Tenascin-C is associated with cored amyloid-{beta} plaques in Alzheimer disease and pathology burdened cognitively normal elderly
(Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - December 7, 2016 Category: Neurology Tags: Erratum Source Type: research

Appreciation of Reviewers
(Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - December 7, 2016 Category: Neurology Tags: Letter To The Editor Source Type: research

Prognostic Biomarkers in Spinal Chordoma: A Systematic Review
(Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - December 7, 2016 Category: Neurology Authors: Zou, M.-X., Lv, G.-H., Wang, X.-B., Li, J. Tags: Letter to the Editor Source Type: research

Examining Neuropathology: Beginning a Dialogue
(Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - December 7, 2016 Category: Neurology Authors: Wiley, C., Hart, M. N., Weidenheim, K. M., Louis, D. N., Lee, S. C. Tags: Feature Source Type: research

Tubular Aggregates and Cylindrical Spirals Have Distinct Immunohistochemical Signatures
We describe the histopathological features of myopathies with tubular aggregates, including a detailed immunohistochemical analysis of congenital myasthenic syndromes with tubular aggregates due to mutations in GFPT1 and DPAGT1, and myopathies with cylindrical spirals. Our findings support the notion that cylindrical spirals, like tubular aggregates, derive primarily from the sarcoplasmic reticulum; however, immunohistochemistry indicates that different molecular components of the sarcoplasmic reticulum may be involved and can be used to distinguish between these different inclusions. The immunohistochemical differences ma...
Source: Journal of Neuropathology and Experimental Neurology - December 7, 2016 Category: Neurology Authors: Brady, S., Healy, E. G., Gang, Q., Parton, M., Quinlivan, R., Jacob, S., Curtis, E., Al-Sarraj, S., Sewry, C. A., Hanna, M. G., Houlden, H., Beeson, D., Holton, J. L. Tags: Original Articles Source Type: research

Endothelial Cell Hypertrophy and Microvascular Proliferation in Meningiomas Are Correlated with Higher Histological Grade and Shorter Progression-Free Survival
Microvascular proliferation (MVP) is a hallmark of glioblastoma. Endothelial cell hypertrophy (ECH), also known as endothelial hyperplasia, is correlated with a shorter survival of patients with gliomas. However, the prognostic value of these 2 morphological features has not been studied in meningiomas. The aim of this study was to evaluate the prognostic value of angiogenesis in meningiomas, most notably ECH, MVP, and microvascular density, which were evaluated using immunohistochemistry with antibodies against CD34 and CD105 (a marker of neovascularization) in a series of 139 meningiomas. ECH, MVP, and CD105 immunoreacti...
Source: Journal of Neuropathology and Experimental Neurology - December 7, 2016 Category: Neurology Authors: Ling, C., Pouget, C., Rech, F., Pflaum, R., Treffel, M., Bielle, F., Mokhtari, K., Casse, J.-M., Vignaud, J.-M., Kalamarides, M., Peyre, M., Gauchotte, G. Tags: Original Articles Source Type: research

Contactin-Associated Protein 1 (CNTNAP1) Mutations Induce Characteristic Lesions of the Paranodal Region
We present the results of nerve biopsy studies of three patients from two unrelated, non-consanguineous families with compound heterozygous CNTNAP1 mutations. The lesions were identical, characterized by a hypomyelinating process; on electron microscopy, we detected, in all nodes of Ranvier, subtle lesions that have never been previously described in human nerves. Transverse bands of the myelin loops were absent, with a loss of attachment between myelin and the axolemma; elongated Schwann cell processes sometimes dissociated the Schwann cell and axon membranes that bound the space between them. These lesions were observed ...
Source: Journal of Neuropathology and Experimental Neurology - December 7, 2016 Category: Neurology Authors: Vallat, J.-M., Nizon, M., Magee, A., Isidor, B., Magy, L., Pereon, Y., Richard, L., Ouvrier, R., Cogne, B., Devaux, J., Zuchner, S., Mathis, S. Tags: Original Articles Source Type: research

Chronic Traumatic Encephalopathy-Like Abnormalities in a Routine Neuropathology Service
Chronic traumatic encephalopathy (CTE) has been described mainly in professional athletes and military personnel and is characterized by deposition of hyperphosphorylated tau at the depths of cortical sulci and around blood vessels. To assess CTE-like changes in a routine neuropathology service, we prospectively examined 111 brains (age 18–60 years). The presence of tau-immunoreactive deposits was staged using guidelines described by others and was correlated with the medical history. 72/111 cases were negative for CTE-like changes; 34/111 were CTE stage <1; 3/111 were CTE stage 1; and 2/111 were CTE stage 2. The ...
Source: Journal of Neuropathology and Experimental Neurology - December 7, 2016 Category: Neurology Authors: Noy, S., Krawitz, S., Del Bigio, M. R. Tags: Original Articles Source Type: research

New Strategy That Delays Progression of Amyotrophic Lateral Sclerosis in G1H-G93A Transgenic Mice: Oral Administration of Xanthine Oxidoreductase Inhibitors That Are Not Substrates for the Purine Salvage Pathway
Amyotrophic lateral sclerosis (ALS), Lou Gehrig’s disease, is a progressive fatal neurodegenerative disease that involves both upper and lower motor neurons. We orally administered 4 xanthine oxidoreductase (XOR) inhibitors to G1H-G93A mice carrying 25 transgene copy numbers of human mutant G93A superoxide dismutase 1, from 80 days of age. Three nonpurine-analogue inhibitors (TEI-6720: Febuxostat, Y-700 and FYX-051), but not allopurinol with a purine analogue ring (pyrazolo pyrimidine ring), significantly delayed disease onset, prolonged survival and the duration of disease stages, improved clinical signs, and allevi...
Source: Journal of Neuropathology and Experimental Neurology - December 7, 2016 Category: Neurology Authors: Kato, S., Kato, M., Kusano, T., Nishino, T. Tags: Original Articles Source Type: research

Quantitative Analysis of the Cellular Microenvironment of Glioblastoma to Develop Predictive Statistical Models of Overall Survival
We examined relationships of the cellular microenvironment, including astrocytes, microglia, oligodendrocytes, and blood vessels, to survival in glioblastoma patients. Using histological staining and quantitative image analyses, we examined the tumor-associated parenchyma of 33 patients and developed statistical models to predict patient outcomes based on the cellular picture of the tumor parenchyma. We found that blood vessel density correlated with poorer prognosis. To examine the role of adjacent parenchymal versus higher tumor cell density bulk parenchymal tissue, we examined the glial components in these highly variab...
Source: Journal of Neuropathology and Experimental Neurology - December 7, 2016 Category: Neurology Authors: Yuan, J. X., Bafakih, F. F., Mandell, J. W., Horton, B. J., Munson, J. M. Tags: Original Articles Source Type: research

Shared and Distinct Patterns of Oligodendroglial Response in {alpha}-Synucleinopathies and Tauopathies
Pathological protein deposits in oligodendroglia are common but variable features of various neurodegenerative conditions. To evaluate oligodendrocyte response in neurodegenerative diseases (NDDs) with different extents of oligodendroglial protein deposition we performed immunostaining for tubulin polymerization-promoting protein p25α (TPPP/p25α), α-synuclein (α-syn), phospho-tau, ubiquitin, myelin basic protein, and the microglial marker HLA-DR. We investigated cases of multiple system atrophy ([MSA] n = 10), Lewy body disease ([LBD] n = 10), globular glial tauopathy ([GGT] n = 7) and progressive s...
Source: Journal of Neuropathology and Experimental Neurology - December 7, 2016 Category: Neurology Authors: Rohan, Z., Milenkovic, I., Lutz, M. I., Matej, R., Kovacs, G. G. Tags: Original Articles Source Type: research

In This Issue
(Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - December 7, 2016 Category: Neurology Tags: In This Issue Source Type: research

2016 Award Recipients -- Barbara J. Crain, MD, PhD and Dennis W. Dickson, MD
(Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - October 17, 2016 Category: Neurology Authors: Hart, M. N., Murray, M. Tags: Meritorious Awards Source Type: research

Surl L. Nielsen, MD
(Source: Journal of Neuropathology and Experimental Neurology)
Source: Journal of Neuropathology and Experimental Neurology - October 17, 2016 Category: Neurology Tags: In Memoriam Source Type: research

Radiation-Induced Cerebral Vascular "Malformations" at Biopsy
Radiation-induced vascular "malformations", designated cavernous hemangiomas/cavernomas ("RICHs"), are seldom biopsied and are usually diagnosed based on neuroimaging features. They are an increasingly recognized complication of both CNS external beam radiation therapy and stereotactic radiosurgery. We identified 13 patients with radiation-induced vascular "malformations" in our surgical neuropathology databases searched from 2000 to 2016; 4 had received their therapy during childhood; 5 had received radiosurgery. Inclusion required identifiable vascular abnormalities on neuroimaging and/or, if recurrent tumor was addition...
Source: Journal of Neuropathology and Experimental Neurology - October 17, 2016 Category: Neurology Authors: Kleinschmidt-DeMasters, B. K., Lillehei, K. O. Tags: Original Articles Source Type: research