Creutzfeldt-Jakob disease in a patient with an R208H mutation of the prion protein gene (PRNP) and a 17-kDa prion protein fragment.

abstract：:The recently identified GGGGCC repeat expansion in the noncoding region of C9ORF72 is the most common pathogenic mutation in patients with frontotemporal dementia (FTD) or amyotrophic lateral sclerosis (ALS). We generated a human neuronal model and investigated the pathological phenotypes of human neurons containing G...

journal_title：Acta neuropathologica

authors： Almeida S,Gascon E,Tran H,Chou HJ,Gendron TF,Degroot S,Tapper AR,Sellier C,Charlet-Berguerand N,Karydas A,Seeley WW,Boxer AL,Petrucelli L,Miller BL,Gao FB

更新日期：2013-09-01 00:00:00

abstract：:We studied the distribution pattern of pathology and cholinergic deficits in the subnuclei of the amygdaloid complex (AC) in five patients with Alzheimer's disease (AD), eight with dementia with Lewy bodies (DLB) and five normal controls. In controls, the basal nucleus contained the highest choline acetyltransferase a...

journal_title：Acta neuropathologica

authors： Sahin HA,Emre M,Ziabreva I,Perry E,Celasun B,Perry R

更新日期：2006-02-01 00:00:00

abstract：:A man of 75 years of age had had lightning pains in the legs for 8 years. Clinical examination demonstrated mild pyramidal signs and involvement of the posterior columns. A morphine pump was placed in the epidural space in the lumbar region to treat the pain. He died two weeks later from a massive pulmonary embolism. ...

journal_title：Acta neuropathologica

authors： Martin JJ,Cras P,De Schutter E

更新日期：1987-01-01 00:00:00

abstract：:Synucleinopathies, such as Parkinson's disease (PD), multiple system atrophy (MSA), and dementia with Lewy bodies (DLB), are defined by the presence of α-synuclein (αSYN) aggregates throughout the nervous system but diverge from one another with regard to their clinical and pathological phenotype. The recent generatio...

journal_title：Acta neuropathologica

authors： Van der Perren A,Gelders G,Fenyi A,Bousset L,Brito F,Peelaerts W,Van den Haute C,Gentleman S,Melki R,Baekelandt V

更新日期：2020-06-01 00:00:00

abstract：:Large cell/anaplastic (LC/A) medulloblastoma (MB) is a recently recognized variant of medulloblastoma known to be associated with an advanced stage and a poor prognosis. Although Eberhart et al. suggested histopathologic grading of medulloblastoma in 2002, no consensus has been reached in terms of determining the crit...

journal_title：Acta neuropathologica

authors： Min HS,Lee YJ,Park K,Cho BK,Park SH

更新日期：2006-07-01 00:00:00

abstract：:Neuregulins belong to a family of multipotent growth-promoting proteins, and have been shown to have a crucial role in accumulating acetylcholine receptor at neuromuscular junctions. A functional role of neuregulins in muscle regeneration has not yet been identified. Using reverse transcription (RT)-PCR, Western blot ...

journal_title：Acta neuropathologica

authors： Hirata M,Sakuma K,Okajima S,Fujiwara H,Inashima S,Yasuhara M,Kubo T

更新日期：2007-04-01 00:00:00

abstract：:The neuropathological staging model of Alzheimer's disease proposed by Braak and Braak [Acta Neuropathol (1991) 82:259] requires that the evolution of neurofibrillary pathology follows a predictable pattern that can be ordered in a regular regional hierarchy. We have operationalized the neuropathological staging syste...

journal_title：Acta neuropathologica

authors： Gertz HJ,Xuereb J,Huppert F,Brayne C,McGee MA,Paykel E,Harrington C,Mukaetova-Ladinska E,Arendt T,Wischik CM

更新日期：1998-02-01 00:00:00

abstract：:The IgM fraction of normal swine serum (NSS) was found to adhere to human peripheral nerve myelin sheaths from randomly selected neuropathies and control nerves in electron microscopic immunocytochemical assays. The reaction could be blocked by preincubation with undiluted fetal calf serum (FCS), whereas endoneurial b...

journal_title：Acta neuropathologica

authors： Sommer C,Schröder JM

更新日期：1988-01-01 00:00:00

abstract：:Four melanin pigment-containing intracranial tumors were found in three Long-Evans rats in the course of experimental oncogenesis by transplacental ethylnitrosourea (ENU). One of them was a leptomeningeal melanoma. Aside from the presence of scattered melanin-pigmented cells, the other three had the typical histologic...

journal_title：Acta neuropathologica

authors： Spence AM,Rubinstein LJ,Conley FK,Herman MM

更新日期：1976-05-18 00:00:00

abstract：:Involvement of the choroid plexus by lymphoma (Hodgkin) is a hitherto undescribed complication. We report herein the case of a 49-year-old man who developed, shortly before death, neurologic symptoms seemingly related to extensive involvement of choroid plexus and surrounding ventricular structures by lymphoma (Hodgki...

journal_title：Acta neuropathologica

authors： Sanchez JE,Garcia JH,Kwee H

更新日期：1977-02-28 00:00:00

abstract：:Spinal and bulbar muscular atrophy (SBMA) is a neuromuscular disease caused by the expansion of a polyglutamine tract in the androgen receptor (AR). The mechanism by which expansion of polyglutamine in AR causes muscle atrophy is unknown. Here, we investigated pathological pathways underlying muscle atrophy in SBMA kn...

journal_title：Acta neuropathologica

authors： Rocchi A,Milioto C,Parodi S,Armirotti A,Borgia D,Pellegrini M,Urciuolo A,Molon S,Morbidoni V,Marabita M,Romanello V,Gatto P,Blaauw B,Bonaldo P,Sambataro F,Robins DM,Lieberman AP,Sorarù G,Vergani L,Sandri M,Pennuto

更新日期：2016-07-01 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：: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

authors： Moreau PM,Vallat JM,Hugon J,Leboutet MJ,Vandevelde M

更新日期：1991-01-01 00:00:00

abstract：:Motor neuron disease (MND) may be complicated by frontotemporal dementia and/or an extrapyramidal movement disorder. Several studies have identified the pathological substrate for dementia in MND as being ubiquitin-immunoreactive inclusions and dystrophic neurites in the extramotor neocortex and hippocampus. Although ...

journal_title：Acta neuropathologica

authors： Mackenzie IR,Feldman H

更新日期：2004-04-01 00:00:00

abstract：:In addition to motor neurone degeneration, up to 50% of amyotrophic lateral sclerosis (ALS) patients present with cognitive decline. Understanding the neurobiological changes underlying these cognitive deficits is critical, as cognitively impaired patients exhibit a shorter survival time from symptom onset. Given the ...

journal_title：Acta neuropathologica

authors： Henstridge CM,Sideris DI,Carroll E,Rotariu S,Salomonsson S,Tzioras M,McKenzie CA,Smith C,von Arnim CAF,Ludolph AC,Lulé D,Leighton D,Warner J,Cleary E,Newton J,Swingler R,Chandran S,Gillingwater TH,Abrahams S,Spires-

更新日期：2018-02-01 00:00:00

abstract：:Marinesco-Sjögren syndrome (MSS) features cerebellar ataxia, mental retardation, cataracts, and progressive vacuolar myopathy with peculiar myonuclear alterations. Most MSS patients carry homozygous or compound heterozygous SIL1 mutations. SIL1 is a nucleotide exchange factor for the endoplasmic reticulum resident cha...

journal_title：Acta neuropathologica

authors： Roos A,Buchkremer S,Kollipara L,Labisch T,Gatz C,Zitzelsberger M,Brauers E,Nolte K,Schröder JM,Kirschner J,Jesse CM,Goebel HH,Goswami A,Zimmermann R,Zahedi RP,Senderek J,Weis J

更新日期：2014-05-01 00:00:00

abstract：:Impaired axonal transport of the fast or slow component has been reported in patients with sporadic amyotrophic lateral sclerosis (ALS), animal models for ALS, and familial ALS-linked mutant Cu/Zn superoxide dismutase (SOD1) transgenic mice. However, little is known about the impairment of axonal transport in mutant S...

journal_title：Acta neuropathologica

authors： Sasaki S,Warita H,Abe K,Iwata M

更新日期：2005-07-01 00:00:00

abstract：:We have recently shown that glioma cell lines, as well as cells of human malignant gliomas in situ, synthesize tropoelastin. In addition, glioma cells degrade tropoelastin using metalloproteinase(s), and the resulting peptides, incapable of assembling in the extracellular fibers, interact with the 67-kDa cell surface ...

journal_title：Acta neuropathologica

authors： Hinek A,Jung S,Rutka JT

更新日期：1999-04-01 00:00:00

abstract：:The blood-brain barrier (BBB) was studied in rats with electrolyte-induced demyelination (EID), an experimental model for central pontine myelinolysis. Intravenously injected peroxidase was extravasated at 3 h post hypertonic saline injection (PHS) into regions frequently involved in EID. Increased pinocytotic activit...

journal_title：Acta neuropathologica

authors： Rojiani AM,Prineas JW,Cho ES

更新日期：1994-01-01 00:00:00

abstract：:An unusual case of cerebellar neurocytoma with rhabdomyomatous differentiation in a 6-year-old boy is reported. Immunocytochemical and ultrastructural features of the tumour were studied. Abortive synapse formation, the presence of clear vesicles and synaptophysin immunoreactivity of the tumour cells indicated its int...

journal_title：Acta neuropathologica

authors： Pal L,Santosh V,Gayathri N,Das S,Das BS,Jayakumar PN,Shankar SK

更新日期：1998-03-01 00:00:00

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 ...

journal_title：Acta neuropathologica

authors： Reiniger L,Lukic A,Linehan J,Rudge P,Collinge J,Mead S,Brandner S

更新日期：2011-01-01 00:00:00

abstract：:Modern neuropathology serves a key function in the multidisciplinary management of brain tumor patients. Owing to the recent advancements in molecular neurooncology, the neuropathological assessment of brain tumors is no longer restricted to provide information on a tumor's histological type and malignancy grade, but ...

journal_title：Acta neuropathologica

authors： Riemenschneider MJ,Jeuken JW,Wesseling P,Reifenberger G

更新日期：2010-11-01 00:00:00

abstract：:In this study, we used immunohistochemistry to screen for alpha-synuclein pathology in the brains of 241 individuals without clinical evidence of neurologic disease, and discovered 36 cases (15%) with incidental Lewy bodies (LBs) and one case, a 96-year-old woman (0.4%), with inclusions similar to those seen in multip...

journal_title：Acta neuropathologica

authors： Fujishiro H,Ahn TB,Frigerio R,DelleDonne A,Josephs KA,Parisi JE,Eric Ahlskog J,Dickson DW

更新日期：2008-09-01 00:00:00

abstract：:Charcot-Marie-Tooth disease type 1 B (CMT1B) is a demyelinating neuropathy caused by mutations in the myelin protein zero (P0) gene (MPZ). A few cases of CMT1B were recently found to be characterized by focally folded myelin sheaths in nerve biopsy specimens; the significance of this association is unknown. Here, we d...

journal_title：Acta neuropathologica

authors： Fabrizi GM,Taioli F,Cavallaro T,Rigatelli F,Simonati A,Mariani G,Perrone P,Rizzuto N

更新日期：2000-09-01 00:00:00

abstract：:The present study investigated the expression of c-erbB-2 in 59 meningiomas, including different histological subtypes and anaplastic variants, by immunocytochemistry and molecular biological techniques. Immunohistochemistry using the monoclonal antibody FWP-51 directed against c-erbB-2-encoded oncoprotein gp185 demon...

journal_title：Acta neuropathologica

authors： Schlegel J,Ullrich B,Stumm G,Gass P,Harwerth IM,Hynes NE,Kiessling M

更新日期：1993-01-01 00:00:00

abstract：:Experimental allergic neuritis (EAN) was induced in normal and irradiated Lewis rats by passively transferring T cells sensitized to SP-26, a peptide fragment of P2 myelin protein. The recipients became sick 4-8 days post transfer and the degree of disability correlated directly with the dose of T cells. Smaller doses...

journal_title：Acta neuropathologica

authors： Hahn AF,Feasby TE,Lovgren D,Wilkie L

更新日期：1993-01-01 00:00:00

abstract：:Rat sciatic nerve was assayed for total collagen and total protein both immediately post-mortem and at weekly intervals after death. No significant difference was found in collagen and protein content between corresponding nerve segments assayed fresh and up to 4 weeks post mortem. However, variation was found in peri...

journal_title：Acta neuropathologica

authors： McFarlane KR,Pollock M,Myers DB

更新日期：1980-01-01 00:00:00

abstract：:A case history illustrating the potential clinical significance of subependymoma is presented. Fine structural studies indicate that the tumor is composed of cells having the cytoplasmic features of ependyma, astrocytes, and transitional cells. Its composition and structure are alike those in the adult mammalian subep...

journal_title：Acta neuropathologica

authors： Azzarelli B,Rekate HL,Roessmann U

更新日期：1977-11-28 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：:A case of classic medulloblastoma that metastasized, despite the absence of local recurrence, to extraneural sites 7 years after treatment is reported. The metastases were, in contrast to the primary tumor, of large cell type and displayed abortive myogenic and, in one site, also rhabdoid differentiation. The primary ...

journal_title：Acta neuropathologica

authors： Donner LR

更新日期：2005-05-01 00:00:00