JOURNAL OF NEUROPATHOLOGY & EXPERIMENTAL NEUROLOGY 是美国神经病理学家协会 (AANP) 的官方期刊。该杂志发表关于神经病理学和实验神经科学的同行评审研究、书评、信件和协会新闻，涵盖基础神经科学的广泛领域，重点是人类神经系统疾病。它由来自世界各地的神经病理学家、神经学家、神经外科医生、病理学家、精神病学家和基础神经科学家编写，并为它们编写。自 1942 年以来一直连续出版。

The Journal of Neuropathology and Experimental Neurology is the official journal of the American Association of Neuropathologists (AANP). This journal publishes peer-reviewed research, book reviews, letters, and association news related to neuropathology and experimental neuroscience, covering a wide range of areas in basic neuroscience with a focus on human neurological diseases. It has been continuously published since 1942, featuring contributions from neuropathologists, neurologists, neurosurgeons, pathologists, psychiatrists, and basic neuroscientists from around the world.

《Journal of Neuropathology and Experimental Neurology》是美国神经病理学家协会（AANP）的官方期刊。该期刊发表关于神经病理学和实验神经科学的同行评审研究、书评、信件和协会新闻，涵盖基础神经科学的广泛领域，特别关注人类神经系统疾病。自1942年以来，该期刊一直持续出版，作者包括来自世界各地的神经病理学家、神经学家、神经外科医生、病理学家、精神病学家和基础神经科学家。

Journal of Neuropathology and Experimental Neurology

Publication of the WHO Classification of Tumors of the Nervous System in 2000 represents the most recent combined effort of an international group of distinguished neuropathologists who have been focussing intensively on this issue since 1988. At that time, the late Dr. Klaus Zulch and Dr. William Fields convened a meeting in Houston to focus on three aspects that they thought required special attention since deliberations by a committee working under the sponsorship of the World Health Organization (WHO) had published their consensus document in 1979 (1).

The three areas of focus at the 1988 meeting in Houston, Texas included the concept of primitive neuroectodermal tumor and its relationship to medulloblastoma, grading of glial tumors and histo-biologic aspects of meningioma. Proceedings of this meeting were published and served as background studies for a second meeting of the WHO …

Lucy Balian Rorke

Journal of Neuropathology & Experimental Neurology

Commentary on the WHO Classification of Tumors of the Nervous System

关于世界卫生组织神经系统肿瘤分类的评论

The loss and degeneration of spinal cord motor neurons result in muscle denervation in spinal muscular atrophy (SMA), but whether there are primary pathogenetic abnormalities of muscle in SMA is not known. We previously detected increased DNA fragmentation and downregulation of Bcl-2 and Bcl-X(L) expression but no morphological changes in spinal motor neurons of SMA fetuses. Here, we performed histological and morphometric analysis of myotubes and assessed DNA fragmentation and Bcl-2/Bcl-X(L) expression in skeletal muscle from fetuses with type I SMA (at approximately 12 and 15 weeks' gestational ages, n = 4) and controls (at approximately 10-15 weeks' gestational ages, n = 7). Myotubes were smaller in the SMA than in control samples at all ages analyzed (p < 0.001) and were often arranged in clusters close to isolated and larger myotubes. Numbers of terminal deoxynucleotidyl transferase dUTP nick end labeling-positive cells in control and SMA fetuses were similar, and no differences in Bcl-2 or Bcl-X(L) immunostaining between control and SMA muscle were identified. Areas with smaller myotubes and the morphometric analysis suggested a delay in growth and maturation in SMA muscle. These results suggest that spinal motor neurons and skeletal muscle undergo different pathogenetic processes in SMA during development; they imply that muscle as well as motor neurons may be targets for early therapeutic intervention in SMA.

Rebeca Martínez-Hernández

Carolina Soler-Botija

Eva Also

Laura Alias

Lidia Caselles

Ignasi Gich

Sara Bernal

Eduardo F. Tizzano

The Developmental Pattern of Myotubes in Spinal Muscular Atrophy Indicates Prenatal Delay of Muscle Maturation

脊髓性肌萎缩中肌管的发育模式表明肌肉成熟存在产前延迟

Nicotinamide N-methyltransferase (NNMT) has been proposed as a link between the environmental and genetic factors of Parkinson disease (PD). Therefore, we explored the hypothesis that high levels of NNMT expression may predispose to the development of PD. Regions of high mRNA expression were shown in the spinal cord, medulla, and temporal lobe, with lowest expression in the cerebellum, subthalamic nucleus, and caudate nucleus. Using 2 NNMT antibodies, the protein was shown to be expressed in multipolar neurons in the temporal lobe, caudate nucleus, and spinal cord, granular neurons of the cerebellum, dopaminergic neurons in the substantia nigra, and in the axons of the third nerve. Expression of NNMT was compared in PD and non-PD control cerebella and caudate nucleus. PD tissue exhibited significantly increased levels of NNMT protein and activity. PD disease duration was inversely correlated with the level of expression in cerebellum. This is the first demonstration that patients with PD have higher levels of NNMT activity and protein in brain tissue than those without PD and that NNMT expression is associated with neurons that degenerate in PD.

Richard B. Parsons

Marie-Louise Smith

Adrian C. Williams

Rosemary H. Waring

David B. Ramsden

Expression of Nicotinamide N-Methyltransferase (E.C. 2.1.1.1) in the Parkinsonian Brain

帕金森病脑中烟酰胺N-甲基转移酶（E.C. 2.1.1.1）的表达

There are currently no standardized therapies for Parkinson disease (PD). Curcumin shows anti-amyloidogenic properties in vitro and may be a promising treatment for PD. We evaluated the effects of curcumin supplementation on clinical scales and misfolded, phosphorylated α-synuclein (p-syn) accumulation in skin biopsies in 19 PD patients who received curcumin supplementation for 12 months and 14 PD patients to treated with curcumin. The patients underwent autonomic (COMPASS-31), motor (MDS-UPDRS and H&Y) and nonmotor (NMSS) questionnaires and skin biopsies to evaluate clinical involvement and p-syn load in skin nerves at the beginning and the end of study. Curcumin and curcuminoid levels were assayed in plasma and CSF. Supplemented patients showed detectable CSF curcuminoid levels that were lower than those in plasma. They showed a decrease of COMPASS-31 and NMSS scores, and a slight p-syn load decrease versus untreated patients who displayed a worsening of these parameters despite increased levodopa doses. Multiple regression models showed a significant effect of curcumin supplementation in decreasing the worsening of the clinical parameters and p-syn load at after curcumin treatment. These data suggest that curcumin can cross the blood-brain barrier, that it is effective in ameliorating clinical parameters and that it shows a tendency to decrease skin p-syn accumulation in PD patients.

Vincenzo Donadio

Alex Incensi

Giovanni Rizzo

Enrico Fileccia

Francesco Ventruto

Antonella Riva

Domenico Tiso

Martino Recchia

Veria Vacchiano

Rossella Infante

Giovanna Petrangolini

Pietro Allegrini

Silvia Avino

Roberta Pantieri

Barbara Mostacci

Patrizia Avoni

Rocco Liguori

The Effect of Curcumin on Idiopathic Parkinson Disease: A Clinical and Skin Biopsy Study

姜黄素对特发性帕金森病的影响：一项临床与皮肤活检研究

Eileen Healy

Meritorious Contributions to Neuropathology

神经病理学杰出贡献

Abstract Ischemic strokes pose serious risks to human health. We aimed to elucidate the function of NOD-like receptor X1 (NLRX1) in a rat middle cerebral artery occlusion (MCAO)-induced cerebral ischemia/reperfusion injury (CIRI) model and in an oxygen-glucose deprivation/reperfusion (OGD/R)-treated human microglial cell line (HMC3) model. Following NLRX1 upregulation, infarct volumes were measured with 2,3,5-triphenyltetrazolium chloride staining and pathological examination was conducted with hematoxylin-eosin staining. Results suggested that levels of NLRX1 were decreased in brain tissue of MCAO rats and in OGD/R-stimulated HMC3 cells. NOD-like receptor X1 overexpression mitigated the neuronal damage, reduced tumor necrosis factor-α and interleukin-6 expression, alleviated microglial activation, and induced autophagy in vivo and in vitro. Additionally, a coimmunoprecipitation assay indicated that NLRX1 bound to autophagy-related gene 5 (ATG5) to elevate ATG5 expression in HMC3 cells. Further, the elevated NLR family pyrin domain containing 3 (NLRP3), apoptosis-associated speck-like protein containing a CARD, and cleaved caspase 1 expression in MCAO rats and HMC3 cells with OGD/R induction was reduced after NLRX1 upregulation. Importantly, ATG5 depletion abrogated the effects of NLRX1 elevation on NLRP3 inflammasome signaling. These results indicate that NLRX1 promotes autophagy and inactivates NLRP3 inflammasome signaling by binding ATG5 in experimental cerebral ischemia. These data may help the development of novel therapeutic strategies for ischemic stroke.

Yufen Peng

Yong Long

Chenyi Wan

NOD-like receptor X1 promotes autophagy and inactivates NLR family pyrin domain containing 3 inflammasome signaling by binding autophagy-related gene 5 to alleviate cerebral ischemia/reperfusion-induced neuronal injury

NOD样受体X1通过结合自噬相关基因5促进自噬并失活NLR家族含吡啶结构域3炎症小体信号，以减轻脑缺血/再灌注引起的神经元损伤

Julieann C Lee

Amar Gajjar

Melissa R Perrino

Angela Delaney

Christopher L Tinkle

Paul Klimo

Larissa V Furtado

Yen-Chun Liu

Alex Breuer

Soniya N Pinto

Asim K Bag

David W Ellison

Brent A Orr

Concurrent Pit-1 and SALL4 staining in primitive component of pituitary blastoma: A case report with clinicopathologic features including Cushing syndrome presentation, germline <i>DICER1</i> mutation, and significant therapeutic response to chemoradiation

垂体母细胞瘤原始成分中同时存在Pit-1和SALL4染色：一份包含库欣综合征表现、种系<i>DICER1</i>突变及对放化疗显著治疗反应的临床病理特征的病例报告

Studying comorbidities in early onset Alzheimer disease (AD) may provide an advantageous perspective on their pathogenesis because aging factors may be largely inoperative for these subjects. We compared AD comorbidities between early-onset sporadic cases and American and Colombian cases with PSEN1 mutations. AD neuropathological changes (ADNC) were very severe in all groups but more severe in the PSEN1 groups. Lewy body disease and cerebral white matter rarefaction were the most common (up to 60%) of AD comorbidities, followed by arteriolosclerosis (up to 37%), and large-vessel atherosclerosis (up to 20%). Differences between the 3 groups included earlier age of onset in the American PSEN1 cases, shorter disease duration in sporadic cases, and more frequent large-vessel atherosclerosis and cerebral amyloid angiopathy in the Colombian PSEN1 cases. Logistic regression models adjusted for age and sex found the presence of a PSEN1 mutation, an apolipoprotein ε4 allele and TDP-43 pathology to predict an earlier age of onset; Hispanic ethnicity and multiracial subjects were predictive of severe CAA. Comorbidities are common in early onset AD and should be considered when planning clinical trials with such subjects. However, they may be at least partially dependent on ADNC and thus potentially addressable by anti-amyloid or and/anti-tau therapies.

Diego Sepulveda-Falla

Carlos Andrés Villegas Lanau

Charles White III

Geidy E Serrano

Juliana Acosta-Uribe

Barbara Mejía-Cupajita

Nelson David Villalba-Moreno

Pinzhang Lu

Markus Glatzel

Julia K Kofler

Bernardino Ghetti

Matthew P Frosch

Francisco Lopera Restrepo

Kenneth S Kosik

Thomas G Beach

Comorbidities in early-onset sporadic versus presenilin-1 mutation-associated Alzheimer disease dementia: Evidence for dependency on Alzheimer disease neuropathological changes

早发性散发性与 presenilin-1 突变相关阿尔茨海默病痴呆的合并症：依赖于阿尔茨海默病神经病理学改变的证据

Abstract Intracranial aneurysms (IAs) affect 1%-5% of the public and are a major cause of subarachnoid hemorrhage. Currently, there is no medical treatment to prevent the progression or rupture of IAs. Recent studies have defined IA as a chronic inflammatory disease in which macrophages infiltrate intracranial arteries via the CCL2-CCR2 axis. The chemokine signal regulator FROUNT mediates this axis, and it can be inhibited by the anti-alcoholism drug disulfiram. Therefore, inhibition of macrophage infiltration by interfering with FROUNT using disulfiram may represent a strategy to prevent exacerbation of IAs. Here, effects of disulfiram were investigated in vitro and in an animal model of IAs. FROUNT expression was observed on infiltrated macrophages both in human IAs and in the rat IA model by immunohistochemistry. In vitro treatment with disulfiram suppressed CCL2-mediated migration of cultured rat macrophages in a transwell system. Disulfiram administered in a rat model of IAs inhibited both the initiation and the enlargement of IAs in a dose-dependent manner; this was accompanied by suppression of macrophage infiltration. These results suggest that pharmacological inhibition of the CCL2-CCR2-FROUNT signaling cascade could be a treatment of patients with IAs.

Isao Ono

Masahiko Itani

Akihiro Okada

Akitsugu Kawashima

Etsuko Toda

Yoshiki Arakawa

Yuya Terashima

Tomohiro Aoki

Potential of the pharmacological inhibition of CCL2-CCR2 axis via targeting FROUNT to prevent the initiation and the progression of intracranial aneurysms in rats

通过靶向FROUNT抑制CCL2-CCR2轴的药理学潜力，以预防大鼠颅内动脉瘤的形成和进展

Abbreviation	J Neuropathol Exp Neurol
Journal Impact	3.28
Quartiles(Global)	PATHOLOGY(Q2)

ISSN	0022-3069, 1554-6578
h-index	178

Publication Information	Publisher: Oxford University Press，Publishing cycle: Monthly，Journal Type: journal，Open Access Journals: No
Basic data	Year of publication: 1942，Proportion of original research papers: 93.98%，Self Citation Rate:9.40%， Gold OA Rate: 19.42%
Average review cycle	网友分享经验：较慢,6-12周
Average recruitment ratio	网友分享经验：一般

Journal of Neuropathology and Experimental Neurology

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Commentary on the WHO Classification of Tumors of the Nervous System

The Developmental Pattern of Myotubes in Spinal Muscular Atrophy Indicates Prenatal Delay of Muscle Maturation

Expression of Nicotinamide N-Methyltransferase (E.C. 2.1.1.1) in the Parkinsonian Brain

The Effect of Curcumin on Idiopathic Parkinson Disease: A Clinical and Skin Biopsy Study

Meritorious Contributions to Neuropathology

NOD-like receptor X1 promotes autophagy and inactivates NLR family pyrin domain containing 3 inflammasome signaling by binding autophagy-related gene 5 to alleviate cerebral ischemia/reperfusion-induced neuronal injury

Meritorious Contributions to Neuropathology

Concurrent Pit-1 and SALL4 staining in primitive component of pituitary blastoma: A case report with clinicopathologic features including Cushing syndrome presentation, germline DICER1 mutation, and significant therapeutic response to chemoradiation

Comorbidities in early-onset sporadic versus presenilin-1 mutation-associated Alzheimer disease dementia: Evidence for dependency on Alzheimer disease neuropathological changes

Potential of the pharmacological inhibition of CCL2-CCR2 axis via targeting FROUNT to prevent the initiation and the progression of intracranial aneurysms in rats