Frontotemporal dementia with trans ‐activation response DNA‐binding protein 43 presenting with catatonic syndrome
Catatonia is a clinical syndrome characterized by symptoms such as immobility, mutism, stupor, stereotypy, echophenomena, catalepsy, automatic obedience, posturing, negativism, gegenhalten and ambitendency. This syndrome occurs mostly in mood disorder and schizophrenic patients, and is related to neuronal dysfunction involving the frontal lobe. Some cases of frontotemporal dementia (FTD) with catatonia have been reported, but these cases were not examined by autopsy. Here, we report on a FTD case which showed catatonia after the first episode of brief psychotic disorder. At the age of 58, the patient had a sudden onset of ...
Source: Neuropathology - October 1, 2017 Category: Neurology Authors: Ryohei Watanabe, Ito Kawakami, Mitsumoto Onaya, Shinji Higashi, Nobutaka Arai, Haruhiko Akiyama, Masato Hasegawa, Tetsuaki Arai Tags: Case Report Source Type: research
Frontotemporal lobar degeneration due to P301L tau mutation showing apathy and severe frontal atrophy but lacking other behavioral changes: A case report and literature review
The clinical features in cases that have mutations in the microtubule‐associated protein tau gene but lack prominent behavioral changes remain unclear. Here, we describe detailed clinical and pathological features of a case carrying the P301L tau mutation that showed only apathy until the middle stage of the course. The mother of this case was suspected to have mild cognitive decline at age 46. However, before she was fully examined, she had a subarachnoid hemorrhage at age 49 and died at age 53. An autopsy was not done. The proband of this pedigree, a 60‐year‐old right‐handed Japanese man at the time of death, beg...
Source: Neuropathology - October 1, 2017 Category: Neurology Authors: Tomoko Miki, Osamu Yokota, Shintaro Takenoshita, Yoko Mori, Kiyohiro Yamazaki, Yuki Ozaki, Shu ‐ichi Ueno, Takashi Haraguchi, Hideki Ishizu, Shigetoshi Kuroda, Seishi Terada, Norihito Yamada Tags: Case Report Source Type: research
The perivascular microenvironment in Epstein –Barr virus positive primary central nervous system lymphoma: The role of programmed cell death 1 and programmed cell death ligand 1
It has been shown that high expression of certain immune checkpoint molecules, including those of the programmed death protein 1/programmed death ligand 1 (PD‐1/PD‐L1) axis, can be utilized to regulate immunosuppression in the microenvironment of malignant neoplasms. For the purpose of clarifying the immune‐escape mechanism of primary central nervous system lymphomas (PCNSLs), particularly in Epstein–Barr virus (EBV)‐positive cases, markers for PD‐1, PD‐L1, tumor‐associated macrophages (TAMs), and tumor‐infiltrating lymphocytes (TILs) in 39 surgical specimens of PCNSLs (17 EBV‐positive, 22 EBV‐negativ...
Source: Neuropathology - October 1, 2017 Category: Neurology Authors: Yasuo Sugita, Takuya Furuta, Koichi Ohshima, Satoru Komaki, Junko Miyoshi, Motohiro Morioka, Hideyuki Abe, Takanori Nozawa, Yukihiko Fujii, Hitoshi Takahashi, Akiyoshi Kakita Tags: Original Article Source Type: research
Primary central nervous system extranodal nasal ‐type natural killer/T‐cell lymphoma with CD20 expression
We report a unique case of primary CNS extranodal natural killer/T‐cell lymphoma (PCNS ENKTCL) with CD20 expression and the monoclonal rearrangement of Ig heavey chain (IgH) gene. Resection specimens were evaluated using HE‐stained sections, immunohistochemistry, in situ hybridization and PCR. Histopathologic examination, immunohistochemistry and molecular studies showed the intermediate‐sized lymphoma cells expressing CD2, CD3ε, granzyme B, TIA‐1, CD20 and Epstein–Barr virus‐encoded RNA, with germline T‐cell receptor gene and the monoclonal rearrangement of IgH gene. Clinicopathologic and radiographic evalu...
Source: Neuropathology - October 1, 2017 Category: Neurology Authors: Dujuan Li, Fangfang Fu, Lifei Lian Tags: Case Report Source Type: research
Combined morphological, immunohistochemical and genetic analyses of medulloepithelioma in the posterior cranial fossa
We report a case of medulloepithelioma in the posterior cranial fossa that was diagnosed by both morphological and genetic analyses based on this classification. A 10‐month‐old girl was admitted to our hospital with consciousness disturbance and vomiting. Neuroimaging revealed a partially calcified mass and cyst formation in the posterior cranial fossa. Partial resection of the tumor was performed and histological findings revealed multilayered rosettes with LIN28A staining, but genetic analysis showed no amplification of the C19MC microRNA cluster at 19q14.32. Therefore, we diagnosed the tumor as medulloepithelioma be...
Source: Neuropathology - October 1, 2017 Category: Neurology Authors: Kosuke Kusakabe, Shohei Kohno, Akihiro Inoue, Toshimoto Seno, Sachiko Yonezawa, Kyoko Moritani, Yosuke Mizuno, Mie Kurata, Riko Kitazawa, Hisamichi Tauchi, Hideaki Watanabe, Shinji Iwata, Junko Hirato, Takeharu Kunieda Tags: Case Report Source Type: research
Issue Information
(Source: Neuropathology)
Source: Neuropathology - October 1, 2017 Category: Neurology Tags: Issue Information Source Type: research
Cover Image
(Source: Neuropathology)
Source: Neuropathology - October 1, 2017 Category: Neurology Tags: Cover Image Source Type: research
Evidence that glial cells attenuate G47R transthyretin accumulation in the central nervous system
Neuropathology,Volume 38, Issue 1, Page 11-21, February 2018. (Source: Neuropathology)
Source: Neuropathology - September 28, 2017 Category: Neurology Source Type: research
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Neuropathology,Volume 38, Issue 1, Page 11-21, February 2018. (Source: Neuropathology)
Source: Neuropathology - September 28, 2017 Category: Neurology Source Type: research
Possible concurrence of TDP ‐43, tau and other proteins in amyotrophic lateral sclerosis/frontotemporal lobar degeneration
Neuropathology,Volume 38, Issue 1, Page 72-81, February 2018. (Source: Neuropathology)
Source: Neuropathology - September 27, 2017 Category: Neurology Source Type: research
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Neuropathology,Volume 38, Issue 1, Page 72-81, February 2018. (Source: Neuropathology)
Source: Neuropathology - September 27, 2017 Category: Neurology Source Type: research
Molecular mechanisms of the co ‐deposition of multiple pathological proteins in neurodegenerative diseases
Neuropathology,Volume 38, Issue 1, Page 64-71, February 2018. (Source: Neuropathology)
Source: Neuropathology - September 25, 2017 Category: Neurology Source Type: research
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Neuropathology,Volume 38, Issue 1, Page 64-71, February 2018. (Source: Neuropathology)
Source: Neuropathology - September 25, 2017 Category: Neurology Source Type: research
Pathological and immunoblot analysis of phosphorylated TDP ‐43 in sporadic amyotrophic lateral sclerosis with pallido‐nigro‐luysian degeneration
Neuropathology, EarlyView. (Source: Neuropathology)
Source: Neuropathology - September 14, 2017 Category: Neurology Source Type: research
Neuropathological comorbidity associated with argyrophilic grain disease
Neuropathology,Volume 38, Issue 1, Page 82-97, February 2018. (Source: Neuropathology)
Source: Neuropathology - September 14, 2017 Category: Neurology Source Type: research
