Frontotemporal lobar degeneration with motor neuron disease-type inclusions predominates in 76 cases of frontotemporal degeneration.

abstract：:Neurofibromas represent one of the hallmarks of neurofibromatosis 1 (NF1) patients. Tumor progression of neurofibromas to malignant peripheral nerve sheath tumors (MPNST) is a frequent and life threatening complication. To learn more about processes involved in malignant transformation, we evaluated differential gene ...

journal_title：Acta neuropathologica

authors： Holtkamp N,Mautner VF,Friedrich RE,Harder A,Hartmann C,Theallier-Janko A,Hoffmann KT,von Deimling A

更新日期：2004-02-01 00:00:00

abstract：:Aggregation and toxicity of the amyloid β-peptide (Aβ) are considered as critical events in the initiation and progression of Alzheimer's disease (AD). Recent evidence indicated that soluble oligomeric Aβ assemblies exert pronounced toxicity, rather than larger fibrillar aggregates that deposit in the forms of extrace...

journal_title：Acta neuropathologica

authors： Kumar S,Wirths O,Stüber K,Wunderlich P,Koch P,Theil S,Rezaei-Ghaleh N,Zweckstetter M,Bayer TA,Brüstle O,Thal DR,Walter J

更新日期：2016-04-01 00:00:00

abstract：:Medulloblastoma (MB) represents approximately 4% of adult brain tumours, and as such is a poorly studied disease. Although many adult MB are treated using paediatric MB protocols, the reported outcomes are inferior to those observed in children. It remains unclear whether biologic differences underlie these clinical o...

journal_title：Acta neuropathologica

authors： Al-Halabi H,Nantel A,Klekner A,Guiot MC,Albrecht S,Hauser P,Garami M,Bognar L,Kavan P,Gerges N,Shirinian M,Roberge D,Muanza T,Jabado N

更新日期：2011-02-01 00:00:00

abstract：:The original version of this article unfortunately contained a typesetting error in Fig 3c. The corrected Fig. 3 is given in the following page. ...

journal_title：Acta neuropathologica

authors： Liu APY,Gudenas B,Lin T,Orr BA,Klimo P Jr,Kumar R,Bouffet E,Gururangan S,Crawford JR,Kellie SJ,Chintagumpala M,Fisher MJ,Bowers DC,Hassall T,Indelicato DJ,Onar-Thomas A,Ellison DW,Boop FA,Merchant TE,Robinson GW,N

更新日期：2020-02-01 00:00:00

abstract：:Striped skunks were inoculated intracerebrally with the scrapie agent (suspension of brain from a naturally infected Suffolk sheep) or intramuscularly with street rabies virus (suspension of salivary glands from naturally infected skunks). Those given the scrapie agent developed clinical signs of weakness, posterior a...

journal_title：Acta neuropathologica

authors： Bundza A,Charlton KM

更新日期：1988-01-01 00:00:00

abstract：:To identify antigenic differences between gliomas and normal brain, we have immunohistochemically studied the expression of lymphocyte adhesion molecules (ICAM-1, ICAM-2, ICAM-3, VCAM-1, E-selectin and CD58), epidermal growth factor receptor (EGFR) and extracellular matrix proteins (collagen IV, fibronectin, laminin, ...

journal_title：Acta neuropathologica

authors： Mäenpää A,Kovanen PE,Paetau A,Jäáskeläinen J,Timonen T

更新日期：1997-09-01 00:00:00

abstract：:We have studied microtubule-associated protein 2 (MAP2) expression in anterior horn neurons in the cervical and lumbar spinal cords of 19 cases of adult-onset sporadic amyotrophic lateral sclerosis (ALS) using immunohistochemistry. Specimens from 7 patients without neurological disease served as controls. MAP2 express...

journal_title：Acta neuropathologica

authors： Kikuchi H,Doh-ura K,Kawashima T,Kira J,Iwaki T

更新日期：1999-01-01 00:00:00

abstract：:Astrocytic alpha-synuclein-immunoreactive inclusions have recently been noted to develop in sporadic Parkinson's disease (PD). Here, the presence of immunoreactive astrocytes is reported in 14 autopsy cases with clinically diagnosed PD and a neuropathological stage of 4 or higher. The labeled astrocytes occur preferen...

journal_title：Acta neuropathologica

authors： Braak H,Sastre M,Del Tredici K

更新日期：2007-09-01 00:00:00

abstract：:Several cases of progressive multifocal leukoencephalopathy (PML) have been associated with simian virus 40 (SV40), rather than with JC virus (JCV), the polyomavirus originally isolated from PML tissue. PML has, therefore, been defined as a demyelinating syndrome with possible multiple viral etiologies. Tissues from t...

journal_title：Acta neuropathologica

authors： Stoner GL,Ryschkewitsch CF

更新日期：1998-09-01 00:00:00

abstract：:Recent studies of genetic abnormalities in pediatric low-grade gliomas (LGGs) have focused on activation of the ERK/MAPK pathway by KIAA1549-BRAF gene fusions in the majority of pilocytic astrocytomas (PAs) and by rare mutations in elements of the pathway across histopathologically diverse LGGs. This study reports tha...

journal_title：Acta neuropathologica

authors： Tatevossian RG,Tang B,Dalton J,Forshew T,Lawson AR,Ma J,Neale G,Shurtleff SA,Bailey S,Gajjar A,Baker SJ,Sheer D,Ellison DW

更新日期：2010-12-01 00:00:00

abstract：:In looking for a possible influence of nuclear inclusions (NIs) on neurodegeneration in human brains, we quantified morphological features of pontine neurons of three unrelated cases of neuronal intranuclear inclusion disease (NIID) and five control cases. Cross-sectional area of each neuronal nucleus and the indices ...

journal_title：Acta neuropathologica

authors： Uchihara T,Tanaka J,Funata N,Arai K,Hattori T

更新日期：2003-02-01 00:00:00

abstract：:The current classification of human sporadic prion diseases recognizes six major phenotypic subtypes with distinctive clinicopathological features, which largely correlate at the molecular level with the genotype at the polymorphic codon 129 (methionine, M, or valine, V) in the prion protein gene and with the size of ...

journal_title：Acta neuropathologica

authors： Parchi P,de Boni L,Saverioni D,Cohen ML,Ferrer I,Gambetti P,Gelpi E,Giaccone G,Hauw JJ,Höftberger R,Ironside JW,Jansen C,Kovacs GG,Rozemuller A,Seilhean D,Tagliavini F,Giese A,Kretzschmar HA

更新日期：2012-10-01 00:00:00

abstract：:Hydrocephalic neonates were observed in a small breeding colony of rats. Normal rats from this colony were obtained and brother-sister mated for seven generations. The overall prevalence of hydrocephalics was approximately 23%; however, in one subline, the prevalence approached 50%. Breeding data suggested the trait t...

journal_title：Acta neuropathologica

authors： Kohn DF,Chinookoswong N,Chou SM

更新日期：1981-01-01 00:00:00

abstract：:We examined the immunohistochemical localization of the proinflammatory cytokines tumor necrosis factor-alpha, lymphotoxin and interferon-gamma in 22 autopsy brains of patients with either cerebrovascular disease (CVD) or other neurological diseases as well as 2 non-neurological control brains. These cytokines were co...

journal_title：Acta neuropathologica

authors： Tomimoto H,Akiguchi I,Wakita H,Kinoshita A,Ikemoto A,Nakamura S,Kimura J

更新日期：1996-09-01 00:00:00

abstract：:We describe features of a patient that broadens the clinical and pathological spectrum of neurofilament inclusion disease (NFID). The patient was a 52-year-old man with a 5--6 year history of progressive, asymmetrical spastic weakness of the upper and lower extremities; L-DOPA-unresponsive parkinsonism; and SPECT evid...

journal_title：Acta neuropathologica

authors： Josephs KA,Uchikado H,McComb RD,Bashir R,Wszolek Z,Swanson J,Matsumoto J,Shaw G,Dickson DW

更新日期：2005-04-01 00:00:00

abstract：:We studied a case of primary leptomeningeal glioma (PLG) on the left parietal lobe of a 74-year-old woman and compared the tissue with heterotopic glial tissue from another case. The PLG tumor consisted of spindle-shaped cells with marked nuclear atypism, which tended to be arranged in a fascicular pattern, and the ma...

journal_title：Acta neuropathologica

authors： Kakita A,Wakabayashi K,Takahashi H,Ohama E,Ikuta F,Tokiguchi S

更新日期：1992-01-01 00:00:00

abstract：:Olfactory dysfunction is a frequent and early feature of patients with neurodegenerative disorders such as Alzheimer's disease (AD) and Parkinson's disease (PD) and is very uncommon in patients with frontotemporal dementia (FTD). Mechanisms underlying this clinical manifestation are poorly understood but the premature...

journal_title：Acta neuropathologica

authors： Mundiñano IC,Caballero MC,Ordóñez C,Hernandez M,DiCaudo C,Marcilla I,Erro ME,Tuñon MT,Luquin MR

更新日期：2011-07-01 00:00:00

abstract：:The authors describe in biopsies from 6 cases of Werdnig-Hoffmann disease, including 2 of the more benign type, the ultrastructural typical aspects of denervation. They compare their findings with those of other workers. The striking points are the great variation in the diameter of the muscle fibres and the myofibril...

journal_title：Acta neuropathologica

authors： Szliwowski HB,Drochmans P

更新日期：1975-01-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease with no effective treatments. Numerous RNA-binding proteins (RBPs) have been shown to be altered in ALS, with mutations in 11 RBPs causing familial forms of the disease, and 6 more RBPs showing abnormal expression/distribution in ALS albeit...

journal_title：Acta neuropathologica

authors： Bakkar N,Kovalik T,Lorenzini I,Spangler S,Lacoste A,Sponaugle K,Ferrante P,Argentinis E,Sattler R,Bowser R

更新日期：2018-02-01 00:00:00

abstract：:Inappropriate apoptosis has been implicated in the mechanism of neuronal death in Huntington's disease (HD). In this study, we report the expression of apoptotic markers in HD caudate nucleus (grades 1-4) and compare this with controls without neurological disease. Terminal transferase-mediated biotinylated-UTP nick e...

journal_title：Acta neuropathologica

authors： Vis JC,Schipper E,de Boer-van Huizen RT,Verbeek MM,de Waal RM,Wesseling P,ten Donkelaar HJ,Kremer B

更新日期：2005-03-01 00:00:00

abstract：:Peroxiredoxin-ll (Prxll) and glutathione peroxidase-l (GPxl) are regulators of the redox system that is one of the most crucial supporting systems in neurons. This system is an antioxidant enzyme defense system and is synchronously linked to other important cell supporting systems. To clarify the common self-survival ...

journal_title：Acta neuropathologica

authors： Kato S,Kato M,Abe Y,Matsumura T,Nishino T,Aoki M,Itoyama Y,Asayama K,Awaya A,Hirano A,Ohama E

更新日期：2005-08-01 00:00:00

abstract：:In a study designed to identify the neuropathological features typical of chronic inflammatory demyelinating polyneuropathy (CIDP), we reviewed the sural nerve biopsy findings in 105 patients with this disorder. The patients' mean age at biopsy was 49 years. In 65% of patients the disease had a progressive and in 35% ...

journal_title：Acta neuropathologica

authors： Rizzuto N,Morbin M,Cavallaro T,Ferrari S,Fallahi M,Galiazzo Rizzuto S

更新日期：1998-12-01 00:00:00

abstract：:We report an experimental model of Creutzfeldt-Jakob's disease (CJD) in mice leading to the formation of giant autophagic vacuoles (AV) in neurons of the cerebral cortex. These AV appear at the end of the incubation period (4-6 months postinoculation), together with spongy changes and clinical symptoms. Autophagy, a p...

journal_title：Acta neuropathologica

authors： Boellaard JW,Schlote W,Tateishi J

更新日期：1989-01-01 00:00:00

abstract：:Mislocalization and abnormal deposition of TDP-43 into the cytoplasm (TDP-43 proteinopathy) is a hallmark in neurons of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). However, the pathogenic mechanism of the diseases linked to TDP-43 is largely unknown. We hypothesized that the failu...

journal_title：Acta neuropathologica

authors： Nagano S,Jinno J,Abdelhamid RF,Jin Y,Shibata M,Watanabe S,Hirokawa S,Nishizawa M,Sakimura K,Onodera O,Okada H,Okada T,Saito Y,Takahashi-Fujigasaki J,Murayama S,Wakatsuki S,Mochizuki H,Araki T

更新日期：2020-11-01 00:00:00

abstract：:The endothelial cells of three cases of cerebellar capillary hemangioblastoma were studied by means of electron microscopy. Crystalloid bodies, not previously described in the vessels of the central nervous system (CNS), were found in 5%-10% of the endothelial cells, more often in the capillaries with small irregular ...

journal_title：Acta neuropathologica

authors： Ho KL

更新日期：1985-01-01 00:00:00

abstract：:A large series of central and peripheral nervous system tumors was studied for the presence of glial fibrillary acidic protein (GFAP) and gamma-enolase (neuron-specific enolase, NSE), using specific monoclonal antibodies (mAbs). Occurrence in and specificity of GFAP to glial and mixed tumors was confirmed and depended...

journal_title：Acta neuropathologica

authors： Cras P,Martin JJ,Gheuens J

更新日期：1988-01-01 00:00:00

abstract：:A study was undertaken to determine the pathological significance of previously unrecognized intracytoplasmic eosinophilic inclusions (IEIs) in ependymoma. The study group consisted of 58 ependymomas, all of which were pathologically characterized and graded according to the 1993 WHO classification. Electron microscop...

journal_title：Acta neuropathologica

authors： Kawano N,Ohba Y,Nagashima K

更新日期：2000-02-01 00:00:00

abstract：:Two experiments were completed in which brains and spinal cords from lambs affected with spongy degeneration of the central nervous system (CNS) were analysed for water, sodium and potassium, as measures of cerebral oedema. In a third experiment, lambs with CNS spongy degeneration were tested for permeability of cereb...

journal_title：Acta neuropathologica

authors： Hooper PT

更新日期：1975-01-01 00:00:00

abstract：:Excitotoxic stimulation of NMDA receptors results in the activation of a variety of cellular responses. The inducible transcription factor NF-kappaB is known to be involved in excitotoxic responses by neurons. Here, we show that NF-kappaB activation occurs in a biphasic manner in hippocampal slices following a 20-min ...

journal_title：Acta neuropathologica

authors： Caba E,Bahr BA

更新日期：2004-09-01 00:00:00

abstract：:The Norrbottnian type of Gaucher disease is characterized by infantile or juvenile onset and variable degrees of neurological symptoms, some of which develop only after splenectomy. A full neuropathological description of this type of Gaucher disease has not been reported previously. The brains of five patients were e...

journal_title：Acta neuropathologica

authors： Conradi NG,Sourander P,Nilsson O,Svennerholm L,Erikson A

更新日期：1984-01-01 00:00:00