Neuropathologic characterization of INF2-related Charcot-Marie-Tooth disease: evidence for a Schwann cell actinopathy.

abstract：:How to assess the substantial neuronal loss in a neurodegenerative disease such as Alzheimer disease is still being debated. Recently, stereological procedures have been proposed that claim improved accuracy and statistical power, but the results of some of these investigations have been controversial. In this study w...

journal_title：Journal of neuropathology and experimental neurology

authors： Everall IP,DeTeresa R,Terry R,Masliah E

更新日期：1997-11-01 00:00:00

abstract：:Genetic and lifestyle-related risk factors for Alzheimer disease (AD) are associated with an increase in oxidative stress, suggesting that oxidative stress is involved at an early stage of the pathologic cascade. Moreover, oxidative stress is mechanistically and chronologically associated with other key features of AD...

journal_title：Journal of neuropathology and experimental neurology

authors： Nunomura A,Castellani RJ,Zhu X,Moreira PI,Perry G,Smith MA

更新日期：2006-07-01 00:00:00

abstract：:The changes of nitric oxide synthase (NOS) activity and expression in experimental diabetic neuropathy have not been examined. Increases in ganglia NOS might be similar to those that follow axotomy, whereas declines in endothelial NOS (eNOS) and immunological NOS (iNOS) might explain dysfunction of microvessels or mac...

journal_title：Journal of neuropathology and experimental neurology

authors： Zochodne DW,Verge VM,Cheng C,Höke A,Jolley C,Thomsen K,Rubin I,Lauritzen M

更新日期：2000-09-01 00:00:00

abstract：:Mutations in peripheral myelin protein 22 (PMP22) result in the most common form of Charcot-Marie-Tooth (CMT) disease, CMT1A. This hereditary peripheral neuropathy is characterized by dysmyelination of peripheral nerves, reduced nerve conduction velocity, and muscle weakness. APMP22 point mutation in L16P (leucine 16 ...

journal_title：Journal of neuropathology and experimental neurology

authors： Scurry AN,Heredia DJ,Feng CY,Gephart GB,Hennig GW,Gould TW

更新日期：2016-04-01 00:00:00

abstract：:Infection with human immunodeficiency virus (HIV) clade C is the most common HIV infection worldwide, yet its impact on the nervous system remains largely unknown. Autopsy studies from regions affected by this virus are scarce, and HIV dementia has only rarely been reported from these countries. Most patients who deve...

journal_title：Journal of neuropathology and experimental neurology

authors： Mahadevan A,Shankar SK,Satishchandra P,Ranga U,Chickabasaviah YT,Santosh V,Vasanthapuram R,Pardo CA,Nath A,Zink MC

更新日期：2007-09-01 00:00:00

abstract：:Tuberous sclerosis (TSC) is a multi-system disorder characterized by hamartomatous tumors and abnormal brain development, with multiple foci of disrupted neuronal migration and giant dysmorphic neurons within cortical tubers. TSC is associated with mutations in 2 genes, TSC1 and TSC2, which encode hamartin and tuberin...

journal_title：Journal of neuropathology and experimental neurology

authors： Catania MG,Mischel PS,Vinters HV

更新日期：2001-07-01 00:00:00

abstract：:Recently, we demonstrated a significant increase of an oxidized nucleoside derived from RNA, 8-hydroxyguanosine (8OHG), and an oxidized amino acid, nitrotyrosine in vulnerable neurons of patients with Alzheimer disease (AD). To determine whether oxidative damage is an early- or end-stage event in the process of neurod...

journal_title：Journal of neuropathology and experimental neurology

authors： Nunomura A,Perry G,Aliev G,Hirai K,Takeda A,Balraj EK,Jones PK,Ghanbari H,Wataya T,Shimohama S,Chiba S,Atwood CS,Petersen RB,Smith MA

更新日期：2001-08-01 00:00:00

abstract：:AWe report on two siblings (brother and sister) who developed cerebral PNETs at the age of 5 years and 6 months, respectively. Both children were treated by operation followed by polychemotherapy. The brother also received cranio-spinal irradiation. Nevertheless, the children died about 12 months and 24 months post-op...

journal_title：Journal of neuropathology and experimental neurology

authors： Reifenberger J,Janssen G,Weber RG,Boström J,Engelbrecht V,Lichter P,Borchard F,Göbel U,Lenard HG,Reifenberger G

更新日期：1998-02-01 00:00:00

abstract：:This is a report of an unusual, densely cellular, midcerebellar neoplasm in a seven-year-old boy. Although clinically consistent with a medulloblastoma, immunohistochemistry and electron microscopy demonstrated glial and rhabdomyoblastic differentiation in the tumor. We discuss the differential diagnosis of this tumor...

journal_title：Journal of neuropathology and experimental neurology

authors： Dickson DW,Hart MN,Menezes A,Cancilla PA

更新日期：1983-11-01 00:00:00

abstract：:Increasing evidence has demonstrated a vital role of microRNAs (miRNAs) in diverse biological processes. However, their functions in developing brain with hypoxia-ischemia (HI) remain largely unknown. Through a miRNA microarray analysis in a P10 rat model of cerebral HI, we found that miR-30d-5p was one of the most de...

journal_title：Journal of neuropathology and experimental neurology

authors： Zhao F,Qu Y,Zhu J,Zhang L,Huang L,Liu H,Li S,Mu D

更新日期：2017-08-01 00:00:00

abstract：:The external segment of the globus pallidus (GPe) in humans and the equivalent structure in rodents, the globus pallidus (GP), influence signal processing in the basal ganglia under normal and pathological conditions. Parvalbumin (PV) immunoreactivity defines 2 main neuronal subpopulations in the GP/GPe: PV-immunoposi...

journal_title：Journal of neuropathology and experimental neurology

authors： Fernández-Suárez D,Celorrio M,Lanciego JL,Franco R,Aymerich MS

更新日期：2012-11-01 00:00:00

abstract：:Peripheral neuropathy is a significant complication of diabetes resulting in increased patient morbidity and mortality. Deficiencies of neurotrophic substances (e.g. NGE NT-3, and IGF-I) have been proposed as pathogenetic mechanisms in the development of distal symmetrical sensory diabetic polyneuropathy, and salutary...

journal_title：Journal of neuropathology and experimental neurology

authors： Schmidt RE,Dorsey DA,Beaudet LN,Parvin CA,Escandon E

更新日期：2001-03-01 00:00:00

abstract：:Chronic experimental allergic encephalomyelitis (EAE), produced in inbred guinea pigs given a single inoculation during the juvenile period with isologous spinal cord in complete Freund's adjuvant, has been studied by light and electron microscopy. Most animals showed a delayed onset of nurologic signs from 12 to 68 w...

journal_title：Journal of neuropathology and experimental neurology

authors： Snyder DH,Valsamis MP,Stone SH,Raine CS

更新日期：1975-05-01 00:00:00

abstract：:Multiple sclerosis (MS) is an inflammatory demyelinating disorder of the CNS. A recent study identified 4 patterns of demyelination in active MS lesions. The characteristics of pattern II lesions suggested a primary inflammatory mechanism of myelin injury, while pattern III lesions showed features consistent with dyin...

journal_title：Journal of neuropathology and experimental neurology

authors： Mahad DJ,Trebst C,Kivisäkk P,Staugaitis SM,Tucky B,Wei T,Lucchinetti CF,Lassmann H,Ransohoff RM

更新日期：2004-03-01 00:00:00

abstract：:The periaqueductal gray (PAG) plays a central role in the integration of defense responses to threatening or stressful stimuli. Little is known about the neurochemical development of the human PAG around the time of birth, when the fetus makes the transition to extrauterine life and independent defense responses are n...

journal_title：Journal of neuropathology and experimental neurology

authors： Reddy SC,Panigrahy A,White WF,Kinney HC

更新日期：1996-04-01 00:00:00

abstract：:The presence of diffuse or primitive senile plaques in the neocortex of cognitively normal elderly at autopsy has been presumed to represent normal aging. Alternatively, these patients may have developed dementia and clinical Alzheimer disease (AD) if they had survived. In this setting, these patients could be subject...

journal_title：Journal of neuropathology and experimental neurology

authors： Hulette CM,Welsh-Bohmer KA,Murray MG,Saunders AM,Mash DC,McIntyre LM

更新日期：1998-12-01 00:00:00

abstract：:Ischemic brain damage can be prevented or at least significantly reduced when there is a preceding brief ischemic period that does not exceed the threshold for tissue damage--a phenomenon termed "ischemic preconditioning" (ischemic PC). Experimental PC in rodents is now considered to be a model for transient ischemic ...

journal_title：Journal of neuropathology and experimental neurology

authors： Sommer C

更新日期：2008-02-01 00:00:00

abstract：:Early inflammatory events may inhibit functional recovery after injury in both the peripheral and central nervous systems. We investigated the role of the inflammatory tumor necrosis factor/nuclear factor-kappaB (NF-kappaB) axis on events subsequent to sciatic nerve crush injury in adult rats. Electrophoretic mobility...

journal_title：Journal of neuropathology and experimental neurology

authors： Smith D,Tweed C,Fernyhough P,Glazner GW

更新日期：2009-06-01 00:00:00

abstract：:Brain atrophy is a common finding in patients with AIDS, but the relationship of atrophy to HIV-associated dementia is unclear. We used unbiased, stereological methods on postmortem brain specimens to estimate volumes of different brain regions in patients prospectively diagnosed with and without HIV-associated dement...

journal_title：Journal of neuropathology and experimental neurology

authors： Subbiah P,Mouton P,Fedor H,McArthur JC,Glass JD

更新日期：1996-10-01 00:00:00

abstract：:This morphometric study explores temporal and topographic changes in the estimated neuronal number in human neocortex during the latter half of gestation and early infancy. Neuronal estimates are calculated from standardized measurements of cortical layer thickness and neuronal density in 6 neocortical regions in 9 hu...

journal_title：Journal of neuropathology and experimental neurology

authors： Rabinowicz T,de Courten-Myers GM,Petetot JM,Xi G,de los Reyes E

更新日期：1996-03-01 00:00:00

abstract：:Ethanol is a potent neurotoxin particularly for the developing nervous system. Intrauterine exposure to ethanol during the last trimester of human gestation can produce a broad spectrum of neuropathologic consequences. This period of human brain development is roughly equivalent to the first week of rodent postnatal l...

journal_title：Journal of neuropathology and experimental neurology

authors： Nowoslawski L,Klocke BJ,Roth KA

更新日期：2005-06-01 00:00:00

abstract：:Rates of remyelination decline with age and this has been attributed to slower recruitment of oligodendrocyte progenitor cells (OPCs) into areas of demyelination and slower differentiation of OPCs into remyelinating oligodendrocytes. When considering causes for reduced recruitment rates, intrinsic causes (alterations ...

journal_title：Journal of neuropathology and experimental neurology

authors： Chari DM,Crang AJ,Blakemore WF

更新日期：2003-09-01 00:00:00

abstract：:Selenoprotein P (Sepp1) is involved in selenium homeostasis. Mice with a deletion of Sepp1, replacement of it by the shortened form Sepp1(Delta240-361), or deletion of its receptor apolipoprotein E receptor 2 develop severe neurologic dysfunction when fed low-selenium diet. Because the brainstems of Sepp1(-/-) mice ha...

journal_title：Journal of neuropathology and experimental neurology

authors： Valentine WM,Abel TW,Hill KE,Austin LM,Burk RF

更新日期：2008-01-01 00:00:00

abstract：:Microglial activation and behavioral abnormalities occur before neuronal loss in experimental murine prion disease; the behavioral changes coincide with a reduction in synaptic plasticity. Because synaptic plasticity depends on an intact perineuronal net (PN), a specialized extracellular matrix that surrounds parvalbu...

journal_title：Journal of neuropathology and experimental neurology

authors： Franklin SL,Love S,Greene JR,Betmouni S

更新日期：2008-03-01 00:00:00

abstract：:The endoneurial lead and water content was serially evaluated in the nerves of rats fed lead carbonate and related to the onset and severity of segmental demyelination and remyelination. Lead began to accumulate significantly in the endoneurium by 5 days, reached a maximum level (71 microgram/g dry weight) by 34 days,...

journal_title：Journal of neuropathology and experimental neurology

authors： Windebank AJ,McCall JT,Hunder HG,Dyck PJ

更新日期：1980-11-01 00:00:00

abstract：:A spontaneous spongy degeneration of the brain stem and spinal cord was discovered in a murine model of accelerated senescence (SAM), cared for under both conventional (SAM-P/8) and specific pathogen-free (SAM-P/8/Ta) conditions. SAM-P/8 and SAM-P/8/Ta showed no clinical neurological abnormalities, yet there was a det...

journal_title：Journal of neuropathology and experimental neurology

authors： Yagi H,Irino M,Matsushita T,Katoh S,Umezawa M,Tsuboyama T,Hosokawa M,Akiguchi I,Tokunaga R,Takeda T

更新日期：1989-09-01 00:00:00

abstract：:This study establishes the sequence of myelination in a population of autopsied infants from birth through the second postnatal year. Myelination was assessed in 62 precisely defined central nervous system (CNS) sites of 162 infants with diverse diseases who were autopsied from 1972 to 1984 at Children's Hospital, Bos...

journal_title：Journal of neuropathology and experimental neurology

authors： Brody BA,Kinney HC,Kloman AS,Gilles FH

更新日期：1987-05-01 00:00:00

abstract：:Growing evidence indicates that alterations within the peripheral nervous system (PNS) are involved at an early stage in the amyotrophic lateral sclerosis (ALS) pathogenetic cascade. In this study, magnetic resonance imaging (MRI), neurophysiologic analyses, and histologic analyses were used to monitor the extent of P...

journal_title：Journal of neuropathology and experimental neurology

authors： Riva N,Chaabane L,Peviani M,Ungaro D,Domi T,Dina G,Bianchi F,Spano G,Cerri F,Podini P,Corbo M,Carro UD,Comi G,Bendotti C,Quattrini A

更新日期：2014-07-01 00:00:00

abstract：:In the central nervous system, tuberous sclerosis complex (TSC) is characterized by a range of lesions including cortical tubers, white matter heterotopias, subependymal nodules, and subependymal giant cell astrocytomas (SEGAs). Recent studies have implicated an important role for the TSC genes TSC1 and TSC2, in a sig...

journal_title：Journal of neuropathology and experimental neurology

authors： Chan JA,Zhang H,Roberts PS,Jozwiak S,Wieslawa G,Lewin-Kowalik J,Kotulska K,Kwiatkowski DJ

更新日期：2004-12-01 00:00:00

abstract：:In adult cerebral astrocytomas, the more anaplasia that is present, the more malignancy that occurs. Cell proliferation antigen staining (MIB-1) and DNA labeling methods for apoptosis using paraffin sections from 39 cases were compared with histopathological grading systems for predicting patient survival. Cases were ...

journal_title：Journal of neuropathology and experimental neurology

authors： Rhodes RH

更新日期：1998-08-01 00:00:00