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.

Spectrum of Neuropathophysiology in Spinal Muscular Atrophy Type I
We present a pathophysiologic model for SMA-I as a protein deficiency disease affecting a neuronal network with variable clinical thresholds. Because new treatment strategies improve survival of infants with SMA-I, a better understanding of these factors will guide future treatments. (Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - December 19, 2014 Category: Neurology Tags: Original Articles Source Type: research

Visualization of HTLV-1–Specific Cytotoxic T Lymphocytes in the Spinal Cords of Patients With HTLV-1–Associated Myelopathy/Tropical Spastic Paraparesis
AbstractActivated human T-lymphotropic virus type-1 (HTLV-1)–specific CD8-positive cytotoxic T lymphocytes (CTLs) are markedly increased in the periphery of patients with HTLV-1–associated myelopathy/tropical spastic paraparesis (HAM/TSP), an HTLV-1–induced inflammatory disease of the CNS. Although virus-specific CTLs play a pivotal role to eliminate virus-infected cells, the potential role of HTLV-1–specific CTLs in the pathogenesis of HAM/TSP remains unclear. To address this issue, we evaluated the infiltration of HTLV-1–specific CTLs and the expression of HTLV-1 proteins in the spinal cords of 3 patients with ...
Source: Journal of Neuropathology and Experimental Neurology - December 19, 2014 Category: Neurology Tags: Original Articles Source Type: research

In This Issue
No abstract available (Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - December 19, 2014 Category: Neurology Tags: In This Issue Source Type: research

Appreciation of Reviewers
No abstract available (Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - November 18, 2014 Category: Neurology Tags: Appreciation of Reviewers Source Type: research

Networks of the Brain
No abstract available (Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - November 18, 2014 Category: Neurology Tags: Book Review Source Type: research

Early Pathologic Changes in Hereditary Diffuse Leukoencephalopathy With Spheroids
In conclusion, our observations suggest that the pathology of HDLS may initially be characterized by multifocal lesions in subcortical white matter regions. Moreover, pigmented microglia poorly express CSF1R and are distributed diffusely throughout the white matter at the early disease stage, preceding axonal damage and myelin loss. (Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - November 18, 2014 Category: Neurology Tags: Original Articles Source Type: research

The Calcium-Binding Protein EFhd2 Modulates Synapse Formation In Vitro and Is Linked to Human Dementia
AbstractEFhd2 is a calcium-binding adaptor protein that has been found to be associated with pathologically aggregated tau in the brain in Alzheimer disease and in a mouse model of frontotemporal dementia. EFhd2 has cell type–specific functions, including the modulation of intracellular calcium responses, actin dynamics, and microtubule transport. Here we report that EFhd2 protein and mRNA levels are reduced in human frontal cortex tissue affected by different types of dementia with and without tau pathology. We show that EFhd2 is mainly a neuronal protein in the brain and is abundant in the forebrain. Using short hairpi...
Source: Journal of Neuropathology and Experimental Neurology - November 18, 2014 Category: Neurology Tags: Original Articles Source Type: research

Imaging of Spontaneous Ventriculomegaly and Vascular Malformations in Wistar Rats: Implications for Preclinical Research
AbstractWistar rats are widely used in biomedical research and commonly serve as a model organism in neuroscience studies. In most cases when noninvasive imaging is not used, studies assume a consistent baseline condition in rats that lack visible differences. While performing a series of traumatic brain injury studies, we discovered mild spontaneous ventriculomegaly in 70 (43.2%) of 162 Wistar rats that had been obtained from 2 different vendors. Advanced magnetic resonance (MR) imaging techniques, including MR angiography and diffusion tensor imaging, were used to evaluate the rats. Multiple neuropathologic abnormalities...
Source: Journal of Neuropathology and Experimental Neurology - November 18, 2014 Category: Neurology Tags: Original Articles Source Type: research

Early and Persistent Expression of Phosphorylated α-Synuclein in the Enteric Nervous System of A53T Mutant Human α-Synuclein Transgenic Mice
We examined the time course of pSer129 α-synuclein presence in the gut of these mice between 1.5 and 22 months of age using immunohistochemistry and paraffin-embedded tissue blots. The pSer129 α-synuclein accumulated early (before the onset of motor signs) and persistently in the enteric nervous system and was concomitantly found in the brain. These results suggest that the accumulation of phosphorylated α-synuclein in the enteric and central nervous systems may result from parallel pathologic processes when the disease is linked to a mutation of α-synuclein. (Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - November 18, 2014 Category: Neurology Tags: Original Articles Source Type: research

Brain Injury in Canine Models of Cardiac Surgery
AbstractNeuropathology and neurologic impairment were characterized in a clinically relevant canine model of hypothermic (18°C) circulatory arrest (HCA) and cardiopulmonary bypass (CPB). Adult dogs underwent 2 hours of HCA (n = 39), 1 hour of HCA (n = 20), or standard CPB (n = 22) and survived 2, 8, 24, or 72 hours. Neurologic impairment and neuropathology were much more severe after 2-hour HCA than after 1-hour HCA or CPB; histopathology and neurologic deficit scores were significantly correlated. Apoptosis developed as early as 2 hours after injury and was most severe in the granule cells of the hippocampal dentate gyru...
Source: Journal of Neuropathology and Experimental Neurology - November 18, 2014 Category: Neurology Tags: Original Articles Source Type: research

Regional Ischemic Immune Myopathy: A Paraneoplastic Dermatomyopathy
AbstractNecrosis and regeneration of scattered muscle fibers are common features of many active acquired and immune myopathies. We studied a series of patients with acquired myopathies with an unusual pattern of regional, rather than scattered, muscle fiber necrosis and regeneration. Retrospective review of records of 7 patients with acquired myopathies having regional muscle fiber necrosis on muscle biopsy. Clinical features of patients included proximal symmetric weakness in arms and legs with a subacute onset (100%) beginning at ages between 41 and 92 years, with dysphagia (83%), myalgias (100%), skin rash (67%), and as...
Source: Journal of Neuropathology and Experimental Neurology - November 18, 2014 Category: Neurology Tags: Original Articles Source Type: research

Immunoglobulin Repertoire of Primary Lymphomas of the Central Nervous System
AbstractPrimary lymphoma of the central nervous system (PCNSL) is a diffuse large B-cell lymphoma confined to the CNS. It has been hypothesized that antigen(s) in the CNS may trigger tumor cell proliferation. Because efforts to identify potential antigens have been unsuccessful to date, we studied the B-cell receptor in detail in a comprehensive series of 50 PCNSLs to obtain indirect information on potential antigens. Potentially functional V-D-J rearrangements were identified in all PCNSLs analyzed. Immunoglobulin heavy-chain variable gene segment (IGHV), IGHV4, was the predominant family used by 66% (33 of 50) of PCNSLs ...
Source: Journal of Neuropathology and Experimental Neurology - November 18, 2014 Category: Neurology Tags: Original Articles Source Type: research

A Tick-Borne Encephalitis Model in Infant Rats Infected With Langat Virus
AbstractTick-borne encephalitis virus (TBEV) is the causative agent of human TBE, a severe infection that can cause long-lasting neurologic sequelae. Langat virus (LGTV), which is closely related to TBEV, has a low virulence for human hosts and has been used as a live vaccine against TBEV. Tick-borne encephalitis by natural infection of LGTV in humans has not been described, but one of 18,500 LGTV vaccinees developed encephalitis. The pathogenetic mechanisms of TBEV are poorly understood and, currently, no effective therapy is available. We developed an infant rat model of TBE using LGTV as infective agent. Infant Wistar r...
Source: Journal of Neuropathology and Experimental Neurology - November 18, 2014 Category: Neurology Tags: Original Articles Source Type: research

Positive or Negative Involvement of Heat Shock Proteins in Multiple Sclerosis Pathogenesis: An Overview
This article reviews our current understanding of the involvement of the principal HSP families in MS. (Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - November 18, 2014 Category: Neurology Tags: Review Article Source Type: research

In This Issue
No abstract available (Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - November 18, 2014 Category: Neurology Tags: In This Issue Source Type: research