Patient with rapidly evolving neurological disease with neuropathological lesions of Creutzfeldt ‐Jakob disease, Lewy body dementia, chronic subcortical vascular encephalopathy and meningothelial meningioma
We report a case of rapidly evolving neurological disease in a patient with neuropathological lesions of Creutzfeldt‐Jakob disease (CJD), Lewy body dementia (LBD), chronic subcortical vascular encephalopathy and meningothelial meningioma. The coexistence of severe multiple pathologies in a single patient strengthens the need to perform accurate clinical differential diagnoses in rapidly progressive dementias. (Source: Neuropathology)
Source: Neuropathology - July 31, 2016 Category: Neurology Authors: Maria Gabriella Vita, Dorina Tiple, Alessandra Bizzarro, Anna Ladogana, Elisa Colaizzo, Sabina Capellari, Marcello Rossi, Piero Parchi, Carlo Masullo, Maurizio Pocchiari Tags: Case Report Source Type: research
Cerebellar hemangioblastoma with perivascular pseudorosette formation and glial differentiation: A case report
Hemangioblastoma is a well‐circumscribed, highly vascular, lipid‐rich and low‐grade tumor of uncertain histogenesis. Its histopathological features have been well established. Herein, we present a case of cerebellar hemangioblastoma in a 43‐year‐old woman. Histologically, the tumor was predominantly composed of cellular areas showing eosinophilic or vacuolated stromal cells arranged in nests and sheets. Focally, conventional reticular areas could be seen. Additionally, in some areas, the stromal cells were arranged radially around blood vessels, exhibiting perivascular pseudorosette structures, which were similar...
Source: Neuropathology - July 31, 2016 Category: Neurology Authors: Jian Wang, Xu ‐Yong Lin, Xue‐Shan Qiu, En‐Hua Wang, Lian‐He Yang, (于涓瀚) Juan‐Han Yu Tags: Case Report Source Type: research
‘Changing colors’ of third ventricular tumor in a young woman
(Source: Neuropathology)
Source: Neuropathology - July 31, 2016 Category: Neurology Authors: Kirti Gupta, Madhivanan Karthigeyan, Narendra Kumar, Pravin Salunke Tags: Neuropathology Education Source Type: research
A novel mutation in the PDZ ‐like motif of ZASP causes distal ZASP‐related myofibrillar myopathy
We describe an autosomal dominant inherited pedigree with ZASP‐MFM that is in line with the typical phenotype of distal myopathy without cardiomyopathy and neuropathy, while mild asymmetrical muscle atrophy can be observed in some affected members. Muscle MRI revealed considerable fatty degeneration involved in the posterior compartment of thigh and lower leg, but relatively preserved in rectus femoris, sartorius, gracilis, adductor longus and biceps femoris breve muscles in the later stage. In addition, fatty infiltration of medial gastrocnemius muscle can be initiated as early as in the third decade in asymptomatic ind...
Source: Neuropathology - July 31, 2016 Category: Neurology Authors: Junjun Zheng, Shuyun Chen, Yunqing Chen, Min Zhu, Daojun Hong Tags: Original Article Source Type: research
Decrease of aquaporin ‐4 and excitatory amino acid transporter‐2 indicate astrocyte dysfunction for pathogenesis of cortical degeneration in HIV‐associated neurocognitive disorders
Human immunodeficiency virus (HIV) encephalitis and degeneration of cerebral cortex are established histopathologies of HIV‐associated neurocognitive disorders (HAND). We previously reported decreased excitatory amino acid transporter‐2 (EAAT‐2) and astrocytic apoptosis in cortical degeneration using SIVmac239 and simian‐human immunodeficiency virus (SHIV)‐infected macaques and human AIDS autopsy cases. In the present study, we added highly pathogenic SIVsm543‐3‐infected macaques. These animals showed similar degenerative changes in the frontal cortex. Using 11 SIV‐infected macaques, three SIVsm543‐3, fiv...
Source: Neuropathology - July 31, 2016 Category: Neurology Authors: Hui Qin Xing, Yu Zhang, Kimiko Izumo, Shiho Arishima, Ryuji Kubota, Xiang Ye, Qiping Xu, Kazuyasu Mori, Shuji Izumo Tags: Original Article Source Type: research
Meningeal Ewing Sarcoma/Peripheral PNET: Clinicopathological, Immunohistochemical and FISH study of four cases
This study investigates the clinical pathological features of four cases of this tumor from archives of 6 years in our hospital. Patients were within the median age of 21.5 years and male to female ratio was 1:1. The tumors distributed at the supra‐tentorial location, posterior fossa and lumbar vertebral canal, usually presenting as the dura‐sited nodule or having close connection with the meninges within the cranium or vertebral canal. Histopathologically, small round undifferentiated tumor cells with hypercellularities, scant cytoplasm and inconspicuous nucleoli were observed, although some components such as atypic...
Source: Neuropathology - July 31, 2016 Category: Neurology Authors: Changshu Ke, Qiuhong Duan, Hui Yang, Feng Zhu, Meng Yan, San ‐peng Xu, Sheng Zhou, Feng Wan, Kai Shu, Ting Lei, Li‐ming Xia Tags: Original Article Source Type: research
Familial amyotrophic lateral sclerosis with an I104F mutation in the SOD1 gene: Multisystem degeneration with neurofilamentous aggregates and SOD1 inclusions
We previously reported familial amyotrophic lateral sclerosis (FALS) of 11 years duration in a 57‐year‐old woman, who received artificial ventilation for 5 years prior to death and exhibited widespread multisystem degeneration and neurofilamentous aggregates, so‐called conglomerate inclusions (CIs). In the present study, we re‐evaluated this autopsied patient (proband) with further immunohistochemical observation as well as mutational analysis of the superoxide dismutase 1 (SOD1) gene. A review of the clinical features of the proband's family revealed five affected members (including the proband) over two success...
Source: Neuropathology - July 22, 2016 Category: Neurology Authors: Haishan Jiang, Hiroshi Shimizu, Atsushi Shiga, Masami Tanaka, Osamu Onodera, Akiyoshi Kakita, Hitoshi Takahashi Tags: Case Report Source Type: research
An autopsied case of MM1 + MM2‐cortical with thalamic‐type sporadic Creutzfeldt‐Jakob disease presenting with hyperintensities on diffusion‐weighted MRI before clinical onset
A 78‐year‐old Japanese man presented with rapidly progressive dementia and gait disturbances. Eight months before the onset of clinical symptoms, diffusion‐weighted magnetic resonance imaging (DWI) demonstrated hyperintensities in the right temporal, right parietal and left medial occipital cortices. Two weeks after symptom onset, DWI showed extensive hyperintensity in the bilateral cerebral cortex, with regions of higher brightness that existed prior to symptom onset still present. Four weeks after clinical onset, periodic sharp wave complexes were identified on an electroencephalogram. Myoclonus was observed 8 wee...
Source: Neuropathology - July 22, 2016 Category: Neurology Authors: Yasushi Iwasaki, Keiko Mori, Masumi Ito, Maya Mimuro, Tetsuyuki Kitamoto, Mari Yoshida Tags: Case Report Source Type: research
An autopsied case of MM1 + MM2‐cortical with thalamic‐type sporadic Creutzfeldt‐Jakob disease presenting with hyperintensities on diffusion‐weighted MRI before clinical onset
A 78‐year‐old Japanese man presented with rapidly progressive dementia and gait disturbances. Eight months before the onset of clinical symptoms, diffusion‐weighted magnetic resonance imaging (DWI) demonstrated hyperintensities in the right temporal, right parietal and left medial occipital cortices. Two weeks after symptom onset, DWI showed extensive hyperintensity in the bilateral cerebral cortex, with regions of higher brightness that existed prior to symptom onset still present. Four weeks after clinical onset, periodic sharp wave complexes were identified on an electroencephalogram. Myoclonus was observed 8 wee...
Source: Neuropathology - July 20, 2016 Category: Neurology Authors: Yasushi Iwasaki, Keiko Mori, Masumi Ito, Maya Mimuro, Tetsuyuki Kitamoto, Mari Yoshida Tags: Case Report Source Type: research
Effect of anti ‐cancer drugs on microglia in patient‐derived breast cancer xenografted mouse models
(Source: Neuropathology)
Source: Neuropathology - July 19, 2016 Category: Neurology Authors: Claire Paquet, Delphine Boche, Morad El Bouchtaoui, Sarah Gourmaud, Anne Janin, Guilhem Bousquet Tags: Letter to the Editor Source Type: research
Effect of anti‐cancer drugs on microglia in patient‐derived breast cancer xenografted mouse models
(Source: Neuropathology)
Source: Neuropathology - July 19, 2016 Category: Neurology Authors: Claire Paquet, Delphine Boche, Morad El Bouchtaoui, Sarah Gourmaud, Anne Janin, Guilhem Bousquet Tags: Letter to the Editor Source Type: research
Issue Information
No abstract is available for this article. (Source: Neuropathology)
Source: Neuropathology - July 18, 2016 Category: Neurology Tags: ISSUE INFORMATION Source Type: research
Cover Image
(Source: Neuropathology)
Source: Neuropathology - July 18, 2016 Category: Neurology Tags: COVER IMAGE Source Type: research
Concurrent TERT promoter and BRAF V600E mutation in epithelioid glioblastoma and concomitant low ‐grade astrocytoma
Epithelioid glioblastoma (E‐GBM) is a rare variant of glioblastoma (GBM), characterized by epithelioid cells with eosinophilic round cytoplasm devoid of stellate cytoplasmic processes. A number of studies have demonstrated that more than half of E‐GBMs harbor a valine to glutamic acid substitution at position 600 of the serine/threonine‐protein kinase BRAF (BRAF V600E). However, there are no previous reports on E‐GBM with telomerase reverse transcriptase (TERT) promoter mutation in addition to BRAF V600E mutation. Here, we report an E‐GBM case in an 18‐year‐old man with BRAF V600E and TERT promoter mutations....
Source: Neuropathology - June 14, 2016 Category: Neurology Authors: Nozomi Matsumura, Nozomi Nakajima, Tatsuya Yamazaki, Takuro Nagano, Kaie Kagoshima, Sumihito Nobusawa, Hayato Ikota, Hideaki Yokoo Tags: Case Report Source Type: research
Adult ‐onset atypical teratoid/rhabdoid tumor featuring long spindle cells with nuclear palisading and perivascular pseudorosettes
Atypical teratoid/rhabdoid tumors (AT/RTs) are rare malignant neoplasms of the CNS that preferentially affect young children. We herein report an adult case of AT/RT surviving for more than 5 years with the residual tumor. The patient, a 24‐year‐old man at onset, presented with a contrast‐enhancing mass lesion in the left occipital lobe, and underwent partial tumor resection. Histologically, the tumor was predominantly composed of long spindle cells exhibiting nuclear palisading and perivascular pseudorosettes, which appeared to mimic mesenchymal, ependymal and Schwann cell tumors. A small number of isolated rhabdoi...
Source: Neuropathology - June 13, 2016 Category: Neurology Authors: Hidehisa Horiguchi, Satoshi Nakata, Sumihito Nobusawa, Shinichi Uyama, Tadashi Miyamoto, Hiromi Ueta, Naomi Fujimoto, Hideaki Yokoo Tags: Case Report Source Type: research
