Neuropathology最新文献

{"title":"Diffuse Hemispheric Glioma, H3 G34-Mutant With Prominent Perivascular Invasion in a Middle-Aged Man: A Case Report and Literature Review of Middle-Aged and Elderly Cases.","authors":"Sayaka Yuzawa, Yonehiro Kanemura, Manami Hayashi, Yuki Kamikokura, Manabu Kinoshita, Mishie Tanino","doi":"10.1111/neup.70022","DOIUrl":"10.1111/neup.70022","url":null,"abstract":"<p><p>Diffuse hemispheric glioma, H3 G34-mutant (DHG), is a newly defined pediatric-type tumor in the 2021 WHO classification of central nervous system (CNS) tumors. DHGs harbor missense mutations at codon 35 of H3F3A (H3.3 G35R/V mutations) and exhibit diverse histopathological features, such as glioblastomas or CNS embryonal tumors. Regardless of histological variation, they demonstrate uniform immunohistochemical and molecular findings: Olig2 negative, ATRX loss, p53 positive, and MGMT promoter methylated. These tumors occur predominantly in the cerebral hemispheres of adolescents and young adults, while they are extremely rare in middle-aged and elderly individuals. Here, we report a middle-aged case of DHG with prominent perivascular invasion. The tumor initially demonstrated a focal glioblastoma-like area, whereas the recurrent tumor predominantly showed perivascular spread of spindle cells. We reviewed previously reported DHG cases in middle-aged and elderly patients and compared their clinicopathological features with those of adolescents and young adults.</p>","PeriodicalId":19204,"journal":{"name":"Neuropathology","volume":"45 4","pages":"e70022"},"PeriodicalIF":1.2,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12283485/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144691006","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Alzheimer's Disease With Cardiac Transthyretin Amyloidosis: A Clinicopathological Study of Autopsy Cases.","authors":"Yasuo Sugita, Takuya Furuta, Kenji Takahashi, Koichi Higaki, Yoshiro Koda, Shin-Ichiro Mori, Shoko Hongo, Hideomi Hamasaki, Akiyoshi Kakita, Mitsuharu Ueda, Keisuke Kitagawa","doi":"10.1111/neup.70011","DOIUrl":"10.1111/neup.70011","url":null,"abstract":"<p><p>The relationship between Alzheimer's disease and cardiac transthyretin amyloidosis (ATTR) has been reported epidemiologically. However, the details of its clinicopathological characteristics are unclear. To clarify the pathogenesis of Alzheimer's disease combined with cardiac ATTR, 50 autopsy cases of Alzheimer's disease with cardiac hypertrophy were examined. Transthyretin amyloid deposition was studied by immunostaining in cases where amyloid deposition was suspected in various organs by HE staining. ATTR in systemic organs was also examined. The pathological diagnosis of Alzheimer's disease was done based on the National Institute on Aging and Alzheimer's Association (NIA-AA) guidelines. Cerebral amyloid angiopathy (CAA) was rated on a 3-point scale according to the Vonsattel scale. The pathological diagnosis of cardiac ATTR was done using a 3-point scale based on previously published findings on amyloid amounts. Six out of 50 patients were found to have cardiac ATTR by immunostaining and protein mass analysis of myocardial tissue. The sex distribution of the six patients was two males (Cases 3 and 6) and four females (Cases 1, 2, 4, and 5), and their ages were 97, 89, 91, 104, 86, and 77 years in Cases 1-6, respectively. In Cases 1-6, the NIAA score/CAA assessment/ATTR stages were intermediate/severe/G3, intermediate/moderate/G3, high/severe/G3, high/severe/G2, high/severe/G2, and intermediate/moderate/G2, respectively. Cases 1-5 also had cerebral infarction. In all cases, Transthyretin amyloid deposition was seen mainly in the vessel walls of various organs throughout the body. In the heart, transthyretin amyloid deposition was observed in the myocardial vessel walls and between myocardial fibers. On autopsy, cardiogenic cerebral infarction or heart failure was considered to be the main cause of death in Cases 1-5. These results indicate that Alzheimer's disease could be regarded as a systemic disease rather than just a localized disease presenting with dementia.</p>","PeriodicalId":19204,"journal":{"name":"Neuropathology","volume":" ","pages":"e70011"},"PeriodicalIF":1.2,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144151381","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Fatal Outcome of Intravenous Thrombolysis With an Unexpected Finding of Amyloid-β-Related Angiitis-A Case Report Highlighting a Relevant Scenario With Acute Focal Neurological Deficits and Minimal Radiological Presentation.","authors":"Kristof Babarczy, Bence L Radics, Orsolya Horvath, Peter Klivenyi, Levente Szalardy","doi":"10.1111/neup.70013","DOIUrl":"10.1111/neup.70013","url":null,"abstract":"<p><p>Cerebral amyloid angiopathy (CAA) has been implicated as a risk for developing lobar intracerebral hemorrhage (ICH) after intravenous thrombolysis (IVT) applied for acute ischemic stroke (AIS). However, there is a paucity of cases reported with histopathological CAA diagnosis in this setting, with a single report to imply the role of CAA-related inflammation (CAA-RI). We report clinical, radiological, and neuropathological observations of a 65-year-old woman who presented with acute left-hemispheric symptoms with an initially unrevealing cranial computed tomography (CT) and received IVT for presumed AIS. The course was rapidly complicated by a huge lobar ICH and a fatal outcome. The autopsy revealed severe CAA, unexpectedly with transmural CAA-RI, a.k.a. amyloid-β-related angiitis (ABRA), and histopathological evidence for vascular amyloid-β phagocytosis. Re-evaluation of initial imaging did not reveal signs of asymmetric confluent white matter edema characteristic of CAA-RI, but raised the suspicion of a tiny left central convexity subarachnoid hemorrhage, a substrate of amyloid spells. The genotype of the apolipoprotein E (ApoE) gene (ApoE) was ε3/ε3. Being the second published thrombolysis-associated fatality with ABRA and among the few with definite CAA, the present case confirms CAA/CAA-RI to be a potential hidden risk for IVT-associated ICHs, urging for awareness of CAA-associated pathologies and clinical-radiological hints in an AIS setting. The findings implicate the relevance of vascular Aβ phagocytosis in the pathogenesis, confirm that CAA-RI may present without prominent edema, highlight that CAA/CAA-RI-related focal neurological deficits (including amyloid spells) can be potential AIS mimics within the IVT time window, and urge for rigorous analysis of pre-IVT CT scans for even subtle sulcal hyperdensities suggesting cSAH/amyloid spell in elderly patients, prompting consideration of magnetic resonance imaging.</p>","PeriodicalId":19204,"journal":{"name":"Neuropathology","volume":" ","pages":"e70013"},"PeriodicalIF":1.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12279614/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144234620","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Motor involvement in frontotemporal lobar degeneration with TAR DNA-binding protein of 43 kDa type C.","authors":"Rika Yamashita, Goichi Beck, Kazue Shigenobu, Airi Tarutani, Yuki Yonenobu, Makiko Kawai, Kohji Mori, Shinichiro Tahara, Yuto Satake, Yuko Saito, Eiichi Morii, Masato Hasegawa, Manabu Ikeda, Hideki Mochizuki, Shigeo Murayama","doi":"10.1111/neup.13026","DOIUrl":"10.1111/neup.13026","url":null,"abstract":"<p><p>The degeneration of pyramidal tracts has been reported in frontotemporal lobar degeneration with TDP-43 (TAR DNA-binding protein 43) pathology (FTLD-TDP) type C. Herein, we examined the detailed pathology of the primary motor area and pyramidal tracts in the central nervous system in four autopsy cases of FTLD-TDP type C, all of which were diagnosed by neuropathological, biochemical, and genomic analyses. Three patients showed right dominant atrophy of the frontal and temporal lobes, while the other patient showed left dominant atrophy. All four patients showed motor symptoms, and two patients had episodes of repeated aspiration. In the primary motor area, phosphorylated TDP-43 (p-TDP-43) or annexin A11-immunoreactive long dystrophic neurites were observed in all cases, and neuronophagia of the Betz cells was frequently observed in two of four cases. In the lower motor system, p-TDP-43 or annexin A11-positive dystrophic neurites were detected in the anterior horn of the spinal cord. Immuno-electron microscopy of the insoluble fraction extracted from all cases showed p-TDP-43 or annexin A11-labelled filaments. In FTLD-TDP type C, neurodegeneration with TDP and annexin A11 pathology was observed mainly in the upper motor neurons of both patients with right- and left predominant temporal atrophy and a short disease duration. Furthermore, a combination of TDP-43 and annexin A11 pathology was visible in the lower motor neurons, albeit less frequently. In summary, we reported the TDP-43 and annexin A11-associated involvement of anterior horn cells of the spinal cord for the first time. The degeneration of the motor system could contribute to dysphagia and aspiration pneumonia at the late stage of FTLD-TDP type C. Little or no TDP pathology was found in the corticospinal tract, unlike in FTLD-TDP type B, suggesting the occurrence of secondary degeneration in FTLD-TDP type C.</p>","PeriodicalId":19204,"journal":{"name":"Neuropathology","volume":" ","pages":"e13026"},"PeriodicalIF":1.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142984099","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Some comments on the morphometry of choroid plexus in neurodegenerative diseases.","authors":"Jean-Marie Serot, Marie Christine Bene, Gilbert Charles Faure","doi":"10.1111/neup.13032","DOIUrl":"10.1111/neup.13032","url":null,"abstract":"","PeriodicalId":19204,"journal":{"name":"Neuropathology","volume":" ","pages":"e13032"},"PeriodicalIF":1.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143441518","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Assessing Co-Localization of ITM2B With Alzheimer's Disease and Limbic-Predominant Age-Related TDP-43 Encephalopathy Neuropathologic Changes.","authors":"Ryan K Shahidehpour, Peter T Nelson, Sukanya Srinivasan, Zhong Yu, Adam D Bachstetter","doi":"10.1111/neup.70003","DOIUrl":"10.1111/neup.70003","url":null,"abstract":"<p><p>Mutations in the Integral membrane protein 2B (ITM2B) gene are linked to the development of familial British and Danish dementias, two relatively early-onset dementia disorders known also to be associated with Tau neurofibrillary tangles (NFTs). However, to date, the involvement of ITM2B in limbic-predominant age-related TDP-43 encephalopathy neuropathologic changes (LATE-NC) remains unclear. To address this question, we used brain samples from the University of Kentucky Alzheimer's Disease Research Center community-based autopsy cohort. We investigated the patterns and co-localizations of ITM2B immunohistochemistry in subiculum, CA1, CA2, CA3 and dentate gyrus of the hippocampus from brains with Alzheimer's disease neuropathologic changes (ADNC), LATE-NC, and comorbid ADNC+LATE-NC, as well as low-pathology controls (n = 4 per disease state). There was frequent co-localization between ITM2B protein and intracellular Tau pathology in ADNC; however, there was a far weaker rate of co-localization between ITM2B and TDP-43 pathology. There also was, as previously described, an association between ITM2B immunostaining and neuritic-appearing amyloid plaques. Additionally, co-localization of intracellular ITM2B pathology with Thioflavin-S in NFTs suggested a potential role for ITM2B in marking neurons undergoing transition from relatively healthy (early NFT-bearing cells) to more severely affected (later NFT-bearing) cellular disease states. This study indicates that ITM2B has a relatively specific pattern of involvement in Tau-related neurodegeneration and in neuritic amyloid plaques, while implying minimal, if any, role for ITM2B in the synergistic relationship between Tau and TDP-43 pathologies.</p>","PeriodicalId":19204,"journal":{"name":"Neuropathology","volume":" ","pages":"e70003"},"PeriodicalIF":1.2,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12309431/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143557666","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Low-Grade Primary Intramedullary Spinal Cord Astroblastoma: A Case Report and Literature Review.","authors":"Irfan Kesumayadi, Atsushi Kambe, Tetsuji Uno, Tomohiro Hosoya, Karen Makishima, Makoto Sakamoto, Kurosaki Masamichi","doi":"10.1111/neup.70016","DOIUrl":"10.1111/neup.70016","url":null,"abstract":"<p><p>Spinal astroblastoma is an exceedingly rare entity characterized by features that overlap with other spinal cord tumors. We present a case of a 67-year-old male who presented with trunk dysesthesia, motor weakness, and progressive hypoesthesia in both lower limbs. Magnetic resonance imaging (MRI) revealed edematous changes in the spinal cord at the C6-Th1 level on T2-weighted sequences, with a centrally enhancing lesion at the C7 level on gadolinium-enhanced T1-weighted imaging. Consistent with previous reports, spinal astroblastomas frequently involve the cervical and thoracic regions. Pathological examination in our case revealed pseudopapillary cellular arrangements surrounding hyalinized microvasculature. Immunohistochemical analysis demonstrated retained INI1/SMARCB1 expression and mixed-origin features, with positive staining for EMA, GFAP, OLIG2, neurofilament, and synaptophysin. The tumor exhibited low-grade characteristics, with no mitotic activity, necrosis, or significant MIB-1 index (0.3%), and followed a gradual clinical course. Genetic profiling revealed no MN1 alteration or fusion genes. Based on these findings, a diagnosis of low-grade spinal astroblastoma, not elsewhere classified, was made. In conclusion, spinal astroblastoma should be considered in the differential diagnosis of primary intramedullary spinal cord tumors, particularly those located in the cervicothoracic region and exhibiting mixed-origin features. The sharing of cases among clinicians is crucial for enhancing awareness and understanding of this rare pathology.</p>","PeriodicalId":19204,"journal":{"name":"Neuropathology","volume":" ","pages":"e70016"},"PeriodicalIF":1.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144216360","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Polymerized Type I Collagen With Polyvinylpyrrolidone Reduces Fibrosis and Improves Nerve Organization and Myelination After Peripheral Nerve Injury.","authors":"Daniel Salas-Treviño, Adolfo Soto-Domínguez, Roberto de Oca-Luna Montes, Cynthia Minerva González-Cantú, Everardo Valdés-Flores, Mauricio Manuel García-Pérez, Yanko Castro-Govea","doi":"10.1111/neup.70012","DOIUrl":"10.1111/neup.70012","url":null,"abstract":"<p><p>Peripheral nerve injuries (PNI) cause a partial or total deficit of sensory and motor function, producing neuropathic pain and loss of productivity in adults. Type I collagen polymerized with polyvinylpyrrolidone (CLG-PVP) has been used previously in other fibrosing diseases due to its regulatory effects on interleukins, cytokines, and adhesion molecules. In the present study, we describe the role of CLG-PVP in the repair of PNI. We used a murine model of PNI through axonotmesis of the sciatic nerve. CLG-PVP treatments were administered in situ and intramuscularly and were compared to a sham procedure and placebo. Histological and histochemical-specific stain evaluations were performed to describe the structural changes in nervous tissue. A significant reduction in tissue fibrosis was observed in the groups treated with CLG-PVP, especially with the intramuscular treatment. Likewise, an increase in the organization of external lamina and nerve remyelination was observed in the treated groups. In addition, a slight improvement in gait was noted in the treated animal groups at the end of the study. After peripheral nerve injury, CLG-PVP restores the nerve's function, structure, and tissue organization. These therapeutic effects were more evident through the intramuscular administration scheme with a weekly dosage. However, randomized controlled clinical trials should be performed to verify its beneficial effects and characterize adverse events.</p>","PeriodicalId":19204,"journal":{"name":"Neuropathology","volume":" ","pages":"e70012"},"PeriodicalIF":1.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144151385","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Metastatic Supratentorial Ependymoma: A Case Presentation and Systematic Review of the Literature.","authors":"Khanh Tan Tran, József Virga, Nour Kurdi, Krisztina Ajna Chalupa, Bernadett Szűcs, Álmos Klekner, Attila Mokanszki, Judit Bedekovics","doi":"10.1111/neup.70024","DOIUrl":"10.1111/neup.70024","url":null,"abstract":"<p><p>Ependymomas are categorized based on anatomical location and specific genetic alterations, with extra-axial metastasis being a rare event, occurring in less than 1% of cases and documented sparsely in the literature. This case study details a 27-year-old male patient diagnosed with supratentorial ependymoma characterized by Zinc Finger Translocation Associated (ZFTA) fusion and World Health Organization (WHO) Grade 2 morphology. Additionally, a systematic review of all reported cases of extra-axial ependymoma metastases was conducted, systematically compiling clinical, morphological, and molecular data from relevant articles. Metastatic ependymoma represents a rare occurrence characterized by diagnostic and therapeutic challenges. A comprehensive review of the literature could provide valuable insights into the underlying biology and support the selection of optimal treatment strategies for such cases.</p>","PeriodicalId":19204,"journal":{"name":"Neuropathology","volume":"45 4","pages":"e70024"},"PeriodicalIF":1.2,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12305400/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144732453","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Further discussion on choroid plexus epithelial cell changes in neurodegenerative disorders.","authors":"Nehal Revuri, Quang La","doi":"10.1111/neup.13024","DOIUrl":"10.1111/neup.13024","url":null,"abstract":"","PeriodicalId":19204,"journal":{"name":"Neuropathology","volume":" ","pages":"263-264"},"PeriodicalIF":1.3,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142872556","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}