Ultrastructure of muscle and sensory nerve in Fabry's disease.

abstract：:A case of cerebellar medullomyoblastoma in a young boy was investigated by electron microscopy. The neuroectodermal component shows the characteristics of a desmoplastic medulloblastoma. The mesodermal component consists of more or less differentiated cross-striated muscle cells. Undifferentiated muscle cells are very...

journal_title：Acta neuropathologica

authors： Walter GF,Brucher JM

更新日期：1979-12-01 00:00:00

abstract：:We have previously reported a pathological investigation of peripheral neuropathy in a horse with knuckling. This report describes details of the muscle and peripheral nerve lesions in two additional cases of light horse yearlings with knuckling. The skeletal muscles showed neurogenic atrophy characterized by scattere...

journal_title：Acta neuropathologica

authors： Furuoka H,Okamoto R,Kitayama S,Asou S,Matsui T,Miyahara K

更新日期：1998-10-01 00:00:00

abstract：:We examined the immunohistochemical localization of p35nck5a, the regulatory subunit of cyclin-dependent kinase 5 (cdk5), in brains obtained postmortem from patients with Parkinson's disease (PD) and controls. We found p35nck5a immunoreactivity in Lewy bodies (LB) in the substantia nigra, locus ceruleus, and neocortex...

journal_title：Acta neuropathologica

authors： Nakamura S,Kawamoto Y,Nakano S,Akiguchi I,Kimura J

更新日期：1997-08-01 00:00:00

abstract：:The caudate nucleus and putamen belong to the selectively vulnerable brain regions which incur neuronal damage in clinical and experimental settings of both hypoglycemia and ischemia. We have previously documented the density and distribution of the hypoglycemic damage in rat caudoputamen, but the evolution of the inj...

journal_title：Acta neuropathologica

authors： Kalimo H,Auer RN,Siesjö BK

更新日期：1985-01-01 00:00:00

abstract：:Chronic wasting disease (CWD), a progressive neurological disorder of captive mule deer, black-tailed deer, hybrids of mule deer and white-tailed deer and Rocky Mountain elk, is characterized neuropathologically by widespread spongiform change of the neuropil, intracytoplasmic vacuolation in neuronal perikarya and ast...

journal_title：Acta neuropathologica

authors： Guiroy DC,Williams ES,Yanagihara R,Gajdusek DC

更新日期：1991-01-01 00:00:00

abstract：:Missense mutations of the V600E type constitute the vast majority of tumor-associated somatic alterations in the v-RAF murine sarcoma viral oncogene homolog B1 (BRAF) gene. Initially described in melanoma, colon and papillary thyroid carcinoma, these alterations have also been observed in primary nervous system tumors...

journal_title：Acta neuropathologica

authors： Schindler G,Capper D,Meyer J,Janzarik W,Omran H,Herold-Mende C,Schmieder K,Wesseling P,Mawrin C,Hasselblatt M,Louis DN,Korshunov A,Pfister S,Hartmann C,Paulus W,Reifenberger G,von Deimling A

更新日期：2011-03-01 00:00:00

abstract：:The presence of many neurofibrillary tangles (NFTs) in the central nervous system is a hallmark of amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia complex (PDC) in people living in the Kii peninsula of Japan and in the island of Guam. To determine whether or not ALS and PDC are on a spectrum of a single ...

journal_title：Acta neuropathologica

authors： Mimuro M,Kokubo Y,Kuzuhara S

更新日期：2007-06-01 00:00:00

abstract：:Based on the Kellie-Munro doctrine according to which the intracranial compartments--blood, brain, and CSF--are in a state of pressure and volume equilibrium, we examined if traumatic lesions of the liquor absorption passages could change the intracranial pressure (ICP). Therefore, we reviewed the arachnoid villi and ...

journal_title：Acta neuropathologica

authors： Dirnhofer R,Sigrist T

更新日期：1979-02-15 00:00:00

abstract：:In Alzheimer's disease (AD) and other tauopathies, the cytosolic protein Tau misfolds and forms intracellular aggregates which accumulate within the brain leading to neurodegeneration. Clinical progression is tightly linked to the progressive spread of Tau pathology throughout the brain, and several lines of evidence ...

journal_title：Acta neuropathologica

authors： Courade JP,Angers R,Mairet-Coello G,Pacico N,Tyson K,Lightwood D,Munro R,McMillan D,Griffin R,Baker T,Starkie D,Nan R,Westwood M,Mushikiwabo ML,Jung S,Odede G,Sweeney B,Popplewell A,Burgess G,Downey P,Citron M

更新日期：2018-11-01 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：: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：:Immunohistochemical studies of monoamine neurons were performed to evaluate toxic effects of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) on young adult mice and compare them with those of their offspring. Mice, 9-11 weeks old (C57BL/6J), injected subcutaneously with a large dose of MPTP (17 mg/kg per day) duri...

journal_title：Acta neuropathologica

authors： Furune S,Miura K,Watanabe K,Nagao S,Takahashi H,Sakai M,Spatz M,Nagatsu I

更新日期：1989-01-01 00:00:00

abstract：:The growth potential of 65 pituitary adenomas was determined by histochemical analysis with Ki-67 and anti-DNA polymerase alpha monoclonal antibodies, bromodeoxyuridine (BrdUdR) labeling, and counts of argyrophilic nucleolar organizer regions (Ag-NORs). The mean proliferating cell indices (PCIs) determined by Ki-67 an...

journal_title：Acta neuropathologica

authors： Shibuya M,Saito F,Miwa T,Davis RL,Wilson CB,Hoshino T

更新日期：1992-01-01 00:00:00

abstract：:The pro-inflammatory and blood-brain barrier (BBB) effects of intratumoral (IT) injection of human recombinant tumor necrosis factor-alpha (rTNF-alpha) were studied in the Fischer rat RT-2 glioma model. Animals received a single stereotaxic injection of either 6 x 10(4) U rTNF-alpha or excipient (vehicle) into the cen...

journal_title：Acta neuropathologica

authors： Wright JL,Merchant RE

更新日期：1997-01-01 00:00:00

abstract：:Three weeks after inoculation of 24-day-old gnotobiotic dogs with Snyder-Hill canine distemper virus, white matter samples were taken from the primary predilection sites for canine distemper virus-associated demyelination. The plasmalogenase activity in extracts was nearly 6-fold greater than control values for a dog ...

journal_title：Acta neuropathologica

authors： Fu SC,Mozzi R,Krakowka S,Higgins RJ,Horrocks LA

更新日期：1980-01-01 00:00:00

abstract：:An experiment was designed to examine the course of degeneration, phagocytosis, and regeneration in the central nervous system following surgical deafferentation. The anterior cerebellar vermis was ablated in young male rats. The animals were sacrificed by perfusion at postoperative times ranging from 24 hrs to 6 mont...

journal_title：Acta neuropathologica

authors： Johnson JE Jr

更新日期：1975-12-19 00:00:00

abstract：:The dynamics of cerebrospinal fluid (CSF) tau and Aβ biomarkers over time in Alzheimer's disease (AD) patients from prodromal pre-symptomatic to severe stages of dementia have not been clearly defined and recent studies, most of which are cross-sectional, present conflicting findings. To clarify this issue, we analyze...

journal_title：Acta neuropathologica

authors： Toledo JB,Xie SX,Trojanowski JQ,Shaw LM

更新日期：2013-11-01 00:00:00

abstract：:Mutations in the fused in sarcoma (FUS) gene are linked to a form of familial amyotrophic lateral sclerosis (ALS), ALS6. The FUS protein is a major component of the ubiquitin-positive neuronal cytoplasmic inclusions in both ALS6 and some rare forms of frontotemporal lobar degeneration (FTLD). The latter are now collec...

journal_title：Acta neuropathologica

authors： Aoki N,Higashi S,Kawakami I,Kobayashi Z,Hosokawa M,Katsuse O,Togo T,Hirayasu Y,Akiyama H

更新日期：2012-09-01 00:00:00

abstract：:Prions cause scrapie and Creutzfeldt-Jakob disease (CJD); these infectious pathogens are composed largely, if not entirely, of protein molecules. No prion-specific polynucleotide has been identified. Purified preparations of scrapie prions contain high titers (greater than or equal to 10(9.5) ID50/ml), one protein (Pr...

journal_title：Acta neuropathologica

authors： Prusiner SB,Gabizon R,McKinley MP

更新日期：1987-01-01 00:00:00

abstract：:Seven patients aged 29 to 76 years with various clinical subtypes of chronic inflammatory demyelinating polyneuropathy (CIDP) were investigated. Sural nerve biopsies were performed between 7 months and 19 years after onset of disease. Quantitative electron microscopy revealed involvement of primary unmyelinated fibers...

journal_title：Acta neuropathologica

authors： Gibbels E,Kentenich M

更新日期：1990-01-01 00:00:00

abstract：:To clarify the significance of the constituents of canine senile plaques (SPs) or cerebrovascular amyloid deposits, paraffin and cryostat sections of canine brains were examined by immunohistochemistry using antibodies against cathepsin B (CB), cathepsin D (CD), cystatin C (CC), alpha-1-antichymotrypsin (ACT), heat sh...

journal_title：Acta neuropathologica

authors： Uchida K,Kuroki K,Yoshino T,Yamaguchi R,Tateyama S

更新日期：1997-03-01 00:00:00

abstract：:Polyclonal antibodies directed towards synaptophysin were raised against a synthesised peptide corresponding to amino acids 246 to 260 of the human synaptophysin sequence. The antibodies, when applied for immunocytochemical staining, showed a staining pattern identical to that of the commercially available monoclonal ...

journal_title：Acta neuropathologica

authors： Stridsberg M,Lundqvist G,Engström U,Wilander E,Su H,Gobl A,Oberg K

更新日期：1994-01-01 00:00:00

abstract：:Pleomorphic xanthoastrocytoma (PXA), a tumor most often presenting superficially over the cerebral hemisphere of young subjects, has certain morphological similarities to fibrous histiocytoma (or fibrous xanthoma) of the meninges and brain, namely the occurrence of lipid-laden neoplastic cells and, frequently, a dense...

journal_title：Acta neuropathologica

authors： Kepes JJ,Rubinstein LJ,Ansbacher L,Schreiber DJ

更新日期：1989-01-01 00:00:00

abstract：:The isolated paired helical filaments (PHF) that occur in the neurofibrillary tangles of Alzheimer's disease were assayed to determine if they contained N-acetyl-glucosamine and N-acetyl-galactosamine residues. The enzyme-linked lectin assay was used to detect their total content in the PHF preparation. The assay empl...

journal_title：Acta neuropathologica

authors： Sparkman DR,Hill SJ,White CL 3rd

更新日期：1990-01-01 00:00:00

abstract：:HTLV-III-like particles were observed within a cell process surrounded by a myelin sheath, in the brain of a 4-year-old boy with AIDS encephalopathy. Similar particles were also observed in the mononuclear macrophage-like cells. ...

journal_title：Acta neuropathologica

authors： Kato T,Dembitzer HM,Hirano A,Llena JF

更新日期：1987-01-01 00:00:00

abstract：:Excitotoxicity is considered a major cell death inductor in neurodegeneration. Yet the mechanisms involved in cell death and cell survival following excitotoxic insults are poorly understood. Expression of active, phosphorylation-dependent mitogen-activated extracellular signal-regulated kinases (MAPK/ERKs), stress-ac...

journal_title：Acta neuropathologica

authors： Ferrer I,Blanco R,Carmona M,Puig B,Domínguez I,Viñals F

更新日期：2002-04-01 00:00:00

abstract：:The intracellular localization of myoglobin(Mb) mRNA in the skeletal muscles of normal subjects and patients with Duchenne muscular dystrophy(DMD) or amyotrophic lateral sclerosis(ALS) was examined by in situ hybridization using a biotin-labeled cDNA probe. In cross sections of normal muscles, Mb mRNA signals were dem...

journal_title：Acta neuropathologica

authors： Mitsui T,Kawai H,Naruo T,Nishino H,Saito S

更新日期：1993-01-01 00:00:00

abstract：:Frontotemporal lobar degeneration with TAR DNA-binding protein 43 inclusions (FTLD-TDP) is the most common pathology associated with frontotemporal dementia (FTD). Repeat expansions in chromosome 9 open reading frame 72 (C9ORF72) and mutations in progranulin (GRN) are the major known genetic causes of FTLD-TDP; howeve...

journal_title：Acta neuropathologica

authors： Pottier C,Bieniek KF,Finch N,van de Vorst M,Baker M,Perkersen R,Brown P,Ravenscroft T,van Blitterswijk M,Nicholson AM,DeTure M,Knopman DS,Josephs KA,Parisi JE,Petersen RC,Boylan KB,Boeve BF,Graff-Radford NR,Veltman JA

更新日期：2015-07-01 00:00:00

abstract：:In almost all of the earlier reported cases of Kufs' disease, the adult form of ceroid lipofuscinosis, the diagnosis was ascertained by cerebral tissue examination, while peripheral biopsy examination revealed an apparent poor diffusion of specific lipofuscinic deposits, the finger print profiles (FPs). We report the ...

journal_title：Acta neuropathologica

authors： Gelot A,Maurage CA,Rodriguez D,Perrier-Pallisson D,Larmande P,Ruchoux MM

更新日期：1998-07-01 00:00:00

abstract：:Temozolomide (TMZ) increases the overall survival of patients with glioblastoma (GBM), but its role in the clinical management of diffuse low-grade gliomas (LGG) is still being defined. DNA hypermethylation of the O (6) -methylguanine-DNA methyltransferase (MGMT) promoter is associated with an improved response to TMZ...

journal_title：Acta neuropathologica

authors： van Thuijl HF,Mazor T,Johnson BE,Fouse SD,Aihara K,Hong C,Malmström A,Hallbeck M,Heimans JJ,Kloezeman JJ,Stenmark-Askmalm M,Lamfers ML,Saito N,Aburatani H,Mukasa A,Berger MS,Söderkvist P,Taylor BS,Molinaro AM,Wessel

更新日期：2015-04-01 00:00:00