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Neuropathology: Official Journal of the Japanese Society of Neuropathology

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https://www.readbyqxmd.com/read/28419566/perirhinal-accumulation-of-neuronal-alpha-synuclein-in-a-multiple-system-atrophy-patient-with-dementia
#1
Mari Saito, Makoto Hara, Momoko Ebashi, Akihiko Morita, Kyoko Okada, Taku Homma, Masahiko Sugitani, Kentaro Endo, Toshiki Uchihara, Satoshi Kamei
We report the case of a 79-year-old Japanese woman who developed cerebellar ataxia followed by rigidity, dysautonomia and cognitive disorders, and was thus clinically diagnosed as having possible MSA with dementia. Neuropathological findings demonstrated not only olivopontocerebellar and striatonigral degeneration with frequent glial cytoplasmic inclusions (GCIs), but also degenerative changes in the parahippocampal region, accentuated in the anterior portion of perirhinal cortex, where neuronal cytoplasmic inclusions (NCIs) and NFTs were numerous while GCIs were limited...
April 16, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28402042/mm1-type-sporadic-creutzfeldt-jakob-disease-with-1-month-total-disease-duration-and-early-pathologic-indicators
#2
Yasushi Iwasaki, Hiroko Kato, Tetsuo Ando, Maya Mimuro, Tetsuyuki Kitamoto, Mari Yoshida
A 62-year-old man presented with abnormal behavior and cognitive impairment. Diffusion-weighted images (DWI) obtained on MRI showed extensive hyperintense regions in the cerebral cortex and striatum. Myoclonus was recognized, and the patient died 1 month after the onset; his condition did not reach the akinetic mutism state. The brain weighed 1300 g and showed no apparent atrophy. Extensive spongiform changes were observed in the cerebral neocortex, striatum, thalamus and cerebellar cortex, but gliosis was mild or absent...
April 12, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28397378/basal-subarachnoid-hemorrhage-by-rupture-of-arteriovenous-malformation-at-the-cerebellopontine-angle
#3
Mio Takayama, Masayuki Kashiwagi, Kenji Hara, Aya Matsusue, Brian Waters, Natsuki Ikematsu, Shin-Ichi Kubo
A man in his late forties had lived as a recluse for more than ten years. He was found dead in his room. At autopsy, subarachnoid hemorrhage (SAH) was detected at the base of the brain, which weighed 1333 g. The cerebellar tonsil was swollen. The cerebral ventricle was enlarged and filled with blood. A hematoma was observed in the upper part of the left side of the cerebellar hemisphere. The location and size of SAH in this case indicated that the rupture of a cerebral aneurysm (CA) had occurred; however, CA was not detected...
April 10, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28387450/severe-demyelination-in-a-patient-with-a-late-infantile-form-of-niemann-pick-disease-type-c
#4
Tsuyoshi Kodachi, Shizuko Matsumoto, Masashi Mizuguchi, Hitoshi Osaka, Nobuyuki Kanai, Eiji Nanba, Kousaku Ohno, Takanori Yamagata
Niemann-Pick disease type C (NPC) is a cholesterol storage disease caused by defective cellular cholesterol transportation. The onset and progression of NPC are variable, and autopsy findings have mainly been reported for the adult and juvenile forms of this disease. Here we report the clinical and pathological findings from a 9-year-old female patient with the late infantile form of NPC due to NPC1 gene mutation. She had notable splenomegaly at 4 months of age. She lost the ability to speak at 18 months of age...
April 7, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28387433/neurosurgical-management-and-pathology-of-lumbosacral-lipomas-with-tethered-cord
#5
REVIEW
Takato Morioka, Nobuya Murakami, Takafumi Shimogawa, Nobutaka Mukae, Kimiakai Hashiguchi, Satoshi O Suzuki, Koji Iihara
Lumbosacral lipomas are the most common form of occult spinal dysraphism. The development of lumbosacral lipomas is from the premature disjunction of the neural tube from the surrounding ectoderm, leaving the neural plate open posteriorly and allowing for the infiltration of mesodermal tissue, including fatty tissue. Since lumbosacral lipomas are a common cause of spinal cord tethering that can lead to progressive neurological deficits, prophylactic neurosurgery for lumbosacral lipomas, including untethering of the spinal cord, is recommended...
April 7, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28387066/calcifying-pseudoneoplasm-of-the-neuraxis-in-direct-continuity-with-a-low-grade-glioma-a-case-report-and-review-of-the-literature
#6
Nayuta Higa, Hideaki Yokoo, Hirofumi Hirano, Hajime Yonezawa, Tatsuki Oyoshi, Yuko Goto, Kazunori Arita
Calcifying pseudoneoplasms of the neuraxis (CAPNON) are presumed to be a non-neoplastic reactive pathology, based on the frequent finding of granulomatous inflammation. To our knowledge, there are few reports of CAPNON in association with a neoplasm. Here, we report the case of a 62-year-old man presenting with headache, which was caused by CAPNON in the left cingulate gyrus. CT scan revealed a calcified mass exhibiting gradual growth and increasing peritumoral edema. MRI showed an intra-axial hypointense mass on T1- and T2-weighted images...
April 7, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28317291/intravascular-t-cell-lymphoma-a-rare-poorly-characterized-entity-with-cytotoxic-phenotype
#7
Tara L Sharma, Gabrielle A Yeaney, Payam Soltanzadeh, Yuebing Li, Claudiu V Cotta
Intravascular T-cell lymphomas are rare, poorly characterized lesions. We discuss the clinical, radiologic and especially the laboratory characteristics of a lesion which presented in a 62-year-old woman with a history of progressive CNS abnormalities. Throughout the course of the disease, radiologic findings consisted mainly of multifocal mixed areas of ischemia and vasogenic edema involving cortical and subcortical regions. A brain biopsy identified an abnormal T-cell population confined to lumens of vessels...
March 20, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28317173/extracellular-matrix-in-the-cns-induced-by-neuropathogenic-viral-infection
#8
Rihito Watanabe, Masatoshi Kakizaki
During the early phase of infection with an extremely neurovirulent murine coronavirus, cl-2, the ER-TR7 antigen (ERag)-positive fibers (ERfibs) associated with laminin and collagen III show a rapid increase in expression levels in the meninges, followed by an appearance of the antigens in the ventricle and brain parenchyma. Then, cl-2 invades the ventricle and ventricular wall along the newly assembled ERfibs after infection, using them as a pathway from the meninges, the initial site of infection. In the lymph nodes and spleen, ERag is mainly produced by fibroblastic reticular cells (FRCs), which play a key role in nursing the ERfibs to form a fibroblastic reticular network (FRN)...
March 20, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28295636/t-cell-lymphoblastic-lymphoma-leukemia-within-an-adrenocorticotropic-hormone-and-thyroid-stimulating-hormone-positive-pituitary-adenoma-a-cytohistological-correlation-emphasizing-importance-of-intra-operative-squash-smear
#9
Rakesh K Gupta, Ravindra K Saran, Arvind K Srivastava, Anita Jagetia, Lalit Garg, Mehar C Sharma
We present a rare case of primary pituitary T cell lymphoma/leukemia (T-LBL) in association with adrenocorticotropic hormone (ACTH) and thyroid stimulating hormone (TSH) expressing pituitary adenoma in a 55-year-old woman highlighting the importance of intra-operative squash smears examination. The patient presented with complaints of headache, diminution of vision and recent onset altered sensorium. MRI revealed a mass lesion in the sellar-suprasellar region with non-visualization of pituitary gland separately, extending to involve adjacent structures diagnosed as invasive pituitary macroadenoma...
March 13, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28276157/pigmented-intraventricular-tumor-in-a-young-adult
#10
Kirti Gupta, Balan Louis Gaspar, Pravin Salunke, Charan Singh Rayat
No abstract text is available yet for this article.
March 9, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28276095/a-case-of-symptomatic-granular-cell-tumor-of-the-sellar-region-with-large-calcification
#11
Shun Yamamuro, Taku Homma, Yuya Hanashima, Sodai Yoshimura, Hideki Oshima, Sumie Ohni, Yoshiaki Kusumi, Atsuo Yoshino
We report here a rare case of symptomatic granular cell tumor (GCT) of the sellar region with a large calcification. A 70-year-old woman presented with a sellar mass, accompanied by bitemporal hemianopia. The patient was diagnosed preoperatively as having a craniopharyngioma or a pituitary adenoma, because of the large calcification. The patient underwent surgical tumor resection via endoscopic trans-sphenoidal surgery and was diagnosed pathologically as having GCT. The patient's postoperative course was uneventful and her visual field disturbance improved soon after the operation...
March 9, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28276094/pathology-of-toxic-leucoencephalopathy-in-drug-abuse-supports-hypoxic-ischemic-pathophysiology-etiology
#12
Murad Alturkustani, Lee-Cyn Ang, David Ramsay
The histopathological features of leucoencephalopathy caused by illicit drugs (such as opioids and cocaine) are well documented in acute cases but not in long-survival cases. There are several hypotheses about the pathogenesis of this disorder, including hypoperfusion, direct drug toxicity resulting from the neurotoxic effects of the drug itself or contaminants in the illicit drug vehicle. We reviewed the post mortem findings in five males (aged 24 to 56 years, with survival intervals ranging from 7 days to 5 months) with a history of illicit drug use and concomitant fatal white matter changes...
March 9, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28261874/samp8-mice-as-a-neuropathological-model-of-accelerated-brain-aging-and-dementia-toshio-takeda-s-legacy-and-future-directions
#13
REVIEW
Ichiro Akiguchi, Mercè Pallàs, Herbert Budka, Haruhiko Akiyama, Masaki Ueno, Jingxian Han, Hideo Yagi, Tomohumi Nishikawa, Yoichi Chiba, Hiroshi Sugiyama, Ryoya Takahashi, Keiko Unno, Keiichi Higuchi, Masanori Hosokawa
Senescence accelerated mice P8 (SAMP8) show significant age-related deteriorations in memory and learning ability in accordance with early onset and rapid advancement of senescence. Brains of SAMP8 mice reveal an age-associated increase of PAS-positive granular structures in the hippocampal formation and astrogliosis in the brain stem and hippocampus. A spongy degeneration in the brain stem appears at 1 month of age and reaches a maximum at 4-8 months. In addition, clusters of activated microglia also appear around the vacuoles in the brain stem...
March 6, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28261869/intraventricular-gliosarcoma-with-dual-sarcomatous-differentiation-a-unique-case
#14
Rajalakshmi Poyuran, Nandeesh Bn, Yerasi Varun Kumar Reddy, Amey R Savardekar
Gliosarcoma, a variant of isocitrate dehydrogenase-wildtype glioblastoma, is largely a lobar surfacing neoplasm often with dural attachment. In this biphasic neoplasm, the sarcomatous component usually takes the form of fibrosarcoma or malignant fibrous histiocytoma. Heterologous sarcomatous differentiation is a rare phenomenon. Here, we present a case of gliosarcoma with liposarcomatous and myosarcomatous differentiation in a 68-year-old man which was purely intraventricular. This is the first report of such a morphologic pattern in this location...
March 6, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28244166/%C3%AE-synuclein-astrogliopathy-a-possible-specific-feature-in-%C3%AE-synucleinopathy
#15
LETTER
Shunsuke Koga, Koji Kasanuki, Dennis W Dickson
No abstract text is available yet for this article.
February 27, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28185325/a-case-of-high-grade-astrocytoma-with-braf-and-atrx-mutations-following-a-long-standing-course-over-two-decades
#16
Satoshi Nakata 中田 聡, Keishi Horiguchi, Shogo Ishiuchi, Yuhei Yoshimoto, Seiji Yamada, Sumihito Nobusawa, Hayato Ikota, Junko Hirato, Hideaki Yokoo
Pediatric high-grade gliomas are rare and occasionally hard to classify. These tumors often feature a well-demarcated histology and are expected to have a better outcome than ordinary diffuse high-grade gliomas in adults. We herein report a case of circumscribed high-grade glioma that showed a distinct molecular profile and followed an excellent course for 26 years. The patient, a 3-year-old boy at onset, presented with a contrast-enhancing mass in the right temporal lobe and underwent resection. Histologically, the tumor mainly consisted of compact bundles of GFAP-positive spindle cells...
February 9, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28168741/comparison-of-at8-immunoreactivity-in-the-locus-ceruleus-and-hippocampus-of-154-brains-from-routine-autopsies
#17
Koichi Okamoto, Masakuni Amari, Toshio Fukuda, Keiji Suzuki, Masamitsu Takatama
We compared semiquantitatively AT8 immunoreactivity in the locus ceruleus (LC) and hippocampus of 154 brains from routine autopsies to investigate the initial sites of phosphorylated tau (phospho-tau) development. The numbers of AT8-positive neurons and the severity of AT8-positive neuropil threads (NTs) in the LC were strongly associated: there were no cases with AT8-positive neurons that lacked NTs and 20 cases (13%) had only NTs in the LC. Phospho-tau pathologies in the LC were almost equally on both sides, although some cases (7...
February 6, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28168739/intracranial-neuromuscular-choristoma-report-of-a-case-with-literature-review
#18
Antonella Coli, Mariangela Novello, Gianpiero Tamburrini, Manila Antonelli, Felice Giangaspero, Libero Lauriola
Neuromuscular choristoma (NMC), also called neuromuscular hamartoma or nerve rhabdomyoma, is a rare lesion of the spinal and cranial nerves composed of skeletal muscle intimately associated with nerve fibers. Its origin has not been precisely clarified and a malformative event, resulting from aberrant differentiation or a true neoplastic growth, have been proposed by authors. We hereby present a cerebellopontine angle NMC enlarging the eighth cranial nerve in a 3-year-old child, that histologically appeared composed of a large amount of striated muscle mixed with nerve fibers...
February 6, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28144989/dentatorubral-pallidoluysian-atrophy-drpla-with-a-small-ganglioglioma-component-containing-neurofibrillary-tangles-and-polyglutamine-aggregation
#19
Seiji Yamada, Tatsuya Yamazaki, Satoshi Nakata, Sumihito Nobusawa, Hayato Ikota, Munenori Ide, Kazuyuki Mizushima, Yasuo Harigaya, Junko Hirato, Hideaki Yokoo
Dentatorubral-pallidoluysian atrophy (DRPLA), one of the polyglutamine diseases, has not been reported in combination with ganglioglioma (GG). Herein, we report an autopsy case of a 72-year-old man with DRPLA with a small GG component harboring neurofibrillary tangles (NFTs) and polyglutamine aggregates. NFTs, cytoplasmic accumulations of hyper-phosphorylated tau, are mainly observed in Alzheimer's disease (AD) and other tau-associated neurodegenerative disorders. NFTs can also be present in normal aging, and are occasionally observed in low-grade central nervous system (CNS) neoplasms such as GG...
January 31, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28139865/silencing-of-otub1-inhibits-migration-of-human-glioma-cells-in-vitro
#20
Li Xu, Jinquan Li, Zhen Bao, Peng Xu, Hao Chang, Jingjing Wu, Yuanqi Bei, Liuwan Xia, Peizhang Wu, Ke Yan, Bing Lu, Gang Cui
OTU domain-containing ubiquitin aldehyde-binding protein 1 (OTUB1) protein, a deubiquitinating enzyme (DUB) which belongs to the ovarian tumor (OTU) family, was reported to be associated with the development of various malignancies. However, the potential function of OTUB1 in human gliomas was still unclear. In this study, we sought to investigate the function of OTUB1 in the pathological process of gliomas and analyze its related clinical significance. Western blot and immunohistochemistry analyses demonstrated that OTUB1 was overexpressed in glioma tissues, and statistical analysis suggested the expression level of OTUB1 was significantly correlated with the WHO grades of human gliomas (P < 0...
January 31, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
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