Abstract:
:The authors describe in biopsies from 6 cases of Werdnig-Hoffmann disease, including 2 of the more benign type, the ultrastructural typical aspects of denervation. They compare their findings with those of other workers. The striking points are the great variation in the diameter of the muscle fibres and the myofibrils, the disorganisation of the myofibrils, the sarcomeres and the filaments, with persistance of the relations between thick and thin filaments at various levels, the modifications of the Z-band and the triads in chains. The folds and the basement membrane are examined. Centrioles are present in a muscle fibre and in a satellite. Glycogen is very abundant. The nerves seem normal but some Schwann cells contain pi granules which are not observed usually at the age of the patient. The end plates and a muscle spindle are normal.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Szliwowski HB,Drochmans Pdoi
10.1007/BF00687923subject
Has Abstractpub_date
1975-01-01 00:00:00pages
281-96issue
4eissn
0001-6322issn
1432-0533journal_volume
31pub_type
杂志文章abstract::The cerebrovascular lesions of severe chronic hypertension were studied by light microscopy in perfusion-fixed, subserially sectioned brains from stroke-prone spontaneously hypertensive rats (SHRSP). The leakage and spread of plasma proteins were visualized by immunohistochemical detection of extravasated fibrinogen a...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690831
更新日期:1985-01-01 00:00:00
abstract::The case of a child is described who at the age of 2 years showed the first evidence of a developing neurological disease. Within a couple of years, profound mental retardation and severe motor deficit with spastic tetraplegia became established. No seizures and no pigmentation of the retina were observed. The conditi...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688145
更新日期:1975-01-01 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
pub_type: 杂志文章
doi:10.1007/s004010000245
更新日期:2000-08-01 00:00:00
abstract::We report two sporadic cases of tauopathy with unusual neuropathological features. The ages of the patients at death were 86 and 74 years, and the disease durations were 4 and 3 years, respectively. The former patient showed progressive dementia and amyotrophy (autopsy revealed that severe cervical spondylosis was res...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-006-0093-5
更新日期:2006-09-01 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
pub_type: 杂志文章,评审
doi:10.1007/s00401-010-0691-0
更新日期:2011-01-01 00:00:00
abstract::A neuropathological study is presented of a case showing the association of tuberous sclerosis of the brain and dysplasia of the corpus callosum as well as omphalocele and malrotated colon. No signs of tuberous sclerosis were found in the internal organs. From a review of the literature this appears to be the fourth c...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF01273269
更新日期:1978-04-26 00:00:00
abstract::The evolvement of amyloid beta (Abeta) deposition in the frontal cerebral cortex of 24 patients of increasing age with Dutch-type hereditary cerebral hemorrhage with amyloidosis (HCHWA-D) was studied using end-specific monoclonal antibodies to Abetax-42 (Abeta42) or Abetax-40 (Abeta40) and markers for degenerating neu...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051143
更新日期:2000-04-01 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
pub_type: 杂志文章
doi:10.1007/s00401-016-1550-4
更新日期:2016-07-01 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
pub_type: 杂志文章
doi:10.1007/BF00228582
更新日期:1993-01-01 00:00:00
abstract::We describe three cases of early- (cases 1-3, 28-39 years) and one of late-onset (case 4, 76 years) Alzheimer's disease (AD) with 'cotton wool' plaques (CWPs) but without a family history indicating autosomal dominant inheritance. The early-onset cases, but not the late-onset case, showed remarkable aggression, disinh...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0737-7
更新日期:2003-10-01 00:00:00
abstract::Immunohistochemical analysis of inflammatory cell density and infiltrate subpopulations in 42 meningiomas was performed. Evaluation of infiltrating cell density was carried out by cell counting. Meningothelial and fibroblastic meningiomas contained an average of 3% mononuclear cells; the few lymphocytes were localized...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294381
更新日期:1989-01-01 00:00:00
abstract::A case of progressive polyneuropathy associated with Waldenström's macroglobulinaemia is reported. A monoclonal IgM-lambda gradient was detected in the serum and cerebro-spinal fluid. By electro-immunoblot analysis antibodies against myelin-associated glycoprotein were found in the serum and cerebro-spinal fluid. The ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690395
更新日期:1984-01-01 00:00:00
abstract::The alterations in oligodendrocytes in myelin basic protein-induced acute experimental autoimmune encephalomyelitis (EAE) in the Lewis rat were studied using the technique of pre-embedding immunolabelling with the Rip monoclonal antibody, which specifically labels the cytoplasm of the cell bodies and processes of olig...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050763
更新日期:1998-01-01 00:00:00
abstract::Sera from guinea pigs with acute or chronic relapsing experimental allergic encephalomyelitis (EAE) were injected into lumbosacral subarachnoid space of normal recipient rats. Seventeen of 37 sera induced demyelination in the CNS, and 27 of 37 sera caused demyelinated peripheral nerve fibers in the roots. The highest ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690994
更新日期:1981-01-01 00:00:00
abstract::A model of hypothermic circulatory arrest with recovery has been developed in the newborn dog. Eleven puppies were anesthetized with halothane, paralyzed and artificially ventilated with 70% nitrous oxide -30% oxygen to paO2 > 60 mm Hg, paCO2 = 33-42 mm Hg and pHa = 7.35-7.42. Animals were surface cooled to 20 degrees...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00227767
更新日期:1993-01-01 00:00:00
abstract::Alzheimer's disease (AD) is a major cause of dementia. Characteristic neuropathological features of AD include neurofibrillary tangles, senile plaques, amyloid angiopathy and microvascular atrophy. The ultra-structure of the microvascular atrophy in AD and its pathogenetic significance have not been defined. This repo...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050386
更新日期:1996-01-01 00:00:00
abstract::Pick's disease is characterized morphologically by severe atrophy of the frontal and temporal lobes and the presence in the cerebral cortex of degenerative neuronal lesions referred to as Pick bodies. In the present study, we analyzed the regional and laminar distribution of Pick bodies in a series of 16 Pick's diseas...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00296179
更新日期:1994-01-01 00:00:00
abstract::Progressive supranuclear palsy (PSP) is a 4R-tauopathy predominated by subcortical pathology in neurons, astrocytes, and oligodendroglia associated with various clinical phenotypes. In the present international study, we addressed the question of whether or not sequential distribution patterns can be recognized for PS...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-020-02158-2
更新日期:2020-08-01 00:00:00
abstract::The ultrastructure of the curly fibers was examined by the transmission and immunoelectron microscopy as well as by the rapid-freeze, deep-etch and replica method. The curly fibers consisted mainly of paired helical filaments (PHF) in the neuropils, both pre- and post-synaptic. On the deep-etch replicas, PHF in the ne...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00334498
更新日期:1990-01-01 00:00:00
abstract::Sensitive detection of alpha-synuclein (alpha-syn) pathology is important in the diagnosis of disorders like Parkinson's disease, dementia with Lewy bodies, and multiple system atrophy and in providing better insights into the etiology of these diseases. Several monoclonal antibodies that selectively react with aggreg...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-008-0375-1
更新日期:2008-07-01 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
pub_type: 杂志文章
doi:10.1007/BF00685251
更新日期:1984-01-01 00:00:00
abstract::Neurotrophins regulate the proliferation and differentiation of neurons in the central nervous system via a family of specialized receptors, including TrkA, TrkB, and TrkC. As little is known about their expression or potential role in human glial tissues and glial tumors, we undertook an immunohistochemical analysis ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050906
更新日期:1998-10-01 00:00:00
abstract::Mutations of isocitrate dehydrogenase 1 (IDH1) gene are most common in glioma, arguably preceding all known genetic alterations during tumor development. IDH1 mutations nearly invariably target the enzymatic active site Arg132, giving rise to the predominant IDH1R132H. Cells harboring IDH1 R132H -heterozygous mutation...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-017-1800-0
更新日期:2018-02-01 00:00:00
abstract::Gerstmann-Sträussler-Scheinker disease (GSS) is a hereditary transmissible spongiform encephalopathy associated with prion protein gene mutation P102L. The age of onset is roughly restricted to around the sixth decade; however, it is unclear whether the disease-specific pathology of GSS is already evident in the pre-c...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0697-y
更新日期:2003-07-01 00:00:00
abstract::This report concerns a clinicopathological study of three additional patients with corticobasal degeneration (CBD), described here for the first time, and a clinicopathological correlation between pyramidal signs and upper motor neuron involvement, in ten autopsy cases of CBD, including seven cases reported by us prev...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-004-0966-4
更新日期:2005-04-01 00:00:00
abstract::A unique pathological finding of astrocytes was observed in the brain of a 20-year-old man who had severe physical and mental retardation. The brain was malformed showing micropolygyria in several cortical areas. A large number of hypertrophic astrocytes with eosinophilic granular substances in their cytoplasm were fo...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00427225
更新日期:1992-01-01 00:00:00
abstract::Synemin is a member of the intermediate protein superfamily. Previous studies in avian and rodent skeletal and cardiac muscles have demonstrated that synemin localises at the Z-band, where it associates with desmin and alpha-actinin. In the present study, the distribution of synemin was examined using immunohistochemi...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0695-0
更新日期:2003-07-01 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
pub_type: 杂志文章
doi:10.1007/s004010050780
更新日期:1998-02-01 00:00:00
abstract::Pediatric ependymomas are highly recurrent tumors resistant to conventional chemotherapy. Telomerase, a ribonucleoprotein critical in permitting limitless replication, has been found to be critically important for the maintenance of tumor-initiating cells (TICs). These TICs are chemoresistant, repopulate the tumor fro...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-014-1327-6
更新日期:2014-12-01 00:00:00
abstract::The regional distribution of the postsynaptic microtubule-associated protein 2 (MAP2) and the presynaptic marker protein synaptophysin was investigated by immunohistochemistry in brains of rats submitted to 30-min forebrain ischemia by four-vessel occlusion. The following brain temperature profiles during ischemia wer...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00230493
更新日期:1993-01-01 00:00:00