Journal of Neuropathology and Experimental Neurology最新文献

Molecular characterization of benign intracranial glioependymal and arachnoid cysts suggest heterogeneous mechanisms of action.

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-04-08 DOI: 10.1093/jnen/nlaf029

Stuart D Harper, Travis Perryman, Lindsey A Dudley, Ashna S Prabhu, Amani Carson, Alondra Delgadillo, Eliana S Oduro, Othneil Sparks, Aidan Gor, Shivani Baisiwala, Ting Zhang, Fausto Rodriguez, Kunal S Patel

{"title":"Molecular characterization of benign intracranial glioependymal and arachnoid cysts suggest heterogeneous mechanisms of action.","authors":"Stuart D Harper, Travis Perryman, Lindsey A Dudley, Ashna S Prabhu, Amani Carson, Alondra Delgadillo, Eliana S Oduro, Othneil Sparks, Aidan Gor, Shivani Baisiwala, Ting Zhang, Fausto Rodriguez, Kunal S Patel","doi":"10.1093/jnen/nlaf029","DOIUrl":"https://doi.org/10.1093/jnen/nlaf029","url":null,"abstract":"Benign-appearing intracranial cysts may become symptomatic due to mass effect and require surgical treatment. Mechanisms underlying cyst formation and growth remain poorly understood. This study identified 16 patients who underwent surgical treatment for benign intracranial cysts. Cyst wall pathologic samples (n = 8) were characterized as arachnoid or glioependymal cysts using H&E and immunofluorescence staining. Five samples (62.5%) were found to be glioependymal while three (37.5%) were arachnoid cysts. Cyst fluid examined in 4 cases resembled cerebrospinal fluid (CSF) and showed no significant differences in composition between pathological subtypes. Single-cell sequencing and RNA expression profile comparisons between glioependymal and arachnoid cysts revealed distinct cellular profiles. Analyses of the innermost cell layer (ependymal cells versus arachnoid cells) suggested differing mechanisms of pathogenesis. Glioependymal cysts harbored upregulated expression of sodium transporters and aquaporin channels, which suggests possible CSF-like fluid production whereas arachnoid cysts may be associated with mechanical fluid accumulation. Thus, intracranial cysts represent multiple unique pathologic entities with differing cell types and RNA expression profiles. Further study into mechanisms of glioependymal cyst formation may allow for targeted interventions to reduce fluid production and avoid surgery.","PeriodicalId":16682,"journal":{"name":"Journal of Neuropathology and Experimental Neurology","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143803468","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

TBX19/SF1 co-expressing pituitary neuroendocrine tumor/pituitary adenomas add to the lineage infidelity.

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-04-08 DOI: 10.1093/jnen/nlaf028

Christie G Turin, Timothy H Ung, B K Kleinschmidt-DeMasters

引用次数: 0

Consecutive brain biopsies illustrate the histological evolution of acute hemorrhagic leukoencephalitis.

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-04-04 DOI: 10.1093/jnen/nlaf019

Felipe J S Jones, Emily Ling-Lin Pai, Rogan Magee, Kelly Boylan, Vivian Chioma, Jessica Little, Daniel Yoshor, Jose L Pascual, Amir Banihashemi, Sashank Prasad, Edward B Lee, Jennifer Orthmann-Murphy

引用次数: 0

Different cortical and subcortical astroglial responsiveness in rats with acute liver failure.

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-04-02 DOI: 10.1093/jnen/nlaf020

Nahla Ouard, Assmaâ Tali, Themoi Demsou Souhoudji, Rajâa Jebbouj, Ihssane El-Bouchikhi, Christopher F Rose, Samir Ahboucha

{"title":"Different cortical and subcortical astroglial responsiveness in rats with acute liver failure.","authors":"Nahla Ouard, Assmaâ Tali, Themoi Demsou Souhoudji, Rajâa Jebbouj, Ihssane El-Bouchikhi, Christopher F Rose, Samir Ahboucha","doi":"10.1093/jnen/nlaf020","DOIUrl":"https://doi.org/10.1093/jnen/nlaf020","url":null,"abstract":"Hepatic encephalopathy (HE) is a neuropsychiatric complication of liver failure. Previous studies described astroglia alterations in HE, but regional changes have not been well investigated. This study addresses regional astroglial response by exploring glial fibrillary acidic protein (GFAP) immunoreactivity in cortical structures including somatosensory (S1Tr and S1BF), piriform (Pir), and perirhinal (PRh) cortices, and subcortical regions including corpus callosum (CC), ventromedial thalamus (VMT), mammillothalamic tract (MTT), and dorsomedial hypothalamic nucleus (DHN) in rats with acute liver failure (ALF) sacrificed at coma stage. Our data showed decreased numbers of astrocytes in S1Tr, Pir, and CC in ALF rats. GFAP-immunoreactive cells were increased within other regions including PRh, VMT, MTT, and DHN. Cell morphometric analysis showed significant increase in GFAP-immunoreactive astrocyte processes and cell bodies in cortical and subcortical regions but not in CC and DHN. However, astrocyte perimeters were increased, particularly in S1Tr and VMT. Our study demonstrates regional specificity including (1) regions with astrocyte activation associated with an increase of GFAP-immunostaining and astrocyte cell counts, together with (2) unaltered GFAP components, and (3) regions characterized by presumably inactive astrocyte with a reduced GFAP-immunostaining. These findings may reflect either different regional alterations in HE, or stages of an alteration progressing differently in different regions.","PeriodicalId":16682,"journal":{"name":"Journal of Neuropathology and Experimental Neurology","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-04-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143772546","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Star-shaped TDP-43 inclusions in the oldest-old. 在最古老的植物中存在星形 TDP-43 包涵体。

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-04-01 DOI: 10.1093/jnen/nlae116

Erin E Connolly, John F Ervin, Brenda L Plassman, Kathleen A Welsh-Bohmer, Shih-Hsiu J Wang

引用次数: 0

Multi-omic analysis of meningeal cerebral amyloid angiopathy reveals enrichment of unsubstituted glucosamine and extracellular proteins.

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-03-29 DOI: 10.1093/jnen/nlaf018

Joshua E Mayfield, Alexander J Rajic, Patricia Aguilar-Calvo, Katrin Soldau, Samantha Flores, Roger Lawrence, Biwsa Choudhury, Majid Ghassemian, Donald P Pizzo, Steven L Wagner, Garrett A Danque, Paige Sumowski, Lawrence A Hansen, Vanessa Goodwill, Jeffery D Esko, Christina J Sigurdson

{"title":"Multi-omic analysis of meningeal cerebral amyloid angiopathy reveals enrichment of unsubstituted glucosamine and extracellular proteins.","authors":"Joshua E Mayfield, Alexander J Rajic, Patricia Aguilar-Calvo, Katrin Soldau, Samantha Flores, Roger Lawrence, Biwsa Choudhury, Majid Ghassemian, Donald P Pizzo, Steven L Wagner, Garrett A Danque, Paige Sumowski, Lawrence A Hansen, Vanessa Goodwill, Jeffery D Esko, Christina J Sigurdson","doi":"10.1093/jnen/nlaf018","DOIUrl":"https://doi.org/10.1093/jnen/nlaf018","url":null,"abstract":"Cerebral amyloid angiopathy (CAA) is a common feature of Alzheimer's disease in which amyloid-β (Aβ) deposits in cerebral and leptomeningeal vessel walls, predisposing vessels to micro- and macro-hemorrhages. The vessel walls contain distinct proteins and heparan sulfate (HS), yet how vascular proteins and HS jointly associate with Aβ is unknown. We conducted the first multi-omics study to systematically characterize the proteins as well as the HS abundance, sulfation level, and disaccharide composition of leptomeninges from 23 moderate to severe CAA cases and controls. We then analyzed the associations between Aβ and other proteins, HS, and apolipoprotein E genotype. We found an increase in a minor HS disaccharide containing unsubstituted glucosamine, as well as 6-O sulfated disaccharides; Aβ40 levels positively correlated with unsubstituted glucosamine. There was also an increase in extracellular proteins derived from brain parenchyma or plasma, including olfactomedin-like protein 3, fibrinogen, serum amyloid protein, apolipoprotein E, and secreted frizzled related protein-3. Our findings of vascular HS and protein alterations specific to CAA-affected leptomeningeal vessels provide molecular insight into the extracellular remodeling that co-occurs with Aβ deposits and may indicate a basis for antemortem diagnostic assay development and therapeutic strategies to impede Aβ-HS interactions.","PeriodicalId":16682,"journal":{"name":"Journal of Neuropathology and Experimental Neurology","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143743236","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Isocitrate dehydrogenase-mutant astrocytoma in persons aged 55 years and older: Survival differences versus the young.

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-03-28 DOI: 10.1093/jnen/nlaf024

Zainab Siddiq, Ahmed Gilani, Timothy H Ung, Bette K Kleinschmidt-DeMasters

{"title":"Isocitrate dehydrogenase-mutant astrocytoma in persons aged 55 years and older: Survival differences versus the young.","authors":"Zainab Siddiq, Ahmed Gilani, Timothy H Ung, Bette K Kleinschmidt-DeMasters","doi":"10.1093/jnen/nlaf024","DOIUrl":"https://doi.org/10.1093/jnen/nlaf024","url":null,"abstract":"Isocitrate dehydrogenase (IDH)-mutant astrocytomas show a peak incidence in young and middle-aged adults and have relatively favorable outcomes. In patients with these tumors ≥55 years at diagnosis, clinical, histopathologic, and prognostic characteristics are less clear. Here, we compared histopathological, immunohistochemical, molecular, and overall survival of 34 patients aged ≥55 years with a group of 84 patients aged 19-54 years; all had IDH mutant astrocytomas. The older cohort had 14 World Health Organization (WHO) grade 2, 7 WHO grade 3, and 13 WHO grade 4 tumors versus 24, 32, and 28 WHO grade 2, 3, and 4 tumors in the younger group. Comparing equal-grade tumors in both cohorts, Kaplan-Meyer survival analysis revealed that patients ≥55 years of age showed worse prognosis despite receiving comparable treatment regimens (Stupp protocol). Roughly equal numbers of noncanonical IDH mutations were seen in both groups (11.76% in ≥55 vs 19.04% in <55). Older patients were more likely to show retention of nuclear protein alpha-thalassemia and mental retardation X-linked syndrome (ATRX) and/or absence of strong P53 staining by immunohistochemistry. Although patients ≥55 years of age with astrocytomas, IDH-mutant, had worse overall survival, many, particularly those with low-grade tumors, had 5 years or greater survival. Employing parallel treatment regimens with chemotherapy, radiation, and maximum safe resection may improve survival of older patients with IDH mutant gliomas.","PeriodicalId":16682,"journal":{"name":"Journal of Neuropathology and Experimental Neurology","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143743235","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Therapeutic potential and challenges of mesenchymal stem cells in neurological disorders: A concise analysis.

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-03-27 DOI: 10.1093/jnen/nlaf021

Enas H Bani Issa, Enas M Alghazo, Raghad Gharaibeh, Noor B Momani, Dana Z Taha, Renad J Jaradat, Ayman Alzu'bi, Fatimah A Almahasneh, Ejlal Abu-El-Rub, Raed M Al-Zoubi

{"title":"Therapeutic potential and challenges of mesenchymal stem cells in neurological disorders: A concise analysis.","authors":"Enas H Bani Issa, Enas M Alghazo, Raghad Gharaibeh, Noor B Momani, Dana Z Taha, Renad J Jaradat, Ayman Alzu'bi, Fatimah A Almahasneh, Ejlal Abu-El-Rub, Raed M Al-Zoubi","doi":"10.1093/jnen/nlaf021","DOIUrl":"https://doi.org/10.1093/jnen/nlaf021","url":null,"abstract":"Neurological diseases comprise a wide array of conditions affecting both the central and peripheral nervous systems. Neurodegenerative diseases encompass a group of debilitating and often fatal neurological disorders for which effective treatments are currently lacking. Stem cells are recognized for their remarkable capacity for proliferation, multilineage differentiation, and self-renewal. The transplantation of stem cells represents a significant advancement in therapeutic strategies for neurological disorders, with applications in both preclinical and clinical settings. Mesenchymal stem cells (MSCs), in particular, have garnered substantial interest due to their unique properties, making them a highly sought-after source of therapeutic cells. Although the efficacy of MSCs in treating neurological disorders is well documented, further research is needed to elucidate the underlying mechanisms and to assess their in vivo applications more comprehensively. This article summarizes current research on the use of MSCs in the treatment of various neurological disorders, including Parkinson disease, stroke, multiple sclerosis, and Alzheimer disease.","PeriodicalId":16682,"journal":{"name":"Journal of Neuropathology and Experimental Neurology","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143730508","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Systemic anaplastic lymphoma kinase-negative anaplastic large cell lymphoma with prominent meningeal involvement: A case report. 全身性无细胞淋巴瘤激酶阴性无细胞大细胞淋巴瘤，脑膜明显受累：病例报告。

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-03-27 DOI: 10.1093/jnen/nlaf023

Yingxue Yang, Ziyan Zhu, Yajie Wang, Yueshan Piao, Hua Lin

引用次数: 0

Expanding the spectrum of TERT promoter mutations in CNS tumors: A case series of non-canonical mutations.

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-03-19 DOI: 10.1093/jnen/nlaf022

David J Cook, Jorge A Trejo-Lopez, Beth A Pitel, Neiladri Saha, Tejaswi Koganti, Jayson Hardcastle, Stephanie Smoley, Matthew Isaacson, Mallika Gandham, Gopinath Sivasankaran, Surendra Dasari, Kar-Ming Fung, Suzanne Z Powell, Darshan Trivedi, Zied Abdullaev, Kenneth Aldape, Martha Quezado, Drew Pratt, Patrick Joseph Cimino, Mark A Edgar, Rachael A Vaubel, Aditya Raghunathan, Caterina Giannini, Robert B Jenkins, Cristiane M Ida

引用次数: 0