Abstract：
:We report here a detailed ultrastructural study of a brain biopsy along with post-mortem brain and optic nerve specimens from a case of Krabbe disease, a relatively rare leukodystrophy caused by a mutation in the gene for galactocerebrosidase (GALC) mapped to the 14q31 region of chromosome 14. GALC is responsible for lysosomal hydrolysis of several galactolipids including galactosylceramide, a major sphingolipids of the white matter of the central nervous system, galactosylsphingosine (psychosine) and galactosyldigluceride. The main neuropathological features such as accumulation of globoid cells, loss of myelin and marked gliosis were observed in the white matter. The monocytic origin of globoid cells was confirmed by CD-68 and ferritin-positivity and periodic acid Schiff (PAS) positivity. Ultrastructural study of the globoid cells showed the accumulation of tubular crystalloids, which are highly specific for this disease. The differences with Gaucher's disease and the pathomechanism of neuropathological damage are discussed.
journal_name
Folia Neuropatholjournal_title
Folia neuropathologicaauthors
Jesionek-Kupnicka D,Majchrowska A,Krawczyk J,Wendorff J,Barcikowska M,Lukaszek S,Liberski PPsubject
Has Abstractpub_date
1997-01-01 00:00:00pages
155-62issue
3eissn
1641-4640issn
1509-572Xjournal_volume
35pub_type
杂志文章abstract：:The present study examined the effect of DS exposure on median nerve development in rats during prenatal life. Pregnant female rats were divided into three groups: a control group, a saline group and a DS group. Offspring of these animals were divided into 2 subgroups: 4 weeks old and 20 weeks old. Nerve samples were ...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：10.5114/fn.2013.34199
更新日期：2013-01-01 00:00:00
abstract：:The importance of chemokines seems to extend far beyond their well-known role as mediators of an inflammatory response. The most interesting hypothesis is that these molecules may influence the migration of progenitor cells during development. Primary sensory neurones have been shown to migrate towards RANTES in vitro...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2003-01-01 00:00:00
abstract：:Morphological alterations and the course of changes in motoneuron counts were studied by light microscopy (cresyl violet staining) in the L2/L3 region of the spinal cord of hemizygotic transgenic rats carrying the amyotrophic lateral sclerosis-associated mutant human gene for Cu,Zn superoxide dismutase (hSOD1G93A) and...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2006-01-01 00:00:00
abstract：:A neuropathological analysis of 172 cases of AIDS in adults was carried out, to determine the occurrence and nature of the opportunistic infections of the central nervous system (CNS). The material comprised 155 cases of men, and 17 women. Mean age of patients was 38 years. Collection under study originated from the p...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：1998-01-01 00:00:00
abstract：:The aim of the study was to analyse in TEM the evolution of changes in structural elements of synaptic junctions of the cerebellar cortex in rats in valproate encephalopathy induced by chronic 12-month administration of sodium valproate - VPA (once daily intragastrically, in a dose of 200 mg/kg b.w.) and after withdra...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2002-01-01 00:00:00
abstract：INTRODUCTION:To investigate the effects of crocin on proliferation and migration of endogenous neural stem cells and the Notch1 signalling pathway in rats after cerebral ischemia reperfusion. MATERIAL AND METHODS:SD rats were randomly divided into the sham operation group, model group and administration group (crocin)...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：10.5114/fn.2020.100063
更新日期：2020-01-01 00:00:00
abstract：INTRODUCTION:Subependymal nodule (SEN) and subependymal giant cell astrocytoma (SEGA) are brain lesions frequently found in tuberous sclerosis (TS). As about 10-15% of SENs enlarge and transform into SEGAs, we examined here the possible mechanism of the phenomenon. MATERIAL AND METHODS:Using Western blot we studied 1 ...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：10.5114/fn.2015.49969
更新日期：2015-01-01 00:00:00
abstract：:In the course of histopathological investigation of the temporal lobe sections, selected from 63 patients treated surgically for intractable epilepsy and finally presented with primary temporal tumors, we found 12 cases expressed both neoplastic process' and developmental disorders. The temporal mass lesions consistin...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：1999-01-01 00:00:00
abstract：:The ultrastructural pathology of cerebral cortex in human hydrocephalus is reviewed and compared with experimental hydrocephalus. Nerve cells show moderate and severe swelling. The neighboring neuropil exhibits notable enlargement of extracellular space, synaptic plasticity and degeneration, damage of myelinated axons...
journal_title：Folia neuropathologica
pub_type： 杂志文章,评审
doi：
更新日期：2010-01-01 00:00:00
abstract：:Multiple system atrophy (MSA) is a rare neurodegenerative disorder associated with parkinsonism, ataxia, and autonomic dysfunction. Its pathology is primarily subcortical comprising vacuolation, neuronal loss, gliosis, and α-synucleinimmunoreactive glial cytoplasmic inclusions (GCI). To quantify cerebellar pathology i...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：10.5114/fn.2015.54420
更新日期：2015-01-01 00:00:00
abstract：:Adenylosuccinase (ADSL) deficiency is an autosomal recessive disorder affecting mainly the nervous system. The disease causes psychomotor retardation, frequently with autistic features and epilepsy. ADSL deficiency may be diagnosed by detection of two abnormal metabolites in body fluids--succinyladenosine (S-Ado) and ...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2008-01-01 00:00:00
abstract：:Telepathology is the diagnostic work of a pathologist at a distance. It includes specific application fields which require specific system solutions. These comprise: a. frozen section service; b. expert consultations; c. remote control measurements, and d. education and training. Applications but, in addition, all asp...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2000-01-01 00:00:00
abstract：:Paralytic tremor (pt), a hereditary neurological disorder of rabbits, is a recessive, X-linked point mutation in exon 2 of the plp gene, responsible for substitution of 38 His by Glu in the PLP molecule. Pt genotype is expressed in a range of phenotypes, distinguished by the severity of neurological symptoms. Variable...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2006-01-01 00:00:00
abstract：BACKGROUND:The efficacy of tempol and its acyl derivative tempol-C8 as retinoprotective agents was compared in a rat model of NMDA-induced retinal ganglion cell (RGC) damage. MATERIAL AND METHODS:Tempol or tempol-C8 in different doses was administered intraperitoneally to 6 weeks old (pre-adolescent) and 9-10 weeks ol...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：10.5114/fn.2014.45570
更新日期：2014-01-01 00:00:00
abstract：:Chordoid glioma is a rare benign neoplasm of uncertain histogenesis occurring in the third ventricle/ /suprasellar region. Recently, data have emerged suggesting that chordoid glioma is a variant of ependymoma related to a specialised ependyma of the subcommisural organ or the lamina terminalis area. In this study, we...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2003-01-01 00:00:00
abstract：INTRODUCTION:Brain injury caused by ischaemic stroke is a major cause of disability and death throughout the world. The present study evaluates the neuroprotective effect of pseudopterosin A (PtA) against ischaemia-induced brain injury. MATERIAL AND METHODS:Ischaemia was induced by pMCAO model, and rats were separated...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：10.5114/fn.2018.76614
更新日期：2018-01-01 00:00:00
abstract：:Morphometric analysis of the cerebellar cortex capillary cross-section area performed in experimental valproate encephalopathy using transmission electron microscopy showed that prolongation of VPA application resulted in more enhanced lumen narrowing manifested in gradual reduction in the mean value of the coefficien...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2001-01-01 00:00:00
abstract：:Metallothioneins (MT) are common proteins in animal tissues. These proteins take part in the homeostasis of the ions of the metals which are necessary for the proper metabolism of the organism (zinc, copper), biosynthesis regulation and zincprotein activity (for example the activity of the zinc-dependant transcription...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2005-01-01 00:00:00
abstract：:The clinical and histological picture and immunocytochemical expression of some cytokines on infiltrates in the central nervous system of Lewis rats were studied in adult rats in the course of short-term therapy of acute experimental allergic encephalomyelitis with interferon beta 1a (IFN beta 1a). There was establish...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2001-01-01 00:00:00
abstract：:The aim of our study was the estimation of the apoptosis process using in situ-end labelling of DNA breaks method on paraffin sections in 5 human cases of Alzheimer's disease (AD), 6 of Creutzfeldt-Jakob disease (CJD) and in 25 mice infected experimentally with the Fujisaki strain of CJD, killed sequentially at one-we...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：1997-01-01 00:00:00
abstract：:Globoid cell leukodystrophy (GLD, also known as Krabbe disease), whose pathophysiology is still not completely elucidated, is an inherited, metabolic, and neurodegenerative disease, caused by the deficiency of β-galactocerebrosidase (GALC) or in very rare cases by lack of active saposin A. We describe two patients, i...
journal_title：Folia neuropathologica
pub_type： 杂志文章,评审
doi：10.5114/fn.2012.32364
更新日期：2012-01-01 00:00:00
abstract：:Increased pineal calcifications and decreased pineal melatonin biosynthesis, both age related, support the notion of a pineal bio-organic timing mechanism. The role of calcification in the pathogenesis of pineal gland dysfunction remains unknown but the available data document that calcification is an organized, regul...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2010-01-01 00:00:00
abstract：:Unmethylated CpG DNA, as a stimulatory molecule, has potent pro-inflammatory effects in the central nervous system (CNS). Dexmedetomidine (DEX) has been confirmed to exert anti-inflammatory effects in CNS. Our study was aimed to explore the effects of DEX on tumor necrosis factor-α (TNF-α) expression in unmethylated C...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：10.5114/fn.2016.64817
更新日期：2016-01-01 00:00:00
abstract：:The gerbils brains after 3- and 4-minute-long ischemia caused by bilateral common carotid artery occlusion and 14 days survival were investigated using lectin techniques. Chosen lectins, represented by synthetic plant glycoproteids, which are specifically bound to particular sugar residues (receptors) located on the c...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：1998-01-01 00:00:00
abstract：:The 'prion-like' transfer of pathogenic proteins may play a role in the pathogenesis of frontotemporal lobar degeneration (FTLD). Propagation of such proteins along anatomical pathways may give rise to specific spatial patterns of the 'signature' neuronal cytoplasmic inclusions (NCI) characteristic of these disorders....
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：10.5114/fn.2017.70482
更新日期：2017-01-01 00:00:00
abstract：:Human T lymphotropic virus type I (HTLV-I)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a chronic progressive myelopathy characterized by bilateral pyramidal tract involvement with sphincteric disturbances. The primary neuropathological feature of HAM/TSP is chronic myelitis characterized by perivas...
journal_title：Folia neuropathologica
pub_type： 杂志文章,评审
doi：
更新日期：2009-01-01 00:00:00
abstract：:PPAX6 is an important transcription factor which plays an essential role in brain morphogenesis and eye development. It is related to migration of neuroblasts to the cerebral cortex and deep telencephalic nuclei, and the specification of cellular and regional identity. Disturbances of brain development in two sib fetu...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2009-01-01 00:00:00
abstract：:Glioblastoma, the most malignant astrocytic tumour, is associated with limited survival and thus rare metastases. We analysed a particularly interesting case - a 51-year-old male diagnosed within 2 years with primary and recurrent glioblastoma, isocitrate dehydrogenase (IDH)-wild type, as well as with numerous extra-c...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：10.5114/fn.2019.83833
更新日期：2019-01-01 00:00:00
abstract：:Myositis specific autoantibodies (MSA) are the most specific markers of idiopathic inflammatory myopathies (IIM). There is no evidence of presence MSA in patients with other neuromuscular or connective tissue diseases. We compared the frequency of MSA in two groups of IIM patients, one from Poland and one from North A...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2000-01-01 00:00:00
abstract：:A contribution of B cells and autoantibodies has been demonstrated in MS leading to interest in the use of such autoantibodies as diagnostic or prognostic markers and as a basis for immunomodulatory therapy. ELISA and Western fail to detect reactivity against epitopes displayed by native antigens expressed on myelin s...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2010-01-01 00:00:00
