascular Density and VEGFR2 Expression in Astrocytomas.

J Neuropathol Exp Neurol 2019 Feb;78(2):172-180

Department of Pathology, Basic Medical Sciences, Fudan University.

Significant angiogenesis is one of the malignant features in astrocytomas. Cotransfactor Yes-associated protein/transcriptional coactivator with PDZ-binding motif (YAP/TAZ) is a major regulator of embryonic angiogenesis, in which it plays an essential role in vascular tip cell migration, blood vessel formation, and vascular barrier maturation. We quantified TAZ expression on blood vessels and parenchyma of astrocytomas of varying malignancy to investigate its role in tumor angiogenesis. Read More

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http://dx.doi.org/10.1093/jnen/nly122DOI Listing
February 2019
4 Reads

Selective Vulnerability of Basal Ganglia: Insights into the Mechanisms of Bilateral Striatal Necrosis.

J Neuropathol Exp Neurol 2019 Feb;78(2):123-129

Division of Human Anatomy - Neuronal Networks Morphology Lab, Department of Mental, Physical Health and Preventive Medicine, University of Campania "Luigi Vanvitelli".

Selective neuronal death in neurodegenerative disorders represents the final step of a cascade of events, including neuroinflammation, regional-specific reactive gliosis, changes of brain-blood barrier structure and functions, metabolic failure and mitochondrial energy impairment. Bilateral striatal necrosis is usually reported in inherited mitochondrial disorders, suggesting a pathogenetic role of the energy impairment by mitochondrial dysfunction. We investigated mechanisms of the selective striatal degeneration, comparing clinical findings of a patient with an acquired bilateral striatal necrosis and experimental data of a selective basal ganglia degenerative model in rats. Read More

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http://dx.doi.org/10.1093/jnen/nly123DOI Listing
February 2019
3 Reads

Multiple Sclerosis: Destruction and Regeneration of Astrocytes in Acute Lesions.

J Neuropathol Exp Neurol 2019 Feb;78(2):140-156

Department of Medicine, The University of Sydney, Camperdown, NSW, Australia.

There are reports that astrocyte perivascular endfeet are damaged in some cases of multiple sclerosis (MS). This study was designed to determine the origin and outcome of astrocyte damage in acute, resolving, and inactive plaques. Ten acute plaques from 10 early MS cases and 14 plaques of differing histological age from 9 subacute and chronic cases were examined immunohistochemically. Read More

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http://dx.doi.org/10.1093/jnen/nly121DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6330170PMC
February 2019
1 Read

Myostatin Inhibition Using ActRIIB-mFc Does Not Produce Weight Gain or Strength in the Nebulin Conditional KO Mouse.

J Neuropathol Exp Neurol 2019 Feb;78(2):130-139

Division of Pediatric Pathology, Department of Pathology and Laboratory Medicine.

Mutations in at least 12 genes are responsible for a group of congenital skeletal muscle diseases known as nemaline myopathies (NMs). NMs are associated with a range of clinical symptoms and pathological changes often including the presence of cytoplasmic rod-like structures (nemaline bodies) and myofiber hypotrophy. Our recent work has identified a variable degree of behavioral benefit when treating 2 NM mouse models due to mutations in Acta1 with myostatin inhibition. Read More

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http://dx.doi.org/10.1093/jnen/nly120DOI Listing
February 2019
2 Reads

Neuroprotective Effects of Gabapentin Against Cerebral Ischemia Reperfusion-Induced Neuronal Autophagic Injury via Regulation of the PI3K/Akt/mTOR Signaling Pathways.

J Neuropathol Exp Neurol 2019 Feb;78(2):157-171

Jiangsu Key Laboratory of Integrated Traditional Chinese and Western Medicine for Prevention and Treatment of Senile Diseases, Medical college of Yangzhou University.

Gabapentin (GBP), an analgesic, adjunct antiepileptic, and migraine prophylactic drug, reduces neuronal injury induced by cerebral ischemia reperfusion (IR). However, the underlying biological molecular mechanism of GBP neuroprotection is not clear. In this study, we confirmed that dose-dependent (75 and 150 mg/kg) GBP treatment could significantly reduce IR-induced neuronal death. Read More

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http://dx.doi.org/10.1093/jnen/nly119DOI Listing
February 2019
6 Reads

The cGMP-Degrading Enzyme Phosphodiesterase-5 (PDE5) in Cerebral Small Arteries of Older People.

J Neuropathol Exp Neurol 2019 Feb;78(2):191-194

Molecular and Clinical Sciences Research Institute, St. Georges University of London, London, United Kingdom.

Cerebral small vessel disease in deep penetrating arteries is a major cause of lacunar infarcts, white matter lesions and vascular cognitive impairment. Local cerebral blood flow in these small vessels is controlled by endothelial-derived nitric oxide, which exerts a primary vasodilator stimulus on vascular myocytes, via cytoplasmic cyclic GMP. Here, we investigated whether the cGMP-degrading enzyme phosphodiesterase-5 (PDE5) is present in small penetrating arteries in the deep subcortical white matter of older people. Read More

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https://academic.oup.com/jnen/advance-article/doi/10.1093/jn
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http://dx.doi.org/10.1093/jnen/nly117DOI Listing
February 2019
10 Reads

A Quantitative Study of Empty Baskets in Essential Tremor and Other Motor Neurodegenerative Diseases.

J Neuropathol Exp Neurol 2019 Feb;78(2):113-122

Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, New Haven, Connecticut; New York, New York; Albany, New York.

The underlying biology of essential tremor (ET) is poorly understood. Purkinje cell (PC) loss has been observed in some studies, although this finding remains somewhat controversial. Basket cells are interneurons whose axonal collaterals form a plexus around PC soma. Read More

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https://academic.oup.com/jnen/article/78/2/113/5261440
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http://dx.doi.org/10.1093/jnen/nly114DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6330169PMC
February 2019
3 Reads

Intraventricular Pilocytic Astrocytoma With KIAA1549/BRAF Fusion Arising in a 44-Year Old.

J Neuropathol Exp Neurol 2019 Feb;78(2):187-190

Department of Pathology, Duke University, Durham, North Carolina.

Rare pilocytic astrocytomas (PA) have been described to arise in the ventricles of children. They are even less common in this location for the adult population. We present the case of a 44-year old man presenting with vision and mental status changes. Read More

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http://dx.doi.org/10.1093/jnen/nly116DOI Listing
February 2019
3 Reads

Retinal Vasculopathy With Cerebral Leukodystrophy: Clinicopathologic Features of an Autopsied Patient With a Heterozygous TREX 1 Mutation.

J Neuropathol Exp Neurol 2019 Feb;78(2):181-186

Departments of Pathology.

Retinal vasculopathy with cerebral leukodystrophy (RVCL) is an autosomal-dominant disorder involving the cerebral, retinal, renal, and other systemic microvessels due to frameshift mutations in the TREX1 gene. Under physiological conditions, the TREX1 protein is localized in the cellular cytoplasm and perinuclear area, but translocates into the nucleus in response to oxidative DNA damage. It has been speculated that aberrant localization of the protein may be associated with systemic microangiopathy in patients with RVCL. Read More

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http://dx.doi.org/10.1093/jnen/nly115DOI Listing
February 2019
4 Reads

Spinal Pleomorphic Xanthoastrocytoma With a QKI-RAF1 Fusion.

J Neuropathol Exp Neurol 2019 Jan;78(1):10-14

Department of Pathology.

Pleomorphic xanthoastrocytoma (PXA) is a slow-growing neoplasm that predominantly affects the pediatric and young adult population. This neoplasm has a good prognosis, with a median 10-year survival rate of 70%. The majority of tumors are supratentorial and arise in the temporal lobe, while spinal tumors are extremely rare, with only 8 reported cases. Read More

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https://academic.oup.com/jnen/advance-article/doi/10.1093/jn
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http://dx.doi.org/10.1093/jnen/nly112DOI Listing
January 2019
18 Reads

Noninvasive, Targeted Creation of Neuromyelitis Optica Pathology in AQP4-IgG Seropositive Rats by Pulsed Focused Ultrasound.

J Neuropathol Exp Neurol 2019 Jan;78(1):47-56

Department of Medicine and Physiology.

Neuromyelitis optica spectrum disorders (herein called NMO) is an autoimmune disease of the CNS characterized by astrocyte injury, inflammation, and demyelination. In seropositive NMO, immunoglobulin G autoantibodies against aquaporin-4 (AQP4-IgG) cause primary astrocyte injury. A passive transfer model of NMO was developed in which spatially targeted access of AQP4-IgG into the CNS of seropositive rats was accomplished by pulsed focused ultrasound through intact skin. Read More

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http://dx.doi.org/10.1093/jnen/nly107DOI Listing
January 2019
3 Reads

MGMT: Immunohistochemical Detection in High-Grade Astrocytomas.

J Neuropathol Exp Neurol 2019 Jan;78(1):57-64

The Preston Robert Tisch Brain Tumor Center, Duke University Medical Center, Durham, NC, USA.

Glioma therapeutic resistance to alkylating chemotherapy is mediated via O6-methylguanine-DNA methyltransferase (MGMT). We hypothesized that a CD45/HAM56/MGMT double-stained cocktail would improve MGMT discrimination in tumor cells versus inflammatory and endothelial cells (IEC). Total MGMT protein was quantified by IHC on 982 glioblastomas (GBM) and 199 anaplastic astrocytomas. Read More

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http://dx.doi.org/10.1093/jnen/nly110DOI Listing
January 2019
14 Reads

Divergent Features of Mitochondrial Deficiencies in LGMD2A Associated With Novel Calpain-3 Mutations.

J Neuropathol Exp Neurol 2019 Jan;78(1):88-98

Neuromuscular Diagnostic Laboratory, Department of Pathology and Laboratory Medicine, American University of Beirut Medical Center, Beirut, Lebanon.

Limb girdle muscular dystrophy type 2A (LGMD2A) is an autosomal recessive disorder characterized by progressive muscle weakness and wasting. LGMD2A is caused by mutations in the calpain-3 gene (CAPN3) that encodes a Ca2+-dependent cysteine protease predominantly expressed in the skeletal muscle. Underlying pathological mechanisms have not yet been fully elucidated. Read More

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http://dx.doi.org/10.1093/jnen/nly113DOI Listing
January 2019
5 Reads
3.797 Impact Factor

SIV-Induced Immune Activation and Metabolic Alterations in the Dorsal Root Ganglia During Acute Infection.

J Neuropathol Exp Neurol 2019 Jan;78(1):78-87

Department of Molecular and Comparative Pathobiology.

Human immunodeficiency virus-associated sensory neuropathy (HIV-SN) remains a frequent neurologic complication of HIV infection. Little is known about alterations in the peripheral nervous system during the early stages of HIV, a time when neuroprotective interventions may be most beneficial. We performed Nanostring gene expression analysis on lumbar dorsal root ganglia (DRG) from 6 simian immunodeficiency virus (SIV)-infected pigtailed macaques killed at 7 days post-inoculation and 8 uninfected controls. Read More

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http://dx.doi.org/10.1093/jnen/nly111DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6289222PMC
January 2019
2 Reads

Combined Adipose-Derived Mesenchymal Stem Cells and Low-Energy Extracorporeal Shock Wave Therapy Protect the Brain From Brain Death-Induced Injury in Rat.

J Neuropathol Exp Neurol 2019 Jan;78(1):65-77

Center for Shockwave Medicine and Tissue Engineering, Kaohsiung Chang Gung Memorial Hospital, Kaohsiung, Taiwan.

This study tested the hypothesis that combined adipose-derived mesenchymal stem cell (ADMSC) and low-energy extracorporeal shock wave (ECSW) therapy could protect brain from brain death (BD)-induced injury. Adult male Sprague Dawley rats were categorized into group 1 (sham control), group 2 (BD), group 3 (BD + ECSW [0.15 mJ/mm2/300 impulses] applied to the skull surface 3 hours after BD induction), group 4 (BD + ADMSC [1. Read More

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https://academic.oup.com/jnen/advance-article/doi/10.1093/jn
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http://dx.doi.org/10.1093/jnen/nly108DOI Listing
January 2019
9 Reads

Sarcolemmal Complement Membrane Attack Complex Deposits During Acute Rejection of Myofibers in Nonhuman Primates.

J Neuropathol Exp Neurol 2019 Jan;78(1):38-46

Axe Neurosciences, Research Center of the CHU de Quebec - CHUL, Quebec, Canada.

We have previously studied in nonhuman primates several aspects of the acute rejection of myofibers, including the histological characteristics, the mechanisms of myofiber elimination by the T cells, and the development of anti-donor antibodies. Here, we report the participation of the complement membrane attack complex (MAC) in this context. We used muscle sections of macaques from experiments of allogeneic muscle precursor cell transplantation with confirmed rejection of the graft-derived myofibers. Read More

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http://dx.doi.org/10.1093/jnen/nly106DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6289216PMC
January 2019
2 Reads
3.797 Impact Factor

Quantitative Assessment of Pathological Tau Burden in Essential Tremor: A Postmortem Study.

J Neuropathol Exp Neurol 2019 Jan;78(1):31-37

Department of Pathology.

Essential tremor (ET) patients develop more cognitive impairment and dementia than controls, although there are surprisingly few data on the neuropathological basis for cognitive changes in ET. In this postmortem study, we assessed tau and other pathologies in 26 ET cases and 73 controls (non-ET) (1:3 matching). The mean age = 88. Read More

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https://academic.oup.com/jnen/advance-article/doi/10.1093/jn
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http://dx.doi.org/10.1093/jnen/nly104DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6289218PMC
January 2019
15 Reads

Severe TUBB4A-Related Hypomyelination With Atrophy of the Basal Ganglia and Cerebellum: Novel Neuropathological Findings.

J Neuropathol Exp Neurol 2019 Jan;78(1):3-9

Division of Pediatric Neurology, Children's Hospital of Eastern Ontario, Ottawa, Ontario Canada.

Hypomyelination with atrophy of the basal ganglia and cerebellum (H-ABC) is a rare hypomyelinating leukodystrophy characterized by infantile or childhood onset of motor developmental delay, progressive rigidity and spasticity, with hypomyelination and progressive atrophy of the basal ganglia and cerebellum due to a genetic mutation of the TUBB4A gene. It has only been recognized since 2002 and the full spectrum of the disorder is still being delineated. Here, we review a case report of a severely affected girl with a thorough neuropathological evaluation demonstrating novel clinical and pathological findings. Read More

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http://dx.doi.org/10.1093/jnen/nly105DOI Listing
January 2019
15 Reads
3.797 Impact Factor

Subchronic Pathobiological Response Following Chronic Repetitive Mild Traumatic Brain Injury in an Aged Preclinical Model of Amyloid Pathogenesis.

J Neuropathol Exp Neurol 2018 Dec;77(12):1144-1162

Experimental Neuropathology and TBI Research Division, Roskamp Institute, Sarasota, Florida.

Repetitive mild traumatic brain injury (r-mTBI) is a risk factor for Alzheimer disease (AD). The precise nature of how r-mTBI leads to, or precipitates, AD pathogenesis remains unclear. In this study, we explore subchronic effects of chronic r-mTBI (12-impacts) administered over 1-month in aged-PS1/APP mice and littermate controls. Read More

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https://academic.oup.com/jnen/advance-article/doi/10.1093/jn
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http://dx.doi.org/10.1093/jnen/nly101DOI Listing
December 2018
13 Reads

Lactate Deficit in an Alzheimer Disease Mouse Model: The Relationship With Neuronal Damage.

J Neuropathol Exp Neurol 2018 Dec;77(12):1163-1176

Department of Physiology, Army Medical University (Third Military Medical University), Chongqing, China.

Cerebral energy metabolism in Alzheimer disease (AD) has recently been given increasing attention. This study focuses on the alterations of cerebral lactate metabolism in the double-transgenic amyloid precursor protein/presenilin 1 (APP/PS1) mouse model of AD. Immunofluorescence staining and Western blotting analysis were used to identify the alterations of lactate content and lactate transporters (MCT1, MCT2, MCT4) in APP/PS1 mouse brains, which display amyloid beta plaques, reduced amounts of neurons and oligodendrocytes, and increased quantity of astrocytes. Read More

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http://dx.doi.org/10.1093/jnen/nly102DOI Listing
December 2018
4 Reads