Neuropathological analysis of an adult case of the Cornelia de Lange syndrome.

abstract：:Glioblastomas, the most malignant human brain tumors, are characterized by marked aneuploidy, suggesting chromosomal instability which may be caused by a defective mitotic spindle checkpoint. We screened 22 glioblastomas for mutations in the mitotic spindle check-point genes hBUB1, hBUBR1 and hBUB3. DNA sequencing rev...

journal_title：Acta neuropathologica

authors： Reis RM,Nakamura M,Masuoka J,Watanabe T,Colella S,Yonekawa Y,Kleihues P,Ohgaki H

更新日期：2001-04-01 00:00:00

abstract：:Massive GGGGCC repeat expansion in the first intron of the gene C9orf72 is the most common known cause of familial frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). Despite its intronic localization and lack of an ATG start codon, the repeat region is translated in all three reading fra...

journal_title：Acta neuropathologica

authors： Mori K,Arzberger T,Grässer FA,Gijselinck I,May S,Rentzsch K,Weng SM,Schludi MH,van der Zee J,Cruts M,Van Broeckhoven C,Kremmer E,Kretzschmar HA,Haass C,Edbauer D

更新日期：2013-12-01 00:00:00

abstract：:The histological and ultrastructural findings of subacute spongiform encephalopathy (SSE) are described in the cerebral cortex and basal ganglia of a homosexual patient who died with acquired immune deficiency syndrome (AIDS). It is suggested that SSE, beside the diffuse AIDS leukoencephalopathy, might be another morp...

journal_title：Acta neuropathologica

authors： Schwenk J,Cruz-Sanchez F,Gosztonyi G,Cervos-Navarro J

更新日期：1987-01-01 00:00:00

abstract：:Double immunofluorescence for paired helical filament (PHF)-tau (AT8) and ubiquitin, enhanced by catalyzed reporter deposition amplification, was combined with thiazin red (TR), a fluorochrome, which has an affinity to fibrillary structures such as neurofibrillary tangles (NFTs). After recording these triple-fluoresce...

journal_title：Acta neuropathologica

authors： Uchihara T,Nakamura A,Yamazaki M,Mori O

更新日期：2001-06-01 00:00:00

abstract：:It was earlier shown that bicuculline-induced status epilepticus gives rise to profound acute changes in the rat cerebral cortex, i.e. edema and neuronal alterations. In the present study, we explored to what extent interruption of the seizure activity reverses the changes observed. To that end, status epilepticus of ...

journal_title：Acta neuropathologica

authors： Söderfeldt B,Kalimo H,Olsson Y,Siesjö BK

更新日期：1983-01-01 00:00:00

abstract：:Progressive myopathy developed in two women who consumed ipecac syrup containing emetine hydrochloride to induce vomiting as part of their anorexia nervosa. Muscle biopsy specimens were characterized by severe disruption of the sarcomeres. The ultrastructural spectrum extended from "Z-band streaming" to the formation ...

journal_title：Acta neuropathologica

authors： Halbig L,Gutmann L,Goebel HH,Brick JF,Schochet S

更新日期：1988-01-01 00:00:00

abstract：:We performed a comparative study to assess cerebral amyloid angiopathy and ApoE genotype in cases of Alzheimer's disease (AD). Ten ApoE 3,3 and ten ApoE 4,4 AD brains, as well as ten normal control brains, were selected after matching for age, sex, and duration of disease. Sections of middle frontal and inferior parie...

journal_title：Acta neuropathologica

authors： Trembath D,Ervin JF,Broom L,Szymanski M,Welsh-Bohmer K,Pieper C,Hulette CM

更新日期：2007-01-01 00:00:00

abstract：:Leukocyte adhesion molecules to endothelium plays an important role in the pathogenesis of inflammatory diseases, including HTLV-I-associated myelopathy (HAM)/tropical spastic paraparesis (TSP). To help define the role of adhesion molecules in HAM/TSP, we studied the expression of lymphocyte function-associated antige...

journal_title：Acta neuropathologica

authors： Umehara F,Izumo S,Takeya M,Takahashi K,Sato E,Osame M

更新日期：1996-01-01 00:00:00

abstract：:Structural proteins of cultured neurofibromatosis (NF) tumor and skin cells were studied with reference to control skin fibroblasts. In polyacrylamide gel electrophoresis (PAGE)/fluorography the banding patterns of the cell lysates were markedly similar. NF tumor cells, however, produced a 60 kD band with a stronger a...

journal_title：Acta neuropathologica

authors： Peltonen J,Näntö-Salonen K,Aho HJ,Kouri T,Virtanen I,Penttinen R

更新日期：1984-01-01 00:00:00

abstract：:We investigated the distribution of prion protein (PrP) in 14 German patients with sporadic Creutzfeldt-Jakob disease (CJD) and compared it with that observed in Japanese patients. Immunohistochemical study revealed diffuse gray matter stainings including synaptic structures in all cases. In addition, 4 patients showe...

journal_title：Acta neuropathologica

authors： Doi-Yi R,Kitamoto T,Ogomori K,Mehraein P,Tateishi J

更新日期：1994-01-01 00:00:00

abstract：:Solanezumab and Crenezumab are two humanized antibodies targeting Amyloid-β (Aβ) which are currently tested in multiple clinical trials for the prevention of Alzheimer's disease. However, there is a scientific discussion ongoing about the target engagement of these antibodies. Here, we report the immunohistochemical s...

journal_title：Acta neuropathologica

authors： Bouter Y,Lopez Noguerola JS,Tucholla P,Crespi GA,Parker MW,Wiltfang J,Miles LA,Bayer TA

更新日期：2015-11-01 00:00:00

abstract：:This study reports the histological, ultrastructural, and immunocytochemical characteristics of intracytoplasmic eosinophilic inclusion bodies occurring in various types of neurons of the human central nervous system. By light microscopy, the inclusions were brightly eosinophilic, slightly birefringent, and sharply de...

journal_title：Acta neuropathologica

authors： Peña CE,Katoh A

更新日期：1989-01-01 00:00:00

abstract：:In this report, we describe the clinical, topographical and immunohistochemical characteristics of neurofilament (NF) inclusion formation induced by the intracisternal inoculation of young adult New Zealand white rabbits at 28-day intervals with 100 micrograms AlCl3 over the course of 267 days. The ability to recover ...

journal_title：Acta neuropathologica

authors： Strong MJ,Gaytan-Garcia S,Jakowec DM

更新日期：1995-01-01 00:00:00

abstract：:In order to elucidate the role of humoral antibodies in the pathogenesis of myelin lesions in experimental allergic encephalomyelitis (EAE) a combined in vivo and in vitro study was done using rabbits immunized with the purified A1 basic protein. Rabbits injected with whole white matter were used for comparison. Demye...

journal_title：Acta neuropathologica

authors： Kristensson K

更新日期：1976-12-21 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 occurrence of oligodendrocytes within astrocytes ("emperipolesis") has been described in demyelinating lesions in cases of multiple sclerosis and also in other non-demyelinating disorders. We found that this finding was common in the cerebral white matter of patients with Creutzfeldt-Jakob disease (CJD). Eight con...

journal_title：Acta neuropathologica

authors： Shintaku M,Yutani C

更新日期：2004-09-01 00:00:00

abstract：:Continuous intrathecal infusion of N-methyl-D-aspartate (NMDA) at the level of the lumbar enlargement of the spinal cord in middle-aged rats produced dose-dependent toxicity of spinal cord neuronal systems. Toxicity was enhanced by coadministration of glycine, but was significantly reduced when NMDA was co-administere...

journal_title：Acta neuropathologica

authors： Nag S,Riopelle RJ

更新日期：1990-01-01 00:00:00

abstract：:In adult mice, administration of the anticonvulsive drug phenytoin caused focal swellings along the Purkinje cell axon correlated with ataxia and incoordination of movements. In our model, we used murine cerebellar slice cultures to study the influence of phenytoin on postnatal Purkinje cell axon differentiation. Almo...

journal_title：Acta neuropathologica

authors： Tauer U,Knoth R,Volk B

更新日期：1998-06-01 00:00:00

abstract：:Pyroglutamylated amyloid-β (pE(3)-Aβ) has been suggested to play a major role in Alzheimer's disease (AD) pathogenesis as amyloid-β (Aβ) oligomers containing pE(3)-Aβ might initiate tau-dependent cytotoxicity. We aimed to further elucidate the associations among pE(3)-Aβ, full-length Aβ and hyperphosphorylated tau (HP...

journal_title：Acta neuropathologica

authors： Mandler M,Walker L,Santic R,Hanson P,Upadhaya AR,Colloby SJ,Morris CM,Thal DR,Thomas AJ,Schneeberger A,Attems J

更新日期：2014-07-01 00:00:00

abstract：:Patients with von Hippel-Lindau disease carry a germline mutation of the Von Hippel-Lindau (VHL) tumor-suppressor gene. We discuss the molecular consequences of loss of VHL gene function and their impact on the nervous system. Dysfunction of the VHL protein causes accumulation and activation of hypoxia inducible facto...

journal_title：Acta neuropathologica

authors： Vortmeyer AO,Falke EA,Gläsker S,Li J,Oldfield EH

更新日期：2013-03-01 00:00:00

abstract：:Apoptosis of neurons and glial cells has been shown to occur in the brain of patients with the acquired immune deficiency syndrome (AIDS) and was postulated as contributing to brain atrophy and white matter damage in these patients. Since apoptotic events may be induced by the Fas-Fas ligand (FasL) system, we analyzed...

journal_title：Acta neuropathologica

authors： Elovaara I,Sabri F,Gray F,Alafuzoff I,Chiodi F

更新日期：1999-10-01 00:00:00

abstract：:Canine distemper virus (CDV) causes severe immunosuppression and neurological disease in dogs, associated with demyelination, and is a model for multiple sclerosis in man. In the early stage of the infection, demyelination is associated with viral replication in the white matter. In acute demyelinating lesions there i...

journal_title：Acta neuropathologica

authors： Vandevelde M,Zurbriggen A

更新日期：2005-01-01 00:00:00

abstract：:The character of the silver positive reticulin network was analyzed with immunofluorescence and immunoperoxidase methods in an intra vitam diagnosed case of primary brain lymphoma. The network was shown to contain connective tissue proteins rich in hexose-sugars, such as type III collagen (classical "reticulin"), basa...

journal_title：Acta neuropathologica

authors： Kalimo H,Lehto M,Näntö-Salonen K,Jalkanen M,Risteli L,Risteli J,Narva EV

更新日期：1985-01-01 00:00:00

abstract：:A 65-year-old woman was admitted to our hospital for forgetfulness, depression and eccentric behavior that had been first noticed 2 years prior to admission. She showed memory impairment, perseveration and repeated violent actions, but no limb-kinetic apraxia. She died 12 years after the onset of symptoms. At autopsy,...

journal_title：Acta neuropathologica

authors： Mizuno Y,Ozeki M,Iwata H,Takeuchi T,Ishihara R,Hashimoto N,Kobayashi H,Iwai K,Ogasawara S,Ukai K,Shibayama H

更新日期：2002-03-01 00:00:00

abstract：:Rats were given isoniazid either in a single large dose or continuously in drinking water and killed 5--105 days later. The distribution of degenerating fibres in various nerves (sensory and mixed) and in various sites along nerves and spinal roots was studied by light and electron microscopy. It was found that sensor...

journal_title：Acta neuropathologica

authors： Jacobs JM,Miller RH,Cavanagh JB

更新日期：1979-10-01 00:00:00

abstract：:Abundant proliferation of cells having the histologic and tinctorial features of microglia, were seen in the brain of 34-year old man who suffered cardiac arrest 10 days before death and whose bone marrow was nearly totally depleted of white-blood-cell precursors. It is suggested, that in adult human brains there are ...

journal_title：Acta neuropathologica

authors： Neuwelt EA,Garcia JH,Mena H

更新日期：1978-09-15 00:00:00

abstract：:Granule-containing vacuoles in the cytoplasm of hippocampal neurons are a neuropathological feature of Alzheimer's disease. Granulovacuolar degeneration (GVD) is not disease-specific and can be observed in other neurodegenerative disorders and even in the brains of non-demented elderly people. However, several studies...

journal_title：Acta neuropathologica

authors： Köhler C

更新日期：2016-09-01 00:00:00

abstract：:An autopsy case of Nasu-Hakola's disease (membranous lipodystrophy) was reported. A 29-year-old Japanese woman whose younger sister had been affected with typical Nasu-Hakola's disease with skeletal and neuropsychiatric syndromes and membrano-cystic lesions in the bones developed forgetfulness and lack of initiative. ...

journal_title：Acta neuropathologica

authors： Matsushita M,Oyanagi S,Hanawa S,Shiraki H,Kosaka K

更新日期：1981-01-01 00:00:00

abstract：:An extensive laminar spongiosis was found in the outer part of the dentate gyrus in an 84-year-old patient. An old cavitary infarct in the parahippocampal gyrus disconnected the dentate gyrus from the entorhinal area. This finding prompted us to seek laminar spongiosis in Alzheimer's disease, where the neuronal loss i...

journal_title：Acta neuropathologica

authors： Duyckaerts C,Colle MA,Seilhean D,Hauw JJ

更新日期：1998-04-01 00:00:00

abstract：:The ultrastructural features of SOD1-positive aggregates were determined to clarify whether these aggregates are associated with the pathogenesis of SOD1 mutant mice. We examined the spinal cord of transgenic mice expressing a G93A mutant human SOD1 gene with fewer copies (gene copy 10). At the early presymptomatic st...

journal_title：Acta neuropathologica

authors： Sasaki S,Warita H,Murakami T,Shibata N,Komori T,Abe K,Kobayashi M,Iwata M

更新日期：2005-03-01 00:00:00