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Neuropathology: Official Journal of the Japanese Society of Neuropathology

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https://read.qxmd.com/read/30723950/atypical-globular-glial-tauopathy-with-a-combination-of-types-i-and-ii-pathology
#1
Tomoya Kon, Fumiaki Mori, Akira Arai, Yasuo Miki, Kunikazu Tanji, Hidekachi Kurotaki, Masahiko Tomiyama, Koichi Wakabayashi
Globular glial tauopathy (GGT) is a group of 4-repeat tauopathies characterized by widespread globular glial inclusions (GGIs). GGT is now classified into three subtypes based on the distribution and morphology of the GGIs. We report an autopsy case of GGT in an 85-year-old woman who presented with semantic dementia, a rare phenotype in GGT. Postmortem examination revealed marked atrophy of the frontotemporal and motor cortices and corticospinal tract degeneration with widespread occurrence of globular neurofibrillary tangles and GGIs...
February 5, 2019: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://read.qxmd.com/read/30646429/globular-glial-tauopathy-type-ii-clinicopathological-study-of-two-autopsy-cases
#2
Hidetomo Tanaka, Shinobu Kawakatsu, Yasuko Toyoshima, Takeshi Miura, Naomi Mezaki, Atsushi Mano, Kazuhiro Sanpei, Ryota Kobayashi, Hiroshi Hayashi, Koichi Otani, Takeshi Ikeuchi, Osamu Onodera, Akiyoshi Kakita, Hitoshi Takahashi
Globular glial tauopathies (GGTs) are four-repeat tauopathies characterized by the presence of two types of tau-positive globular glial inclusions (GGIs): globular oligodendrocytic and astrocytic inclusions (GOIs and GAIs). GGTs are classified into three different neuropathological subtypes: Types I, II and III. We report two patients with GGTs - a 76-year-old woman and a 70-year-old man - in whom the disease duration was 5 and 6 years, respectively. Both patients exhibited upper and lower motor neuron signs and involuntary movements, and the latter also had dementia with frontotemporal cerebral atrophy evident on magnetic resonance imaging...
January 15, 2019: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://read.qxmd.com/read/30632221/inhibition-of-enhancer-of-zest-homologue-2-is-a-potential-therapeutic-target-for-high-myc-medulloblastoma
#3
Manabu Natsumeda, Yang Liu, Satoshi Nakata, Hiroaki Miyahara, Allison Hanaford, Sama Ahsan, Duncan Stearns, Nicolas Skuli, Ulf D Kahlert, Eric H Raabe, Fausto J Rodriguez, Charles G Eberhart
MYC amplification is common in Group 3 medulloblastoma and is associated with poor survival. Group 3 and Group 4 medulloblastomas are also known to have elevated levels of histone H3-lysine 27-tri-methylation (H3K27me3), at least in part due to high expression of the H3K27 methyltransferase enhancer of zest homologue 2 (EZH2), which can be regulated by MYC. We therefore examined whether MYC expression is associated with elevated EZH2 and H3K27me3 in medulloblastoma, and if high-MYC medulloblastomas are particularly sensitive to pharmacological EZH2 blockade...
January 10, 2019: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://read.qxmd.com/read/30609184/metabolic-reprogramming-in-the-pathogenesis-of-glioma-update
#4
REVIEW
Kenta Masui, Hiromi Onizuka, Webster K Cavenee, Paul S Mischel, Noriyuki Shibata
Cancer is a genetic disease that is currently classified not only by its tissue and cell type of origin but increasingly by its molecular composition. Increasingly, tumor classification and subtyping is being performed based upon the oncogene gains, tumor suppressor losses, and associated epigenetic and transcriptional features. However, cancers, including brain tumors, are also characterized by profound alterations in cellular metabolism. At present, even though signature mutations in known metabolic enzymes are recognized as being important, the metabolic landscape of tumors is not currently incorporated into tumor diagnostic categories...
January 4, 2019: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://read.qxmd.com/read/30609132/autopsy-report-of-a-late-delayed-radiation-injury-after-a-period-of-45-years
#5
Satoshi Tanikawa, Yasutaka Kato, Mishie Tanino, Shunsuke Terasaka, Yasuo Kurokawa, Nobutaka Arai, Kazuo Nagashima, Shinya Tanaka
For delayed radiation injury, image analysis has considerably advanced, but neuropathological findings are still required to establish diagnosis. A patient who had received radiation therapy for pineal germinoma at age 14 developed neurological and psychiatric abnormalities after 15 years as a late delayed radiation injury. Autopsy at age 59 revealed diffuse changes in the white matter consisting in order of severity of myelin pallor, demyelination, and necrosis which were characterized by a lack of glial reaction...
January 4, 2019: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://read.qxmd.com/read/30592096/dexmedetomidine-protects-rats-from-postoperative-cognitive-dysfunction-via-regulating-the-gaba-b-r-mediated-camp-pka-creb-signaling-pathway
#6
Yun-Sheng Zhu, Ying-Fen Xiong, Fo-Quan Luo, Jia Min
This work attempts to discuss whether dexmedetomidine (Dex) can protect rats from postoperative cognitive dysfunction (POCD) through regulating the γ-aminobutyric acid-B receptor (GABAB R)-mediated cyclic adenosine monophosphate (cAMP) - protein kinase A (PKA) - cAMP-response element binding (cAMP-PKA-CREB) signaling pathway. Sprague-Dawley rats were divided into a non-surgical group (Control), a surgical group (Model), a surgical group treated with Dex (Model + Dex), a surgical group treated with GABAB R antagonist (Model + CGP 35348) and a surgical group treated with Dex and GABAB R agonist (Model + Dex + Baclofen)...
December 27, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://read.qxmd.com/read/30588667/malignant-progression-of-an-extraventricular-neurocytoma-arising-from-the-viiith-cranial-nerve-a-case-report-and-literature-review
#7
Yasuo Sugita, Takuya Furuta, Satoru Komaki, Koichi Ohshima, Kiyohiko Sakata, Motohiro Morioka
A rare case of extraventricular neurocytoma (EVN) arising from the VIIIth cranial nerve in a 34-year-old woman is reported. The patient had a 20-year history of hearing loss and facial palsy. Computed tomography showed a 3-cm enhancing lesion in the left cerebellopontine angle (CPA). At operation, the tumor was seen to originate from the cochlear and vestibular nerves. The tumor was subtotally resected. Histologically, the tumor consisted of uniform cells with oval to round nuclei and scant cytoplasm. Immunohistochemically, the tumor cells were positive for synaptophysin, but negative for glial fibrillary acid protein and S-100 protein...
December 26, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://read.qxmd.com/read/30536911/psychological-stress-effects-on-myelin-degradation-in-the-cuprizone-induced-model-of-demyelination
#8
Sogol Meknatkhah, Pouya Sharif Dashti, Monireh-Sadat Mousavi, Amirbahador Zeynali, Shahin Ahmadian, Saeed Karima, Ali Akbar Saboury, Gholam Hossein Riazi
Multiple sclerosis (MS) is known as the most common demyelinating disease worldwide in which previous studies have shown that stress is a risk factor for the disease's onset and progression. Nevertheless, further studies are needed to investigate the consequences of stress in MS pathology. In this study, after 5 days of exposure to psychological and physical stress as a repetitive distress modality, rats were treated with cuprizone. The demyelination degree was compared in animal groups using Luxol fast blue staining, immunohistochemical staining for myelin basic protein and transmission electron microscopy...
December 10, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://read.qxmd.com/read/30511495/high-p-mtor-expression-is-associated-with-recurrence-and-shorter-disease-free-survival-in-atypical-meningiomas
#9
Valeria Barresi, Simona Lionti, Lilli La Rocca, Samuel Caliri, Maria Caffo
Due to their widely variable clinical behavior, the post-surgical treatment of atypical meningiomas is controversial. Therefore, prognostic factors able to identify high-risk cases, which may benefit from adjuvant treatments, are warranted. Mammalian target of rapamycin (mTOR) belongs to the PI3K-AKT pathway. Its phosphorylated form (p-mTOR Ser2448) is involved in cell growth, differentiation and tumorigenesis. The aim of this study was to evaluate p-mTOR Ser2448 expression and its eventual correlation with clinicopathological features, recurrence, or disease-free survival (DFS), in atypical meningiomas...
December 3, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://read.qxmd.com/read/30511425/an-autopsy-case-of-progressive-multifocal-leukoencephalopathy-after-rituximab-therapy-for-malignant-lymphoma
#10
Reiji Muto, Yasuo Sugita, Seiya Momosaki, Yuriko Ito, Yoshiyuki Wakugawa, Koichi Ohshima
Progressive multifocal leukoencephalopathy (PML) is a rare fatal demyelinating disease of the central nervous system caused by reactivation of the JC virus (JCV), which is named after the initials of the patient from whom the virus was first isolated. JCV is highly prevalent worldwide, infects humans in early childhood, and the infection persists throughout the course of life in latent form. The present paper deals with the second autopsy case report of rituximab-associated PML in Japan. A 63-year-old woman who had undergone chemotherapy for non-Hodgkin lymphoma developed progressive dysarthria and cerebellar ataxia...
December 3, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://read.qxmd.com/read/30511354/amyotrophic-lateral-sclerosis-of-long-clinical-course-clinically-presenting-with-progressive-muscular-atrophy
#11
Tomoyasu Matsubara, Masaya Oda, Tetsuya Takahashi, Chigusa Watanabe, Yoshiro Tachiyama, Hiroyuki Morino, Hideshi Kawakami, Ryuji Kaji, Hirofumi Maruyama, Shigeo Murayama, Yuishin Izumi
Amyotrophic lateral sclerosis (ALS) primarily affects upper and lower motor neurons. Phosphorylated trans-activation response DNA-binding protein of 43 kDa (TDP-43) inclusion bodies are reportedly a pathological hallmark of sporadic ALS. Here, we present an atypical case of sporadic ALS that progressed very slowly, persisted for 19 years, and clinically appeared to only affect the lower motor neurons; however, upper motor neuron degeneration was detected at autopsy. Furthermore, no inclusion bodies positive for phosphorylated TDP-43, ubiquitin, fused in sarcoma, or superoxide dismutase-1 were detected in the central nervous system...
December 3, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://read.qxmd.com/read/30474266/brain-invasion-by-chronic-lymphocytic-leukemia
#12
Ryohei Otani, Takeo Uzuka, Hadzki Matsuda, Fumi Higuchi, Phyo Kim, Keisuke Ueki
Brain invasion by chronic lymphocytic leukemia (CLL) is very rare, and only a handful of cases have been reported. We here report a case of 61-year-old woman who had been treated for CLL for 14 years presenting with a progressive mental disturbance. Magnetic resonance imaging (MRI) showed discontinuous ring-enhancing lesions compatible with the "open ring" sign, which was considered a demyelinating disorder, in both the frontal lobes. However, on histological examination of the biopsied specimen, infiltration of small lymphocytes positive for CD5, CD20, and CD23, indicating brain invasion by CLL, was seen...
November 25, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://read.qxmd.com/read/30557911/braf-v600e-mutated-central-nervous-system-tumor-with-divergent-morphological-feature-anaplastic-pleomorphic-xanthoastrocytoma-like-and-astroblastoma-like
#13
Suvendu Purkait, Sumit Bansal, Prit B Malgulwar
Mutational analysis of the BRAF gene (BRAF), especially BRAF V600E, is gaining much importance in neuro-oncology practice due to its diagnostic, prognostic and therapeutic implications. This genetic alteration has been described in a wide morphological spectrum of central nervous system tumors. In the present report we describe a BRAF V600E-mutated tumor with divergent morphological appearance comprising of anaplastic pleomorphic xanthoastrocytoma and astroblastoma. Both of these tumor entities are extremely rare and a combined morphology has not been described till now...
February 2019: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://read.qxmd.com/read/30537380/2018-japanese-neuropathology-prizes
#14
(no author information available yet)
No abstract text is available yet for this article.
December 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://read.qxmd.com/read/30537379/reviewers
#15
(no author information available yet)
No abstract text is available yet for this article.
December 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://read.qxmd.com/read/30430658/intracranial-vascular-calcification-with-extensive-white-matter-changes-in-an-autopsy-case-of-pseudopseudohypoparathyroidism
#16
Tamaki Iwase, Mari Yoshida, Yoshio Hashizume, Ikuru Yazawa, Seishiro Takahashi, Takashi Ando, Toshimasa Ikeda, Kazuya Nokura
We herein report an autopsy case of a 69-year-old man with pseudopseudohypoparathyroidism. The patient suffered from mental retardation and spastic tetraparesis and had all the features of Albright's hereditary osteodystrophy with a normal response to parathyroid hormone in the Ellsworth-Howard test. Computed tomography demonstrated symmetrical massive brain calcification involving the bilateral basal ganglia, thalami, dentate nuclei and cerebral gray/white matter junctions, which was consistent with Fahr's syndrome...
November 14, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://read.qxmd.com/read/30411407/autopsied-centenarian-case-of-alzheimer-s-disease-combined-with-hippocampal-sclerosis-tdp-43-and-%C3%AE-synuclein-pathologies
#17
Yasushi Iwasaki, Akira Deguchi, Keiko Mori, Masumi Ito, Yoshinari Kawai, Akio Akagi, Maya Mimuro, Hiroaki Miyahara, Mari Yoshida
A Japanese woman showed slowly progressive memory disturbance starting at the age of 84 years, and disorientation gradually appeared. Head computed tomography revealed severe hippocampal atrophy, whereas the atrophy of the frontal lobe was considerably mild for her age. Behavioral and psychological symptoms of dementia were relatively inconspicuous during the disease course. Apolipoprotein E gene analysis showed ε3/ε4 heterozygosity. She died at the age of 100 years and she was clinically diagnosed as having Alzheimer's disease (AD)...
November 9, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://read.qxmd.com/read/30411412/hydroxychloroquine-induced-autophagic-vacuolar-myopathy-with-mitochondrial-abnormalities
#18
Shaweta Khosa, Negar Khanlou, Gurveer S Khosa, Shri K Mishra
Hydroxychloroquine (HCQ) and chloroquine are used worldwide for malaria as well as connective and rheumatological disorders. They have been reported to be linked to myopathy in patients. We report four patients who were receiving HCQ as part of treatment for connective tissue disorder and who presented with myopathy. The muscle biopsy in these patients was consistent with findings of HCQ toxicity. HCQ muscle toxicity is usually self-limiting after discontinuation of the drug. It also usually tends to be under-reported due to presence of various confounding factors...
November 8, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://read.qxmd.com/read/30402942/nuclear-abnormalities-in-vascular-myocytes-in-cerebral-autosomal-dominant-arteriopathy-with-subcortical-infarcts-and-leukoencephalopathy-cadasil
#19
Dorota Dziewulska, Ewelina Nycz, Cecylia Rajczewska-Oleszkiewicz, Jacek Bojakowski, Dorota Sulejczak
Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a stroke and dementia syndrome with degeneration and loss of vascular smooth muscle cells (VSMCs). The disease is due to mutations in NOTCH3 playing an important role in VSMC differentiation, proliferation and apoptosis. Searching for a possible cause of VSMC dysfunction in CADASIL, we investigated morphology and proliferative activity the affected myocytes. In material from autopsy brains and skin-muscle biopsies of patients with CADASIL diagnosis, assessment of VSMCs in arterial vessels at the level of light and electron microscopy was performed...
November 6, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://read.qxmd.com/read/30318820/distinct-microglia-profile-in-creutzfeldt-jakob-disease-and-alzheimer-s-disease-is-independent-of-disease-kinetics
#20
Katarina Krbot, Peter Hermann, Magdalena Krbot Skorić, Inga Zerr, Diego Sepulveda-Falla, Stefan Goebel, Jakob Matschke, Susanne Krasemann, Markus Glatzel
Activated microglia represent a common pathological feature of neurodegenerative diseases. Sporadic Creutzfeldt-Jakob disease (sCJD) patients show more pronounced microglial activation than Alzheimer's disease (AD) patients. Whether these differences are due to differences in disease kinetics or represent disease-specific changes is unknown. We investigated microglial phenotypes in brains of rapidly progressive AD (rpAD) and sCJD patients matched for clinical presentation, including disease duration. We immunostained the frontal cortex, basal ganglia and cerebellum in 16 patients with rpAD and sCJD using antibodies against markers of microglia and recruited monocytes (ionized calcium-binding adaptor molecule 1, human leukocyte antigen DPQR, Cluster of Differentiation 68), an antibody unique to brain-resident microglia (transmembrane protein 119 (TMEM119)), in addition to antibodies against a marker of astrocytes (glial fibrillary acidic protein), amyloid-β (Aβ) and pathological prion protein...
October 15, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
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