Abstract:
:In an autopsy case of the sporadic juvenile ALS (a 17-year-old girl) intracytoplasmic inclusions are found in the upper and lower motor neurons and in nerve cells of the dentate nucleus, pontine nucleus, brain stem reticular formation, substantia nigra, thalamus, globus pallidus and others. Histochemically they contain RNA-Protein compounds. Electron microscopically, they consist of randomly interwoven tubules with granular endoplasmic reticulums and free ribosomes in the margin. Each tubule measures 90--150 A in diameter and shows no distinct periodic constiction. Amorphous substances as well as ribosome granules are scattered and associated with those tubules. The inclusion-bearing cells are usually swollen and chromatolytic and have a large hydropic nucleus, suggesting a close relation between the development of the inclusion and chromatolysis. Clinically, a rapid progress of the symptoms (total duration: about 12 months) and conspicuous disturbances of the autonomic nerve, such as sinus tachycardia and bladder sphincter dysfunction, should be noticed.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Oda M,Akagawa N,Tabuchi Y,Tanabe Hdoi
10.1007/BF00691069subject
Has Abstractpub_date
1978-12-15 00:00:00pages
211-6issue
3eissn
0001-6322issn
1432-0533journal_volume
44pub_type
杂志文章abstract::Amyloid-beta (Abeta) peptide pathology in Alzheimer's disease (AD) comprises extracellular plaques and cerebral amyloid angiopathy (CAA). In Parkinson's disease (PD), alpha-synuclein forms intraneuronal Lewy bodies (LBs), and cortical LBs are thought to play a major role in cognitive decline designated as PD with deme...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-007-0336-0
更新日期:2008-04-01 00:00:00
abstract::Knockout of genes encoding metabotropic glutamate receptor 5 (mGluR5) or its endogenous regulators, such as Norbin, induce a schizophrenia-like phenotype in rodents, suggesting dysregulation of mGluR5 in schizophrenia. Human genetic and pharmacological animal studies support this hypothesis, but no studies have explor...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-015-1411-6
更新日期:2015-07-01 00:00:00
abstract::Equine grass sickness (EGS) is an acquired disease of unknown aetiology affecting horses kept at grass. The disease is characterised by postganglionic sympathetic and parasympathetic neuronal pathology and is categorised as a dysautonomia. This study undertook a systematic examination of brain stem cranial nerve nucle...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010000289
更新日期:2001-08-01 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
pub_type: 杂志文章
doi:10.1007/BF00688178
更新日期:1989-01-01 00:00:00
abstract::Two cases out of a sample of 41 fetuses and infants with prosencephalic malformation, observed at the Institute of Pathology and Department of Neuropathology of the University of Heidelberg, are described here in detail. These cases presented grossly with microcephaly and missing forebrain, appearing to be cases of ap...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/pl00007438
更新日期:2000-03-01 00:00:00
abstract::A 40-year-old man suffering from amyotrophic lateral sclerosis with symmetrical degeneration of the thalamus and the substantia nigra is reported. The distribution pattern of the thalamic degeneration in the present case was characteristic in that the Nucleus centralis was the severest affected of the thalamic nuclei...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691074
更新日期:1978-12-15 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
pub_type: 杂志文章
doi:10.1007/s004010051004
更新日期:1999-04-01 00:00:00
abstract::A 17 year-old male, who was mentally and bodily retarded from the age of 7 years. At the age of 16 years, several neurological signs appeared. Pathologically, there was widespread myelin loss and tissue sponginess in the white matter of the cerebrum and cerebellum, bilaterally and symmetrically. A great many Alzheimer...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:
更新日期:1976-01-01 00:00:00
abstract::In a study designed to identify the neuropathological features typical of chronic inflammatory demyelinating polyneuropathy (CIDP), we reviewed the sural nerve biopsy findings in 105 patients with this disorder. The patients' mean age at biopsy was 49 years. In 65% of patients the disease had a progressive and in 35% ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050941
更新日期:1998-12-01 00:00:00
abstract::Interphase cytogenetics, i.e., in situ hybridization using probes to chromosome-specific DNA, enables histological identification of cells bearing numerical chromosome aberrations and cytogenetic analysis of composite tumors. We studied routinely processed tissues from seven glioblastomas and three gliosarcomas using ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00389493
更新日期:1994-01-01 00:00:00
abstract::Presence and distribution of S-100 protein (S-100), neuron-specific enolase (NSE), cytokeratin polypeptides, glial fibrillary acidic protein (GFAP), vimentin, actin, lysozyme and pituitary hormones (prolactin, hGH, ACTH, beta-FSH, beta-LH, beta-TSH, alpha subunit) in folliculo-stellate cells (FSC) were studied in seve...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00689825
更新日期:1984-01-01 00:00:00
abstract::The mechanism of antibody-mediated central nervous system (CNS) demyelination in vivo was studied using rabbit eyes. Injection of anti-galactocerebroside (Gal C) antiserum alone into the normal rabbit vitreous body induced demyelination in the epiretinal myelinated fibers. This activity of the antiserum disappeared af...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687890
更新日期:1989-01-01 00:00:00
abstract::Medulloblastoma (MB) represents approximately 4% of adult brain tumours, and as such is a poorly studied disease. Although many adult MB are treated using paediatric MB protocols, the reported outcomes are inferior to those observed in children. It remains unclear whether biologic differences underlie these clinical o...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-010-0780-0
更新日期:2011-02-01 00:00:00
abstract::Experimental neuropathy, characterized by endoneurial edema and demyelination, was induced by inoculating rabbits with a combination of Freund's complete adjuvant (FCA), gangliosides, lecithin and cholesterol. A less severe demyelinating neuropathy could be induced by treatment with FCA alone but no significant change...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00695496
更新日期:1987-01-01 00:00:00
abstract::The effects of ageing, cachexia and neoplasms on striated muscle were examined in histological sections of an autopsy material. Paraffin sections were examined from four separate muscles of 30 previously healthy subjects who died suddenly, from eight cases with cachexia and from 16 cases with cachexia and neoplasms. I...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00685374
更新日期:1982-01-01 00:00:00
abstract::Numerous families exhibiting both frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) have been described, and although many of these have been shown to harbour a repeat expansion in C9ORF72, several C9ORF72-negative FTD-ALS families remain. We performed neuropathological and genetic analysis of a la...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-013-1078-9
更新日期:2013-04-01 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
pub_type: 杂志文章
doi:10.1007/BF00686202
更新日期:1988-01-01 00:00:00
abstract::Myotubular myopathy and centronuclear myopathies (CNM) are congenital myopathies characterized by generalized muscle weakness and mislocalization of muscle fiber nuclei. Genetically distinct forms exist, and mutations in BIN1 were recently identified in autosomal recessive cases (ARCNM). Amphiphysins have been implica...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-010-0754-2
更新日期:2011-02-01 00:00:00
abstract::To assess whether amyloid plaque accumulation in the monkey brain can account for age-related cognitive impairment that begins at about 20 years of age, we measured plaque content in the brains of 14 rhesus monkeys aged 5-30 years. We used immunohistochemistry employing the monoclonal antibody 6E10, which is specific ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050735
更新日期:1997-11-01 00:00:00
abstract::A total of 66 skin biopsies from persons with Alzheimer's disease (AD) or Down's syndrome (DS) and from persons without AD were used in this study. The age range was from 7 to 89 years. Positive immunoreactivity of skin biopsies to monoclonal antibody 4G8, which is reactive to amino acid residue 17-24 of synthetic amy...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00293394
更新日期:1994-01-01 00:00:00
abstract::Considering the possibility of a paracellular pathway for edema resolution, we studied the intracerebral movement of proteins and ionic lanthanum in rats with experimental hydrocephalus. Hydrocephalus was induced by injection of kaolin suspension into the cisterna magna. After induction of hydrocephalus, horseradish p...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687004
更新日期:1985-01-01 00:00:00
abstract::Mislocalization and abnormal deposition of TDP-43 into the cytoplasm (TDP-43 proteinopathy) is a hallmark in neurons of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). However, the pathogenic mechanism of the diseases linked to TDP-43 is largely unknown. We hypothesized that the failu...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-020-02205-y
更新日期:2020-11-01 00:00:00
abstract::Oxidative stress has been implicated in the pathogenesis of a number of diseases including Alzheimer's disease (AD). The oxidative stress hypothesis of AD pathogenesis, in part, is based on beta-amyloid peptide (Abeta)-induced oxidative stress in both in vitro and in vivo studies. Oxidative modification of the protein...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-009-0517-0
更新日期:2009-07-01 00:00:00
abstract::The nature and distribution of vascular changes in acute Wernicke's encephalopathy (WE) were analyzed in three autopsy cases. Lesions of the lateral vestibular nucleus of the medulla oblongata (three cases) and lateral ventricular wall (one case) were examined by reconstruction of 200 serial sections, and the capillar...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00307646
更新日期:1995-01-01 00:00:00
abstract::We studied the densities of utrophin and dystrophin at the motor end-plates of patients with myasthenia gravis (MG) using immunohistochemical analysis. The densities were compared with those found in patients with amyotrophic lateral sclerosis, Lambert-Eaton myasthenic syndrome and normal controls. Utrophin was reduce...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050483
更新日期:1996-07-01 00:00:00
abstract::The spinal cord of 20 patients with amyotrophic lateral sclerosis (ALS) and 5 patients with lower motor neuron disease (LMND) were investigated immunohistochemically using anti-human excitatory amino acid transporter 1 (EAAT1) and EAAT2 antibodies which are the astrocytic transporters. The purpose of the study was to ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004019900159
更新日期:2000-08-01 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
pub_type: 杂志文章
doi:10.1007/BF00702677
更新日期:1979-03-15 00:00:00
abstract::The binding patterns of lectins to normal peripheral nerves were examined. Twelve biotinylated lectins were used in this study; Canavalia ensiformis (Con A), Pisum sativum (PSA), Lens culinaris (LCA), Ricinus communis 1 (RCA-1), Arachis hypogaea (PNA), Glycine max (SBA), Sophora japonica (SJA), Bandeiraea simplicifoli...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294292
更新日期:1993-01-01 00:00:00
abstract::Cerebral capillaries in "overgrown" neural tissue of chick embryo brains ranging in age from 5-18 days of incubation were studied by means of electron microscopy. The vessels in the abnormal tissue showed more extensive and prolonged interdigitations and overlapping of adjacent endothelial cells than did those in norm...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00684800
更新日期:1979-04-12 00:00:00
abstract::A murine model of Tay-Sachs disease, the prototype of the GM2 gangliosidoses, was produced through the targeted disruption of the Hexa gene encoding the subunit of alpha-hexosaminidase A. The mice were completely devoid of beta-hexosaminidase A activity and accumulated GM2 ganglioside in the CNS in an age-dependent ma...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00309622
更新日期:1995-01-01 00:00:00