Abstract：
:This article highlights the features that connect prion diseases with other cerebral amyloidoses and how these relate to neurodegeneration, with focus on tau phosphorylation. It also discusses similarities between prion disease and Alzheimer's disease: mechanisms of amyloid formation, neurotoxicity, pathways involved in triggering tau phosphorylation, links to cell cycle pathways and neuronal apoptosis. We review previous evidence of prion diseases triggering hyperphosphorylation of tau, and complement these findings with cases from our collection of genetic, sporadic and transmitted forms of prion diseases. This includes the novel finding that tau phosphorylation consistently occurs in sporadic CJD, in the absence of amyloid plaques.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Reiniger L,Lukic A,Linehan J,Rudge P,Collinge J,Mead S,Brandner Sdoi
10.1007/s00401-010-0691-0subject
Has Abstractpub_date
2011-01-01 00:00:00pages
5-20issue
1eissn
0001-6322issn
1432-0533journal_volume
121pub_type
杂志文章,评审abstract：:A 48-year-old man with dural arteriovenous malformation (AVM) is reported. Radiologically, the dural AVM was demonstrated mainly in the region of the left transverse sinus. Postmortem examination revealed dural AVM involving the bilateral transverse, superior sagittal and straight sinuses. In addition, numerous malfor...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00307635
更新日期：1990-01-01 00:00:00
abstract：:We present here an unusual case of papillary neuroepithelial tumor of the pineal region. The patient was a 29-year-old female who presented with headaches. A computed tomography scan revealed a tumorous lesion at the pineal region and hydrocephalus. The resected tumor was composed of columnar and cuboidal cells showin...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-004-0898-z
更新日期：2004-10-01 00:00:00
abstract：:In the present study we assessed the neuroprotective effects of the pan-caspase inhibitor z-VAD.fmk [N-benzyloxycarbony-valine-alanine-aspartate-(OMe)-fluoromethylketone], and the caspase-3 inhibitor Ac-DEVD.CHO (acetyl-aspartate-chloromethylketone) in the double-lesion rat model of striatonigral degeneration (SND), t...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-004-0931-2
更新日期：2005-02-01 00:00:00
abstract：:Essential mixed cryoglobulinemia, which can cause hypersensitivity vasculitis, was observed in five patients with peripheral neuropathy. Three cases presented with multifocal neuropathies and two cases with symmetrical polyneuropathy. One had cryoglobulinemia with IgM monoclonal gammopathy IgG polyclonal gammopathy, a...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00686206
更新日期：1988-01-01 00:00:00
abstract：:Three female cats, littermates born from clinically normal parents, were examined at 8 to 10 weeks of age because of a slowly progressive posterior ataxia. Another cat from a previous litter from the same parents suffered from similar neurological symptoms. Histopathological examination of the nervous tissues of these...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00293957
更新日期：1991-01-01 00:00:00
abstract：:Laminin alpha2 (merosin)-deficient congenital muscular dystrophy (CMD) patients show progressive muscle fiber necrosis and ineffective muscle regeneration. This is probably due to decreased formation of multi nucleated myotubes resulting from a myoblast fusion defect. When receiving a mechanical signal from muscle mem...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-004-0884-5
更新日期：2004-09-01 00:00:00
abstract：:It is conventionally accepted that during the early stages of Wallerian degeneration of myelinated peripheral nerve fibres Schmidt-Lanterman incisures represent the sites at which the myelin sheath, together with enclosed axoplasm, is segmented into myelin ovoids. This mechanism is considered by some authors to be fac...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：
更新日期：1979-11-01 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
pub_type： 杂志文章
doi：10.1007/s00401-004-0880-9
更新日期：2004-09-01 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
pub_type： 杂志文章
doi：10.1007/BF00307700
更新日期：1990-01-01 00:00:00
abstract：:A 77-year-old man suffered intermittent hemiconvulsions of unknown etiology on the left side for a period of about 5 weeks. At the autopsy, there was marked neuronal loss, severe proliferation of astrocytes and spongiform changes in the right cerebral cortex. The cerebral white matter showed loosening with astroglial ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00687782
更新日期：1988-01-01 00:00:00
abstract：:Wolfram syndrome or DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy and deafness) is a neurodegenerative disorder characterized by diabetes mellitus and optic atrophy as well as diabetes insipidus and deafness in many cases. We report the post-mortem neuropathologic findings of a patient with Wolfram syn...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-009-0546-8
更新日期：2009-09-01 00:00:00
abstract：:Glioblastoma (GBM), the most common malignant brain tumor, is among the most lethal neoplasms, with a median survival of approximately 1 year. Prognosis is poor since GBMs possess a strong migratory and highly invasive potential, making complete surgical resection impossible. Reduced expression of carboxypeptidase E (...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-011-0940-x
更新日期：2012-07-01 00:00:00
abstract：:Histochemical and electron microscopic studies of the brains inclusive of the leptomeninges containing large blood vessels from 7 patients with mucopolysaccharidosis (MPS) I, II, IIIA and V showed marked increase in mesenchymal elements and the generalized presence of characteristic lesions around cerebral veins and a...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00690379
更新日期：1977-07-15 00:00:00
abstract：:Cerebral cortical lesions of tuberous sclerosis (TSC) and focal cortical dysplasia (FCD) show disturbances in laminar architecture and cellular differentiation. We immunohistochemically studied the expression of doublecortin, a fetal neuronal protein that regulates neuronal migration, in the surgical specimens of five...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-002-0575-z
更新日期：2002-10-01 00:00:00
abstract：:The occurrence is reported of acute myositis in a man with meningoencephalitis due to toxoplasmosis. The ultrastructure and immunohistochemistry of a muscle biopsy of the patient were investigated. Toxoplasma organisms were not found in the muscle biopsy. The perivascular inflammatory cells in the muscle were mainly C...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00334513
更新日期：1990-01-01 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
pub_type： 杂志文章
doi：10.1007/BF00692214
更新日期：1980-01-01 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
pub_type： 杂志文章
doi：10.1007/BF00687928
更新日期：1975-01-01 00:00:00
abstract：:Mouse embryos were infected with murine cytomegalovirus (MCMV) by injecting the virus into the cerebral ventricles at the late gestation. After deliveries, offspring were fed by the mothers until 4 weeks. Cystic brain lesions, regarded as porencephaly or paraventricular cysts, were observed in about 20% of the MCMV-in...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00293377
更新日期：1991-01-01 00:00:00
abstract：:Matrix metalloproteinases (MMPs) are endoproteases that have been implicated in the pathogenesis of inflammatory and vasculitic neuropathies. In systemic lupus erythematosus (SLE), a peripheral neuropathy is frequently seen that is thought to be caused by ischemic nerve damage due to vasculopathy and/or vasculitis of ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-002-0653-2
更新日期：2003-04-01 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
pub_type： 杂志文章
doi：10.1007/s00401-007-0197-6
更新日期：2007-06-01 00:00:00
abstract：:Focal cortical dysplasias (FCDs) are increasingly recognized as one of the most common causes of pharmaco-resistant epilepsies. FCDs were recently divided into various clinico-pathological subtypes due to distinct imaging, electrophysiological, and outcome characteristics. In this review, we will overview the internat...
journal_title：Acta neuropathologica
pub_type： 杂志文章,评审
doi：10.1007/s00401-014-1304-0
更新日期：2014-07-01 00:00:00
abstract：:The calcium-activated protease calpain cleaves a variety of biologically important proteins and serves, therefore, as a key regulator of many cellular functions. Activation of both main isoforms, calpain 1 and calpain 2, was demonstrated previously in Alzheimer's disease. In this report, antibodies specifically recogn...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-002-0528-6
更新日期：2002-07-01 00:00:00
abstract：:The extent of DNA fragmentation analysed using the TUNEL technique was evaluated in post-mortem human brain tissue. Twenty-four patients with clinical and histopathological diagnosis of Alzheimer's disease (AD) and a short post-mortem delay were analysed. We report an increase in the count of TUNEL-labelled cells as t...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010000228
更新日期：2000-12-01 00:00:00
abstract：:Brain tumors are the most common solid tumors of childhood, and the genetic drivers and optimal therapeutic strategies for many of the different subtypes remain unknown. Here, we identify that bithalamic gliomas harbor frequent mutations in the EGFR oncogene, only rare histone H3 mutation (in contrast to their unilate...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-020-02155-5
更新日期：2020-06-01 00:00:00
abstract：:Following maternal chicken pox in the 14th week of pregnancy, a male infant was born with low birth weight, muscle wasting and limb contractures, hypotonia and areflexia. A rising titre of varicella-zoster-specific IgM (by enzyme-linked immunoabsorbent assay) confirmed congenital infection, and electromyogram showed w...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00687781
更新日期：1988-01-01 00:00:00
abstract：:Two young zebra siblings from consecutive pregnancies suffered from neurological disease, characterized by ventral deviation of the neck and tetraparesis which progressed to tetraplegia within a few weeks. On histological examination widespread neuronal degeneration was observed in the ventral horns of the spinal cord...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00684532
更新日期：1977-01-31 00:00:00
abstract：:Low-grade neuroepithelial tumors (LGNTs) are diverse CNS tumors presenting in children and young adults, often with a history of epilepsy. While the genetic profiles of common LGNTs, such as the pilocytic astrocytoma and 'adult-type' diffuse gliomas, are largely established, those of uncommon LGNTs remain to be define...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-016-1539-z
更新日期：2016-06-01 00:00:00
abstract：:Cystic necrosis in the cerebellar white matter was found in three premature infants. The necrosis was characteristically localized in the center of the white matter of the superficial cerebellar folia, sparing the overlying cortex. The patients were aged between 28 and 34 gestational weeks, and had a clinical history ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00315013
更新日期：1995-01-01 00:00:00
abstract：:APO2 ligand (APO2L)/TRAIL is a novel member of the tumor necrosis factor cytokine family and a potent inducer of apoptosis in tumor cell lines. We recently reported that APO2L is consistently expressed in low-grade astrocytomas, anaplastic astrocytomas, glioblastomas, and cell lines derived thereof, and that malignant...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/pl00007399
更新日期：2000-01-01 00:00:00
abstract：:The morphology of toxoplasma and its interaction with the cellular elements of the brain were studied in a patient who dies of extensive cerebral toxoplasmosis superimposed on Hodgkin's disease. The cerebral lesions were devoid of inflammatory cellular response and contained numerous organisms mostly in isolated multi...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00690360
更新日期：1978-06-30 00:00:00
