Abstract：
:The investigations were based on 3 cases with Leigh, 5 cases with Krabbe's, 4 cases of Alpers, 2 cases with Sandhoff, 1 case with Alexander's disease and 1 case with metachromatic leukodystrophy. In 1 case included into the study we have diagnosed nonketotic hyperglycinemia II. All the diseases under examination are recognized as genetically conditioned or are supposed to be of genetic origin. Damage of the white matter in a more delineated form in certain regions was found in Leigh disease. The changes demonstrated a variable degree of intensity from demyelination to necrosis. More extensive lesions of white matter in gyri and semivoal centrum were found in diseases with simultaneously damaged gray matter e.g. in Alpers and Sandhoff disease. The most extensive changes of diffuse demyelination were found in Krabbe's and Alexander's disease. In these diseases demyelination was accompanied with specific morphological structures e.g. globoidal cells (Krabbe's disease) and Rosenthal fibers (Alexander's disease). The peculiar type of demyelination was characteristic for nonketotic hyperglycinemia of type II. It was expressed by demyelination with vacuolization.
journal_name
Folia Neuropatholjournal_title
Folia neuropathologicaauthors
Kałuza J,Marszał E,Jamroz E,Pietruszewski Jsubject
Has Abstractpub_date
1999-01-01 00:00:00pages
217-9issue
4eissn
1641-4640issn
1509-572Xjournal_volume
37pub_type
杂志文章,评审abstract：:Bovine spongiform encephalopathy (BSE) is a zoonosis being the origin of variant Creutzfeldt-Jakob disease and an important cattle disease in its own right. This association has driven both the research into the disease and extensive epidemiological investigations of practical value. Not only has the occurrence of BSE...
journal_title：Folia neuropathologica
pub_type： 杂志文章,评审
doi：
更新日期：2004-01-01 00:00:00
abstract：:The object of our report is the presentation of the morphological picture of cerebellar cortex malformation as a sequel of disturbed neuronal migration. In the disarranged tissue, cavities with a network of meningeal tissue and embedded pathological vessels were noted. The external granule cells did not form a proper ...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2006-01-01 00:00:00
abstract：OBJECTIVES:Ischemic stroke remains one of the top ten causes of death in children. There is evidence for the role of pro-inflammatory cytokines, such as IL-6 and the -174G>C promoter polymorphism of the IL-6 gene, in the occurrence and outcome of stroke in adults. The aim of the present study was to determine a possibl...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2012-01-01 00:00:00
abstract：:Meningiomas are the most common primary extra-axial tumours of the central nervous system, however their metastatic spread beyond central nervous system is rare. Here we present the case of a 54-year-old male with anaplastic meningioma who, 1.5 years after initial diagnosis, developed a tumorous expansion in his left ...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：10.5114/fn.2019.91195
更新日期：2019-01-01 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：:Prion-like transmission of protein aggregates or amyloid in several neurodegenerative diseases, such as Parkinson's disease, Huntington's disease and Alzheimer's disease, in addition to the transmissible spongiform encephalopathies (or prion diseases), has been proposed recently. This is a controversial idea and, in t...
journal_title：Folia neuropathologica
pub_type： 杂志文章,评审
doi：
更新日期：2012-01-01 00:00:00
abstract：INTRODUCTION:During human myogenesis and synaptogenesis, the first contact between multiaxonal nerve terminals and the primary myotube occurs at an early stage of gestation, then monoaxonal nerve terminals form and postsynaptic clusters of acetylcholine-receptor are modified and redistributed to the site of muscle-nerv...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：10.5114/fn.2013.39722
更新日期：2013-01-01 00:00:00
abstract：:Neuronal and axonal degenerative changes in motor vagal neurons (DMNV) and sensory vagal neurons (nTS) in the medulla oblongata in newborns were studied. Material was taken from the autopsies of newborns, live and dead newborns, in different gestational weeks (aborted, immature, premature and mature). 46 cases were st...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2011-01-01 00:00:00
abstract：:The Warburg micro syndrome (WARBM) is a genetically heterogeneous syndrome linked to at least 4 loci. At the clinical level, WARBM is characterized by microcephaly, microphthalmia, microcornea, congenital cataracts, corpus callosum hypoplasia, severe mental retardation, and hypogonadism. In some families additional cl...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：10.5114/fn.2016.62537
更新日期：2016-01-01 00:00:00
abstract：:We demonstrate an unusually intensive accumulation of prion protein (PrP) on neuronal membranes in hamsters infected with the Echigo-1 panencephalopathic strain of Creutzfeldt-Jakob disease. This stands in strong contrast with the poor vacuolation in this model. Several distinct patterns of PrP(TSE) deposit were obser...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2004-01-01 00:00:00
abstract：:The subcellular distribution of glutamate and GABA in synapses of Mongolian gerbils hippocampus was examined using post-embedding immunogold staining method for electron microscopy. Immunolabelling was performed with 10 nm gold-antibody complexes for glutamate and GABA. The gold particle densities gave reliable inform...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：1995-01-01 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:Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary, progressive ischemic disease of small vessels of the brain characterized by migraine with aura (MA), recurrent subcortical ischemic episodes, cognitive decline and psychiatric disorders. CA...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：10.5114/fn.2020.94009
更新日期：2020-01-01 00:00:00
abstract：:The qualitative analysis of changes in major nuclei (n. paranigralis left and right, n. interfascicularis) of the mesocorticolimbic system (ventral tegmental area-VTA) was carried out on 25 cases with Parkinson's disease (PD). The cellular depletion with insignificant gliosis without the presence of macrophages was fo...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：1996-01-01 00:00:00
abstract：BACKGROUND:Cutaneous anaesthesia in necrobiosis lipoidica (NL) has been attributed to the destruction of nerve fibres by the inflammatory process. In the 1980s, one study demonstrated a decrease in S100 positive neural fibres as proof of this. Thermalgic information is mainly dependent on small fibres, either myelinate...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2008-01-01 00:00:00
abstract：:Granular cell astrocytoma (GCA) is an uncommon type of granular cell tumours (GCTs) in the central nervous system. Granular cells in these tumours are of enigmatic origin. We report a case of cerebral GCA in a 59-year-old man who suffered from diabetes and Addison-Biermer disease. The tumour was localized in the left ...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2008-01-01 00:00:00
abstract：:In the present work, we investigated the effect of chronic haloperidol administration on the number of striatal neurons in guinea pigs. For this purpose, adult male guinea pigs were given daily injections of 1, 2 or 3 mg/kg of haloperidol for 6 weeks. After treatment, the animals were anesthetized via brief inhalation...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2011-01-01 00:00:00
abstract：:Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is caused by mutations in the NOTCH3 gene. Pathophysiologically, there seems to be multimerization of the extracellular domain of the protein with a possible gain of function on vascular smooth muscular cells. However,...
journal_title：Folia neuropathologica
pub_type： 信件
doi：10.5114/fn.2015.52414
更新日期：2015-01-01 00:00:00
abstract：:Some data suggest that the central nervous system (CNS) is the main target of Staphylococcus alpha-toxin. Since this pathogen cannot penetrate the blood-brain barrier (BBB), the exact mechanism by which alpha-toxin affects the CNS remains unclear. Recent studies on the role of the innate immune system have shed light ...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2004-01-01 00:00:00
abstract：:Hyponatremia is a common disorder of water-electrolyte balance characterized by the decrease of plasma sodium ions concentration below 135 mM. Although water-electrolyte balance is regulated by a kidney, symptoms of hyponatremia are related to the disturbances of intracranial homeostasis and are attributed to brain sw...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：10.5114/fn.2013.37707
更新日期：2013-01-01 00:00:00
abstract：:The aim of our study was to present six cases with cystic changes within the white matter in infant brains and discuss the variants of this type of neuropathological lesions of the developing brain. Two of them exhibited the changes characteristic for cystic leucoencephalopathy. To the others with moderate involvement...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：1994-01-01 00:00:00
abstract：:The SMN (survival motor neuron) gene plays an important role in ontogenesis and its dysfunction leads to immatu-rity of skeletal muscles and motor neurons in the spinal cord. As a result of SMN mutations the affected cells die and clinical symptoms of spinal muscular atrophy (SMA) develop. Physiologically, SMN togethe...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：2011-01-01 00:00:00
abstract：:Early reviews identified over 20 risk factors associated with Alzheimer's disease (AD) including age, familial inheritance, exposure to aluminium, traumatic brain injury (TBI), and associated co-morbidities such as vascular disease and infection. In the light of recent evidence, this review reconsiders these risk fact...
journal_title：Folia neuropathologica
pub_type： 杂志文章,评审
doi：10.5114/fn.2019.85929
更新日期：2019-01-01 00:00:00
abstract：:Electron microscopic immunocytochemical evaluation of localization and distribution of endothelins 1, 2 and 3 in the CA1 hippocampal sector in rats submitted to 10 min global cerebral ischemia was performed. The studies were done in different postischemic periods (10 min, 3, 6, 12 and 24 h) with postembedding immuno-g...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：1995-01-01 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：: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 ...
journal_title：Folia neuropathologica
pub_type： 杂志文章
doi：
更新日期：1997-01-01 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：: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：: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：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
