Journal of Neuropathology and Experimental Neurology This is an RSS file. You can use it to subscribe to this data in your favourite RSS reader or to display this data on your own website or blog.

Mitotic Events in Cerebellar Granule Progenitor Cells That Expand Cerebellar Surface Area Are Critical for Normal Cerebellar Cortical Lamination in Mice
Abstract: Late embryonic and postnatal cerebellar folial surface area expansion promotes cerebellar cortical cytoarchitectural lamination. We developed a streamlined sampling scheme to generate unbiased estimates of murine cerebellar surface area and volume using stereologic principles. We demonstrate that, during the proliferative phase of the external granular layer (EGL) and folial surface area expansion, EGL thickness does not change and thus is a topological proxy for progenitor self-renewal. The topological constraints indicate that, during proliferative phases, migration out of the EGL is balanced by self-renewal. P...
Source: Journal of Neuropathology and Experimental Neurology - February 14, 2015 Category: Neurology Tags: Original Articles Source Type: research

The Pathogenesis of 3 Neurotropic Flaviviruses in a Mouse Model Depends on the Route of Neuroinvasion After Viremia
In conclusion, the three neurotropic flaviviruses showed different pathogenesis, which were dependent upon overlapping but distinct pathways to CNS invasion after viremia. (Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - February 14, 2015 Category: Neurology Tags: Original Articles Source Type: research

Molecular Classification Defines 4 Prognostically Distinct Glioma Groups Irrespective of Diagnosis and Grade
Abstract: According to World Health Organization criteria, diffuse gliomas are divided into several histological subtypes, including astrocytomas, oligodendrogliomas, and oligoastrocytomas, and 4 malignancy grades (I–IV). Molecular alterations, such as the isocitrate dehydrogenase gene (IDH) mutation or 1p/19q loss, are found in these tumors but are not included in the current classification system. Recently, mutation of α thalassemia/mental retardation syndrome X-linked (ATRX) gene and its loss of expression have been reported in infiltrating gliomas. We evaluated ATRX protein expression in 272 gliomas and its associat...
Source: Journal of Neuropathology and Experimental Neurology - February 14, 2015 Category: Neurology Tags: Original Articles Source Type: research

Pathologic Staging of White Matter Lesions in Adult-Onset Leukoencephalopathy/Leukodystrophy With Axonal Spheroids
Abstract: The pathologic features of adult-onset leukoencephalopathy/leukodystrophy with axonal spheroids (ALAS) are variable, and this has led to different hypotheses as to whether primarily demyelination or axonopathy may underlie this disorder. Typical ALAS pathology is rarely accompanied by focal multiple sclerosis (MS)–like plaques. In ALAS pathology accompanied by focal multiple sclerosis (MS)-like plaques cases, the pathologic features cannot be distinguished from those of progressive MS with diffusely abnormal white matter. To clarify these issues, we examined neuropathologic features in 159 representative sample...
Source: Journal of Neuropathology and Experimental Neurology - February 14, 2015 Category: Neurology Tags: Original Articles Source Type: research

Components of Myelin Damage and Repair in the Progression of White Matter Pathology After Mild Traumatic Brain Injury
We examined the progression of white matter pathology in mice after mild TBI with traumatic axonal injury localized in the corpus callosum. Adult mice received a closed-skull impact and were analyzed from 3 days to 6 weeks post-TBI/sham surgery. At all times post-TBI, electron microscopy revealed degenerating axons distributed among intact fibers in the corpus callosum. Intact axons exhibited significant demyelination at 3 days followed by evidence of remyelination at 1 week. Accordingly, bromodeoxyuridine pulse-chase labeling demonstrated the generation of new oligodendrocytes, identified by myelin proteolipid protein mes...
Source: Journal of Neuropathology and Experimental Neurology - February 14, 2015 Category: Neurology Tags: Original Articles Source Type: research

Protective Role of Anakinra Against Transthyretin-Mediated Axonal Loss and Cell Death in a Mouse Model of Familial Amyloidotic Polyneuropathy
Abstract: Familial amyloidotic polyneuropathy (FAP) is characterized by a length-dependent axonal loss in the peripheral nervous system that results from deposition of extracellular prefibrillar transthyretin (TTR) and amyloid fibrils. We have previously shown that an inflammatory stimulus in the peripheral nerve in a mouse model of FAP triggers local TTR expression and deposition, leading to poor regeneration. We also demonstrated that blocking interleukin-1 (IL-1) signaling by the IL-1 receptor antagonist anakinra is beneficial in preventing nerve TTR deposition and associated toxicity. Here, we investigated whether IL-1...
Source: Journal of Neuropathology and Experimental Neurology - February 14, 2015 Category: Neurology Tags: Original Articles Source Type: research

Immediate, Irreversible, Posttraumatic Coma: A Review Indicating That Bilateral Brainstem Injury Rather Than Widespread Hemispheric Damage Is Essential for Its Production
Abstract: Traumatic brain injury may result in immediate long-lasting coma. Much attention has been given to predicting this outcome from the initial examination because these predictions can guide future treatment and interactions with the patient’s family. Reports of diffuse axonal injury in these cases have ascribed the coma to widespread damage in the deep white matter that disconnects the hemispheres from the ascending arousal system (AAS). However, brainstem lesions are also present in such cases, and the AAS may be interrupted at the brainstem level. This review examines autopsy and imaging literature that assesse...
Source: Journal of Neuropathology and Experimental Neurology - February 14, 2015 Category: Neurology Tags: Review Source Type: research

Reduction in Serum Aquaporin-4 Antibody Titers During Development of a Tumor-Like Brain Lesion in a Patient With Neuromyelitis Optica: A Serum Antibody–Consuming Effect?
We report a patient with NMO whose AQP4 antibody levels decreased simultaneously with clinical deterioration caused by the development of a tumor-like brain lesion. In the seminecrotic biopsied brain lesion, there was activated complement complex, whereas only very scattered immunoreactivity to AQP4 protein was detectable. The decrease in serum AQP4 antibody levels and the loss of AQP4 in the tumor-like lesion could represent a “serum antibody–consuming effect” during lesion formation. (Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - February 14, 2015 Category: Neurology Tags: Brief Report Source Type: research

In This Issue
No abstract available (Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - February 14, 2015 Category: Neurology Tags: In This Issue Source Type: research

Cytomegalovirus and Glioblastoma: A Review of Evidence for Their Association and Indications for Testing and Treatment: Erratum
No abstract available (Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - January 23, 2015 Category: Neurology Tags: Erratum Source Type: research

Friedreich Ataxia: Failure of GABA-ergic and Glycinergic Synaptic Transmission in the Dentate Nucleus
Abstract: Atrophy of large neurons in the dentate nucleus (DN) is an important pathologic correlate of neurologic disability in patients with Friedreich ataxia (FA). Thinning of the DN was quantified in 29 autopsy cases of FA and 2 carriers by measuring the thickness of the gray matter ribbon on stains with anti–glutamic acid decarboxylase, the rate-limiting enzyme in the biosynthesis of γ-amino-butyric acid (GABA). The DN was thinner than normal in all cases of FA, and atrophy correlated inversely with disease duration but not with age at onset or length of the homozygous guanine-adenine-adenine trinucleotide expansion...
Source: Journal of Neuropathology and Experimental Neurology - January 23, 2015 Category: Neurology Tags: Original Articles Source Type: research

Adenosine Kinase Expression in Cortical Dysplasia With Balloon Cells: Analysis of Developmental Lineage of Cell Types
Abstract: Focal cortical dysplasia type IIB (FCDIIB) is a developmental malformation of the cerebral cortex that is associated with pharmacoresistant epilepsy. Overexpression of adenosine kinase (ADK) has been regarded as a pathologic hallmark of epilepsy. We hypothesized that the epileptogenic mechanisms underlying FCDIIB are related to abnormal ADK expression. We used immunohistochemistry to examine the expression of ADK and of heterogeneous cell population markers of astrocytes (glial fibrillary acidic protein), immature glia (vimentin), immature neurons (neuronal class III beta-tubulin, TUJ1), multipotential progenitor...
Source: Journal of Neuropathology and Experimental Neurology - January 23, 2015 Category: Neurology Tags: Original Articles Source Type: research

Cytomegalovirus and Glioblastoma: A Review of Evidence for Their Association and Indications for Testing and Treatment: Erratum
No abstract available (Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - January 16, 2015 Category: Neurology Tags: Erratum Source Type: research

Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS): Postmortem Findings
No abstract available (Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - January 16, 2015 Category: Neurology Tags: Letter to the Editor Source Type: research

The Chromatin-Modifying Protein HMGA2 Promotes Atypical Teratoid/Rhabdoid Cell Tumorigenicity
AbstractAtypical teratoid/rhabdoid tumor (AT/RT) is an aggressive pediatric central nervous system tumor. The poor prognosis of AT/RT warrants identification of novel therapeutic targets and strategies. High-mobility Group AT-hook 2 (HMGA2) is a developmentally important chromatin-modifying protein that positively regulates tumor growth, self-renewal, and invasion in other cancer types. High-mobility group A2 was recently identified as being upregulated in AT/RT tissue, but the role of HMGA2 in brain tumors remains unknown. We used lentiviral short-hairpin RNA to suppress HMGA2 in AT/RT cell lines and found that loss of HM...
Source: Journal of Neuropathology and Experimental Neurology - January 16, 2015 Category: Neurology Tags: Original Articles Source Type: research