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Neuropathology: Official Journal of the Japanese Society of Neuropathology

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https://www.readbyqxmd.com/read/29748975/lysosomes-autophagosomes-and-alzheimer-pathology-in-dementia-with-lewy-body-disease
#1
Rowan Gurney, Yvonne S Davidson, Andrew C Robinson, Anna Richardson, Matthew Jones, Julie S Snowden, David M A Mann
A failure of protein degradation may underpin Lewy body disease (LBD) where α-synuclein is assimilated into the pathognomic Lewy bodies and Lewy neurites. We investigated histological alterations in lysosomes and autophagosomes in the substantia nigra (SN) and cingulate gyrus (CG) in 34 patients with LBD employing antibodies against phosphorylated α-synuclein and lysosomal (lysosomal associated membrane proteins 1 and 2 (LAMP-1 and LAMP-2), cathepsin D (CTSD)) and autophagosomal (microtubule-associated protein light chain 3α (LC3A)) proteins...
May 10, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29718563/first-pathological-report-of-a-de-novo-cd5-positive-diffuse-large-b-cell-lymphoma-patient-presenting-with-guillain-barr%C3%A3-syndrome-like-neuropathy-due-to-neurolymphomatosis
#2
Mikiko Kobayashi, Yasuhiro Sakai, Yuta Kariya, Hitoshi Sakai, Akiyo Hineno, Kiyomitsu Oyanagi, Hiroyuki Kanno
Peripheral neuropathy occurs in approximately 5% of the patients with lymphoma. Two major causes of peripheral neuropathy associated with lymphoma are neurolymphomatosis and paraneoplastic neuropathy such as demyelinating neuropathy. The differential diagnosis between neurolymphomatosis and demyelinating neuropathy is difficult, because electrophysiological findings suggestive of demyelination are frequently observed even in patients with neurolymphomatosis. Here, we report a patient with de novo CD5-positive diffuse large B-cell lymphoma (DLBCL) who presented with Guillain-Barré syndrome (GBS)-like neuropathy...
May 2, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29675936/pediatric-ganglioglioma-with-an-h3-k27m-mutation-arising-from-the-cervical-spinal-cord
#3
Tomohiro Okuda, Nobuhiro Hata, Satoshi O Suzuki, Koji Yoshimoto, Koichi Arimura, Takeo Amemiya, Yojiro Akagi, Daisuke Kuga, Utako Oba, Yuhki Koga, Shouichi Ohga, Toru Iwaki, Koji Iihara
The 2016 edition of the World Health Organization Classification of Tumors of the Central Nervous System introduced "diffuse midline glioma H3 K27M mutant" as a new diagnostic entity. These tumors predominately affect pediatric patients and arise from midline structures such as the brainstem, thalamus and spinal cord. Here, we report a rare patient with spinal ganglioglioma carrying an H3 K27M mutation. A 10-year-old boy presented with an intramedullary tumor in the cervical spinal cord. The lesion was partially removed and histologically diagnosed as ganglioglioma...
April 19, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29665111/inhibition-of-transient-receptor-potential-vanilloid-4-decreases-the-expressions-of-caveolin-1-and-caveolin-2-after-focal-cerebral-ischemia-and-reperfusion-in-rats
#4
Hui Xie, Wei-Cheng Lu
This study aimed to investigate the effects of transient receptor potential vanilloid 4 (TRPV4) inhibition on blood-brain barrier (BBB) integrity and the expressions of caveolae structural proteins caveolin-1 and caveolin-2 in rats with focal cerebral ischemia and reperfusion. BBB permeability was assessed by Evans blue extravasation. The mRNA and protein expressions of caveolin-1 and caveolin-2 were determined by RT-PCR, Western blot and immunohistochemistry assays. We found that BBB permeability significantly increased and reaches its peak at 72 h of reperfusion in cerebral ischemia-reperfusion rats and is able to be ameliorated by administration of HC-067047, an antagonist of TRPV4...
April 17, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29635846/primary-lymphomatoid-granulomatosis-in-the-central-nervous-system-a-report-of-three-cases
#5
Joong-Yub Kim, Kyeong Cheon Jung, Sung-Hye Park, Ji-Young Choe, Ji Eun Kim
Lymphomatoid granulomatosis (LYG) is a rare lymphoproliferative disorder characterized by infiltration of Epstein-Barr virus (EBV)-positive large atypical B-cells in an angiocentric fashion in a mixed inflammatory background. The histologic spectrum of LYG ranges from reactive proliferation to diffuse large B-cell lymphoma according to the number of EBV+ B-cells. It is known that virtually all patients have pulmonary involvement, whereas primary LYG of the other organs has been rarely reported. Herein, we describe three cases of primary LYG of the central nervous system (CNS) without pulmonary lesions, and this is the first collection to be reported in Korea...
April 10, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29635724/cerebellar-high-grade-astrocytoma-with-idh-mutations-in-the-elderly-a-report-of-two-cases
#6
Nozomi Matsumura, Hayato Ikota, Tatsuya Yamazaki, Satoshi Nakata, Sumihito Nobusawa, Junko Hirato, Yuhei Yoshimoto, Hideaki Yokoo
Cerebellar high-grade gliomas are rare, and likely to affect younger patients compared with those of cerebral origin. Recent genetic analyses have revealed that isocitrate dehydrogenase (IDH) 1/2 mutations are rare in infratentorial gliomas. In this paper, we report two elderly cases of IDH-mutated cerebellar high-grade glioma with unusual histological features and uncommon patient ages. One case was an 83-year-old man, whose tumor was predominantly composed of densely packed round-to-polygonal epithelioid cells...
April 10, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29603414/glt-1-mediates-exercise-induced-fatigue-through-modulation-of-glutamate-and-lactate-in-rats
#7
Dongmei Wang, Xingtong Wang
Glutamate has been implicated in exercise-induced fatigue, but the underlying mechanism is unknown. This study aimed to determine whether glutamate transporter-1 (GLT-1) has a key role in the regulation of exercise-induced fatigue. The expressions of GLT-1 and glial fibrillary acidic protein (GFAP) in the supplementary motor area of rats exposed to exhaustion were analyzed by immunohistochemistry. The expression of GLT-1 was further confirmed by Western blotting. The effects of GLT-1 on extracellular levels of glutamate and lactate and on exercise endurance were studied by microdialysis...
March 30, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29603402/expression-of-crym-in-different-rat-organs-during-development-and-its-decreased-expression-in-degenerating-pyramidal-tracts-in-amyotrophic-lateral-sclerosis
#8
Reiji Hommyo, Satoshi O Suzuki, Nona Abolhassani, Hideomi Hamasaki, Masahiro Shijo, Norihisa Maeda, Hiroyuki Honda, Yusaku Nakabeppu, Toru Iwaki
The protein μ-crystallin (CRYM) is a novel component of the marsupial lens that has two functions: it is a key regulator of thyroid hormone transportation and a reductase of sulfur-containing cyclic ketimines. In this study, we examined changes of the expression pattern of CRYM in different rat organs during development using immunohistochemistry and immunoblotting. As CRYM is reportedly expressed in the corticospinal tract, we also investigated CRYM expression in human cases of amyotrophic lateral sclerosis (ALS) using immunohistochemistry...
March 30, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29582466/unique-cell-tropism-of-hhv-6b-in-an-infantile-autopsy-case-of-primary-hhv-6b-encephalitis
#9
Hiroaki Miyahara, Kouki Miyakawa, Haruto Nishida, Shinji Yano, Tomoko Sonoda, So-Ichi Suenobu, Tatsuro Izumi, Tsutomu Daa, Kenji Ihara
Human herpes virus 6 (HHV-6) is known to cause primary encephalitis in the frontal lobes/cerebral hemisphere or reactivated encephalitis in the hippocampus, but the pathogenesis remains unclear. HHV-6B has also been detected in hippocampal samples in patients with mesial temporal lobe epilepsy. A 1 year and 3 months old female, who had been clinically diagnosed with exanthema subitum and febrile convulsion, was found dead on the third day after onset. Macroscopic findings showed massive brain edema. Microscopic examination revealed gemistocytic astrocytes and ballooned oligodendrocytes in the frontal white matter, along with neuronal cell death with microglial infiltration in the frontal cortex...
March 26, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29575092/novel-biomarker-phosphorylated-t-lak-cell-originated-protein-kinase-p-topk-can-predict-outcome-in-primary-central-nervous-system-lymphoma
#10
Masaki Koh, Yumiko Hayakawa, Takuya Akai, Tomohide Hayashi, Takahiro Tomita, Shoichi Nagai, Satoshi Kuroda
This study aimed to assess whether T-lymphokine-activated killer cell-originated protein kinase (TOPK) can be a potent novel biomarker to predict the outcome in patients with primary central nervous system lymphoma (PCNSL). This study enrolled 20 patients who were histologically diagnosed as having diffuse large B-cell type PCNSL between 2005 and 2015. Using surgical specimens, the expression of TOPK and phosphorylated TOPK (p-TOPK) was analyzed on immunohistochemistry. Clinical features such as age, sex, Karnofsky performance status (KPS), ocular involvement, deep brain structure involvement, the number of lesions, chemotherapy and radiation therapy were also collected...
March 25, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29575082/somatic-sprouts-of-the-purkinje-cells-in-a-patient-with-multiple-system-atrophy
#11
Kenji Sakai, Chiho Ishida, Yuko Kato-Motozaki, Atsuro Tagami, Kiyonobu Komai, Masahito Yamada
We describe the post mortem case of a 71-year-old Japanese woman diagnosed as having multiple system atrophy (MSA), showing somatic sprouting formation of Purkinje cells. The patient had suffered from frequent falling episodes and clumsiness of the left hand since the age of 67 years. Orthostatic hypotension and parkinsonism subsequently emerged. Typical neuropathological features of MSA, including degeneration of the striatum, pontine base and cerebellum with abundance of phosphorylated α-synuclein-positive neuronal and glial cytoplasmic and nuclear inclusions in the brain, were observed...
March 25, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29532523/clinicopathological-and-genetic-association-between-epithelioid-glioblastoma-and-pleomorphic-xanthoastrocytoma
#12
Takuya Furuta, Hiroaki Miyoshi, Satoru Komaki, Fumiko Arakawa, Motohiro Morioka, Koichi Ohshima, Mitsutoshi Nakada, Yasuo Sugita
Epithelioid glioblastoma (eGBM) is a rare variant of GBM which was adopted in the 2016 WHO classification. eGBM and pleomorphic xanthoastrocytoma (PXA) sometimes show overlapping features histologically and genetically, such as epithelioid pattern and a highly frequent V600E mutation in the gene for vRAF murine sarcoma viral oncogene homolog B1 (BRAF), respectively. Accurate diagnosis of these rare tumors is challenging according to the new criteria in the revised 2016 WHO classification. It is an urgent task to elucidate the biological properties of the tumors and to select appropriate treatment...
March 13, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29514411/embryonal-tumor-with-multilayered-rosettes-diagnosis-on-intra-operative-squash-smear
#13
Kirti Gupta, Vikram Singh, Ashish Aggarwal, Pravin Salunke
We describe a rare example of infratentorial primitive neuroectodermal tumor categorized as embryonal tumor with multilayered rosettes diagnosed on intra-operative squash smear and frozen section and discuss its key diagnostic features, pitfalls and differentials. Correct interpretation at the time of frozen section is crucial as it helps in deciding the further course of surgery. To the best of our knowledge, this is the first such report in the literature.
March 7, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29508434/an-autopsy-case-of-globular-glial-tauopathy-presenting-with-clinical-features-of-motor-neuron-disease-with-dementia-and-iron-deposition-in-the-motor-cortex
#14
Itsuki Hasegawa, Akitoshi Takeda, Hiroyuki Hatsuta, Yuki Kubo, Masahiko Ohsawa, Yuta Nakano, Takeshi Ikeuchi, Masato Hasegawa, Shigeo Murayama, Yoshiaki Itoh
Globular glial tauopathy (GGT) is a 4-repeat (4R) tauopathy in which 4R tau accumulates to form globular glial inclusions (GGIs), predominantly in oligodendroglia. To date, little has been reported on iron deposits in patients with GGT. We report a case of GGT with iron deposits in a 78-year-old woman presenting with an 8-year history of slowly progressing limb weakness and cognitive decline. Susceptibility-weighted imaging revealed a low signal intensity in the right precentral gyrus, suggesting iron deposition...
March 6, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29504169/gliosarcoma-with-primitive-neuronal-chondroid-osteoid-and-ependymal-elements
#15
Yuka Yoshida, Munenori Ide, Hiroya Fujimaki, Nozomi Matsumura, Sumihito Nobusawa, Hayato Ikota, Hideaki Yokoo
A 51-year-old man presented with a 2-week history of malaise. MRI revealed a large solid and cystic lesion with ring enhancement measuring 6.5 cm in diameter in the right frontal lobe. Histologically, the tumor consisted of various components: diffuse growth of atypical astrocytic cells consistent with glioblastoma, fascicular proliferation of atypical spindle cells such as fibrosarcoma, clusters of primitive neuronal cells, and foci of ependymal cells. The sarcomatous component also focally exhibited chondroid and osteoid differentiation...
March 5, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29502353/novel-tle4-ntrk2-fusion-in-a-ganglioglioma-identified-by-array-cgh-and-confirmed-by-ngs-potential-for-a-gene-targeted-therapy
#16
Nitya Prabhakaran, Miguel A Guzman, Pournima Navalkele, Edna Chow-Maneval, Jacqueline R Batanian
Gangliogliomas are rare neoplasms of the central nervous system that mostly originate in the temporal lobe and are associated with seizures. Literature mentions that BRAF mutations are most commonly associated with gangliogliomas. We discuss a unique case of ganglioglioma originating in the posterior fossa that showed multiple losses and a unique interstitial deletion at 9q21 by an array-comparative genome hybridization (array-CGH). The deletion led to a novel molecular fusion (TLE4-NTRK2) which was confirmed by next generation sequencing and provides a potential for a gene-targeted therapy...
March 4, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29468756/copper-deposition-in-oligodendroglial-cells-in-an-autopsied-case-of-hepatolenticular-degeneration
#17
Mayu Nishimuta, Kenta Masui, Tomoko Yamamoto, Yuichi Ikarashi, Katsutoshi Tokushige, Etsuko Hashimoto, Yoji Nagashima, Noriyuki Shibata
We present a case of hepatolenticular degeneration, so-called Wilson's disease (WD), in a 31-year-old Japanese man with broader deposition of copper in the liver, kidney and brain. The liver showed severe cirrhotic changes with macronodular pseudolobule formation, but there was little difference in immunohistochemical expression patterns of the copper transporter ATP7B between the control and present case. In the brain, there were both WD-related lesions such as the scattering of Opalski cells and changes caused by hepatic encephalopathy including the appearance of Alzheimer type II glia...
February 21, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29423960/le-hazard-ou-la-n%C3%A3-cessit%C3%A3-comorbid-pathologies-of-neurodegenerative-diseases
#18
EDITORIAL
Toshiki Uchihara, Toru Iwaki
No abstract text is available yet for this article.
February 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29266441/strictly-hypoxic-ischemic-encephalopathy
#19
LETTER
Riki Okeda
No abstract text is available yet for this article.
February 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29250829/low-bilirubin-kernicterus-in-otc-deficiency
#20
LETTER
Jon F Watchko
No abstract text is available yet for this article.
February 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
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