An autopsy case of the "demyelinating type" of Wilson's disease.

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：:A desmoplastic primary cerebral neuroblastoma originating in the frontal lobe of a boy who died at the age of 6 years contained dense core vesicles within the cytoplasm of neoplastic cells as evidence of neuronal differentiation. Sarcomatous transformation had occurred at the time of recurrence. At autopsy, he also ha...

journal_title：Acta neuropathologica

authors： Goldammer D,Goebel HH

更新日期：1980-01-01 00:00:00

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：:Accumulation of amyloid-β (Aβ) and neurofibrillary tangles in the brain, inflammation and synaptic and neuronal loss are some of the major neuropathological hallmarks of Alzheimer's disease (AD). While genetic mutations in amyloid precursor protein and presenilin-1 and -2 (PS1 and PS2) genes cause early-onset familial...

journal_title：Acta neuropathologica

authors： Wahlster L,Arimon M,Nasser-Ghodsi N,Post KL,Serrano-Pozo A,Uemura K,Berezovska O

更新日期：2013-02-01 00:00:00

abstract：:Seven patients with the typical clinical picture and muscle biopsy findings of classical Werdnig-Hoffmann disease showed Wallerian degeneration in their biopsied sural nerves. In dorsal root ganglia of one patient there were residual nodules and several chromatolytic neurons. By electron microscopy the changes of chro...

journal_title：Acta neuropathologica

authors： Carpenter S,Karpati G,Rothman S,Watters G,Andermann F

更新日期：1978-05-24 00:00:00

abstract：:Muscle fiber abnormalities are described in three cases of neonatal maple syrup disease. There were important variations in fiber diameters. Lesions consisted in focal or diffuse destruction of myofibrils. In view of recent biochemical and clinical data, a direct relation between elevated branched-chain amino acid lev...

journal_title：Acta neuropathologica

authors： Ferrière G,de Castro M,Rodriguez J

更新日期：1984-01-01 00:00:00

abstract：:A retrospective study of 450 consecutive AIDS autopsy cases (397 males, 53 females; mean age at death 38.4 years) in Vienna, Austria, between 1984 and 1999 compares the central nervous system (CNS) findings in three cohorts: 1984-1992 (190 cases), 1993-1995 (162 cases) and 1996-1999 (98 cases, after introduction of tr...

journal_title：Acta neuropathologica

authors： Jellinger KA,Setinek U,Drlicek M,Böhm G,Steurer A,Lintner F

更新日期：2000-08-01 00:00:00

abstract：:In the cytoplasm of Schwann cells of a sural nerve biopsy from a 21-year-old female patient with chronic neuropathy we noted numerous unique, usually double membrane-bound, osmiophilic, granular or globular inclusions, approximately 30-600 microm in diameter. Some of these membrane-bound vesicular or tubular structure...

journal_title：Acta neuropathologica

authors： Schröder JM,Wang JF,Sindern E,Malin JP

更新日期：1999-10-01 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：:A 25-year-old homosexual AIDS patient presented with progressive cognitive, motor and behavioral disturbances consistent with HIV encephalopathy. CT scans demonstrated progressive diffuse brain atrophy. Neuropathology showed predominant cortical changes including severe neuronal loss corroborated by morphometry. Only ...

journal_title：Acta neuropathologica

authors： Gray F,Haug H,Chimelli L,Geny C,Gaston A,Scaravilli F,Budka H

更新日期：1991-01-01 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：:Recently, 43-kDa TAR DNA-binding protein (TDP-43) was identified as a component of ubiquitinated inclusions (UIs) in sporadic amyotrophic lateral sclerosis (SALS). To clarify whether TDP-43 immunoreactivity is present in neuronal inclusions in familial ALS (FALS), we examined immunohistochemically the brains and spina...

journal_title：Acta neuropathologica

authors： Tan CF,Eguchi H,Tagawa A,Onodera O,Iwasaki T,Tsujino A,Nishizawa M,Kakita A,Takahashi H

更新日期：2007-05-01 00:00:00

abstract：:A 50-year-old carpenter died in Western Pennsylvania of rabies on January 4, 1979. He had been hospitalized in an intensive care unit for 28 days. The diagnosis was made postmortem from light and electron microscopic examination of central nervous system tissue. Immunofluorescence studies confirmed the diagnosis later...

journal_title：Acta neuropathologica

authors： Lamas CC,Martinez AJ,Baraff R,Bajwa J

更新日期：1980-01-01 00:00:00

abstract：:The immunohistochemical distribution of alpha and beta subunits of S-100 protein (S-100 alpha, S-100 beta, respectively) in 138 cases of human brain tumors was investigated by the avidin-biotin immunoperoxidase method. Brain tumors can be divided into four groups: group 1 [S-100 alpha (+) and/or S-100 beta (+)]; astro...

journal_title：Acta neuropathologica

authors： Hayashi K,Hoshida Y,Horie Y,Takahashi K,Taguchi K,Sonobe H,Ohtsuki Y,Akagi T

更新日期：1991-01-01 00:00:00

abstract：:To investigate the pathogenetic role of human T lymphocyte virus type I (HTLV-I) in central nervous system disease, a rat model for HTLV-I-associated myelopathy/tropical spastic paraparesis, designated as HAM rat disease, has been established. Wistar-King-Aptekman-Hokudai strain rats with induced HTLV-I infection deve...

journal_title：Acta neuropathologica

authors： Kasai T,Ikeda H,Tomaru U,Yamashita I,Ohya O,Morita K,Wakisaka A,Matsuoka E,Moritoyo T,Hashimoto K,Higuchi I,Izumo S,Osame M,Yoshiki T

更新日期：1999-02-01 00:00:00

abstract：:The idiopathic inflammatory myopathies (IIM) are a heterogeneous group of neuromuscular disorders subdivided into polymyositis (PM), sporadic inclusion body myositis (sIBM) and dermatomyositis (DM). Chemokines play an essential role in sustained inflammation associated with IIM. We studied the distribution of the alph...

journal_title：Acta neuropathologica

authors： De Paepe B,De Keyzer K,Martin JJ,De Bleecker JL

更新日期：2005-06-01 00:00:00

abstract：:The post-mortem diagnosis of lysosomal storage diseases can be confounded by the unavailability of suitable material. Here we report the diagnosis of GM1-gangliosidosis in a cross-bred dog, from which only formalin-fixed brain was available, by a combination of electron microscopy and the detection of elevated levels ...

journal_title：Acta neuropathologica

authors： Whitfield P,Johnson AW,Dunn KA,Delauche AJ,Winchester BG,Franklin RJ

更新日期：2000-10-01 00:00:00

abstract：:Detailed neuropathologic and immunohistologic analysis of a case of serologically and polymerase chain reaction-confirmed human immunodeficiency virus type I (HTLV-I)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is reported in a 73-year-old North American black woman. In addition to the usual neuropath...

journal_title：Acta neuropathologica

authors： Wu E,Dickson DW,Jacobson S,Raine CS

更新日期：1993-01-01 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

authors： Tsutsui Y,Kashiwai A,Kawamura N,Kadota C,Nagahama M

更新日期：1991-01-01 00:00:00

abstract：:An analysis is presented of the immunohistological and ultrastructural features in a series of 118 surgically removed pituitary adenomas all of which were studied immunohistologically using antisera to growth hormone (GH), prolactin (PRL) ACTH, beta FSH, beta LH and beta TSH, and 75 of which were studied ultrastructur...

journal_title：Acta neuropathologica

authors： Esiri MM,Adams CB,Burke C,Underdown R

更新日期：1983-01-01 00:00:00

abstract：:The selective toxicity of silica dust for macrophages has been used to assess the role of these cells in experimental allergic neuritis (EAN). Inbred Lewis rats were inoculated with bovine dorsal roots in Freund's complete adjuvant (day 0). In two experiments, animals received 200 mg of silica dust in 1 cm3 of saline ...

journal_title：Acta neuropathologica

authors： Craggs RI,King RH,Thomas PK

更新日期：1984-01-01 00:00:00

abstract：:Interleukin-6 (IL-6) immunoreactivity has previously been shown in plaques in Alzheimer's disease (AD) and elevated IL-6 concentrations have been measured biochemically in brains of AD patients. In this study, we investigated the appearance of IL-6 immunoreactivity in AD plaques according to the stage of plaque format...

journal_title：Acta neuropathologica

authors： Huell M,Strauss S,Volk B,Berger M,Bauer J

更新日期：1995-01-01 00:00:00

abstract：:Data on the relationship between Parkinson's disease (PD) and stroke have been conflicting, some studies showing a reduced risk of stroke during life, and others indicating an increased risk of stroke-related death. Consecutive cases (n=617) of autopsy-proven idiopathic PD (Lewy body disease of the brain stem type) an...

journal_title：Acta neuropathologica

authors： Jellinger KA

更新日期：2003-05-01 00:00:00

abstract：:The diagnosis of infantile neuro-axonal dystrophy (INAD) in a 5-year-old patient was confirmed by the ultrastructural study of neuromuscular, skin and conjunctival biopsy specimens. Abnormal networks of smooth membranous, lamellar and tubular profiles were found in presynaptic terminals and in conjunctival and dermal ...

journal_title：Acta neuropathologica

authors： Martin JJ,Leroy JG,Libert J,van Eygen M,Logghe N

更新日期：1979-03-15 00:00:00

abstract：:Onion bulbs are concentric lamellar structures formed by Schwann or perineurial cells, which may be seen in several generalized or localized diseases of the peripheral nerve. There is debate regarding the pathogenesis of localized tumefactions displaying these microscopic structures. We report the fifth case, to our k...

journal_title：Acta neuropathologica

authors： LaPoint SF,Powers JM,Woodruff JM,MacCollin M,Jacoby LB,Vortmeyer AO,Zhuang Z,Fong CT,Ifthikharuddin SF,Teot L,Coniglio JU,Sullivan RP

更新日期：2000-01-01 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

authors： Qaddoumi I,Orisme W,Wen J,Santiago T,Gupta K,Dalton JD,Tang B,Haupfear K,Punchihewa C,Easton J,Mulder H,Boggs K,Shao Y,Rusch M,Becksfort J,Gupta P,Wang S,Lee RP,Brat D,Peter Collins V,Dahiya S,George D,Konomos

更新日期：2016-06-01 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：: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：: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：:The neurone-centred view of the past disregarded or downplayed the role of astroglia as a primary component in the pathogenesis of neurological diseases. As this concept is changing, so is also the perceived role of astrocytes in the healthy and diseased brain and spinal cord. We have started to unravel the different ...

journal_title：Acta neuropathologica

authors： Pekny M,Pekna M,Messing A,Steinhäuser C,Lee JM,Parpura V,Hol EM,Sofroniew MV,Verkhratsky A

更新日期：2016-03-01 00:00:00