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 arteries. The periadventitial space was greatly distended and filled with viscous fluid and numerous mononuclear cells containing large cytoplasmic vacuoles; these cells stained positively for glycosaminoglycans (GAG). In contrast, the neurons showed only a slight increase of GAG over the normal controls but contained an excessive amount of glycolipid-like material. The amount of GAG in the leptomeninges, inclusive of the large blood vessels, was 10.8, 6.5, 4.5 and 2.2 times greater in patients with MPS I, II, V and IIIA respectively, than the mean of unaffected controls. Dermatan sulfate (DS) accounted for most of the GAG increase in MPS I, II and V [mixed excretors of DS and heparan sulfate (HS)], and HS for the GAG increase in MPS IIIA (HS excretor). With the exception of the patient with MPS IIIA, whose GAG content and composition were the same in both the neural and mesenchymal elements, in all the other MPS types the mesenchymal elements contained more GAG, with a preponderance of DS. We conclude that the mesenchymal elements contribute substantially to the increased content of GAG in the brain and its coverings, mostly in the form of dermatan sulfate.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Dekaban AS,Constantopoulos Gdoi
10.1007/BF00690379subject
Has Abstractpub_date
1977-07-15 00:00:00pages
1-7issue
1eissn
0001-6322issn
1432-0533journal_volume
39pub_type
杂志文章abstract::Glucosylceramide lipidosis results from a defective lysosomal degradation of this glycolipid. Lipid degradation is controlled by two components, the enzyme beta-glucocerebrosidase and a sphingolipid activator protein. While most Gaucher cases are due to mutations within the gene that codes for the lysosomal enzyme, on...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050960
更新日期:1999-01-01 00:00:00
abstract::Neurofibrillary tangles were induced in the motor neurons of the rabbit spinal cord by the intrathecal injection of colchicine, vinblastine, and vincristine. The tangles stained intensely by immunofluorescence and by the peroxidase-anti-peroxidase procedure using neurofilament antisera raised against chicken brain ant...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690460
更新日期:1980-01-01 00:00:00
abstract::In 2011, genome-wide association studies implicated a polymorphism near CD33 as a genetic risk factor for Alzheimer's disease. This finding sparked interest in this member of the sialic acid-binding immunoglobulin-type lectin family which is linked to innate immunity. Subsequent studies found that CD33 is expressed in...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
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abstract::Frontotemporal dementia (FTD) is a prevalent neurodegenerative disease of heterogeneous histopathology. Neuropathological subtypes are identified on the basis of the presence or absence of tau- or ubiquitin-positive neuronal inclusions. Our recent work has established four disease stages that are independent of neurop...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-004-0917-0
更新日期:2004-12-01 00:00:00
abstract::Primary brain tumours (gliomas) initiate a strong host response and can contain large amounts of immune cells (myeloid cells) such as microglia and tumour-infiltrating macrophages. In gliomas the course of pathology is not only controlled by the genetic make-up of the tumour cells, but also depends on the interplay wi...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-014-1274-2
更新日期:2014-09-01 00:00:00
abstract::A critical appraisal is presented of nearly two decades of research publications and review articles advocating the bidirectional transcytosis of fluid-phase molecules, most notably native horseradish peroxidase (HRP), through the normal and experimentally modified blood-brain barrier (BBB). Extracellular routes circu...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/BF00294368
更新日期:1989-01-01 00:00:00
abstract::MicroRNA (miRNA) expression was assessed in human cerebral cortical gray matter (GM) and white matter (WM) in order to provide the first insights into the difference between GM and WM miRNA repertoires across a range of Alzheimer's disease (AD) pathology. RNA was isolated separately from GM and WM portions of superior...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-010-0756-0
更新日期:2011-02-01 00:00:00
abstract::Several cases of progressive multifocal leukoencephalopathy (PML) have been associated with simian virus 40 (SV40), rather than with JC virus (JCV), the polyomavirus originally isolated from PML tissue. PML has, therefore, been defined as a demyelinating syndrome with possible multiple viral etiologies. Tissues from t...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050894
更新日期:1998-09-01 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::The gastrocnemius muscles of 3 groups of 10 rats, sacrified 5, 7, and 12 days respectively, following tenotomy, were submitted to different types of fixation, fixative and embedding. The occurrence of target fibres is shown not to be an artefact due to the histological procedures. Further examination demonstrates that...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690981
更新日期:1978-05-24 00:00:00
abstract::Inappropriate apoptosis has been implicated in the mechanism of neuronal death in Huntington's disease (HD). In this study, we report the expression of apoptotic markers in HD caudate nucleus (grades 1-4) and compare this with controls without neurological disease. Terminal transferase-mediated biotinylated-UTP nick e...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-004-0957-5
更新日期:2005-03-01 00:00:00
abstract::The accumulation of misfolded α-synuclein (aSyn) and neuron loss define several neurodegenerative disorders including Parkinson's disease (PD) and dementia with Lewy bodies (DLB). However, the precise relationship between pathology and neurotoxicity and why these processes disproportionately affect certain neuron subp...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-018-1829-8
更新日期:2018-06-01 00:00:00
abstract::We report a patient who showed pathological features of both multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) at autopsy. The clinical features included severe cerebellar ataxia, autonomic failure, and rigid-akinetic parkinsonism. The clinical diagnosis was MSA. Pathological examination showed se...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010100433
更新日期:2002-01-01 00:00:00
abstract::Monosodium-L-glutamate given subcutaneously to pregnant rats caused acute necrosis of the acetylcholinesterase-positive neurons in the area postrema. The same effect has been observed in the area postrema of fetal rats. The process of neuronal cell death and the elimination of debris by microglia cells proved to be si...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00686787
更新日期:1987-01-01 00:00:00
abstract::We investigated the distribution of prion protein (PrP) in 14 German patients with sporadic Creutzfeldt-Jakob disease (CJD) and compared it with that observed in Japanese patients. Immunohistochemical study revealed diffuse gray matter stainings including synaptic structures in all cases. In addition, 4 patients showe...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294174
更新日期:1994-01-01 00:00:00
abstract::Specific pathological hallmarks have been described in amyotrophic lateral sclerosis (ALS), which include motor neuronal loss, Bunina bodies (BBs) and skein like inclusions (SLIs). We investigated the relation between these three lesions in the cervical and lumbar anterior horns and the hypoglossal nuclei of 20 ALS pa...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-001-0507-3
更新日期:2002-06-01 00:00:00
abstract::Using a photochemical method silver was demonstrated in the brains of 1-, 14-, and 45-day-old rats which had been exposed to silver on gestational days 18 and 19. In the brain tissue of new-born rats, silver was found in the lysosomes of neurons and astroglia. Minor changes in the anatomic distribution of silver were ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691995
更新日期:1983-01-01 00:00:00
abstract::Gliomatosis cerebri (GC), a rare and deadly CNS neoplasm characterized by involvement of at least three cerebral lobes, predominantly affects adults. While a few small series have reported its occurrence in children, little is known about the molecular characteristics of pediatric GC. We reviewed clinical, radiologica...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-015-1532-y
更新日期:2016-02-01 00:00:00
abstract::Using a 3H-labelled virion DNA probe applied to tissue sections, we have previously identified the precise microscopic anatomical location of herpes simplex virus (HSV) during the acute and latent stages of infection of the mouse trigeminal ganglia and central nervous system (CNS). In the present investigation, we com...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00692842
更新日期:1987-01-01 00:00:00
abstract::Peroxidase-labeled lectins were used for detection of specific monosaccharide residues in amyloid plaques in brains of scrapie-infected mice. The lectins tested recognize the following residues: beta-D-galactosyl (Ricinus communis agglutinin 120, RCA-1), alpha-D-galactosyl and alpha-D-galactopyranoside (Bandeirea simp...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688295
更新日期:1986-01-01 00:00:00
abstract::Laquinimod (LAQ) is a new oral immunomodulatory compound that reduces relapse rate, brain atrophy and disability progression in multiple sclerosis (MS). LAQ has well-documented effects on inflammation in the periphery, but little is known about its direct activity within the central nervous system (CNS). To elucidate ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-012-1009-1
更新日期:2012-09-01 00:00:00
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journal_title:Acta neuropathologica
pub_type: 杂志文章
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更新日期:2009-06-01 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::Neuropeptides have turned out to be promising new parameters, in addition to the routinely performed histochemical diagnosis, of Hirschsprung's disease (HD). Studies of the peptidergic innervation of the affected intestinal segment of patients with HD have demonstrated a marked reduction in the density of several neur...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-002-0613-x
更新日期:2003-01-01 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 spatial patterns of diffuse, primitive, classic (cored) and compact (burnt-out) subtypes of beta/A4 deposits were studied in coronal sections of the frontal lobe and hippocampus, including the adjacent gyri, in nine cases of Alzheimer's disease (AD). If the more mature deposits were derived from the diffuse deposi...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00454896
更新日期:1993-01-01 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
pub_type: 杂志文章
doi:10.1007/s00401-007-0198-5
更新日期:2007-04-01 00:00:00
abstract::The mechanism of inflammatory demyelination in canine distemper encephalitis (CDE) is uncertain but macrophages are thought to play an important effector role in this lesion. Serum and cerebrospinal fluid (CSF), containing anti-canine distemper virus and anti-myelin antibodies from dogs with CDE were tested for their ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688176
更新日期:1989-01-01 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::A mixed lymphoblastic T cell lymphoma and gonadotroph cell pituitary adenoma occurred 25 years after first resection of the adenoma. Within 1 year the lymphoma overgrew the adenoma, but was still restricted to the sellar region. Histologically, lymphoma and adenoma components were tightly admixed. Possible pathogeneti...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050990
更新日期:1999-03-01 00:00:00