Abstract:
:Frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) is an autosomal dominant neurodegenerative disorder caused by mutations in the MAPT gene which encodes the microtubule-associated protein tau. This hereditary tauopathy is a rare clinical syndrome, affecting approximately two hundred kindreds and about six hundred individuals bearing thirty nine known MAPT mutations. The disorder is thought to be related to the altered proportion of tau protein isoforms or the ability of tau to bind to microtubules and to promote microtubule assembly and organization. The clinical presentation of FTDP-17 includes behavioral, cognitive and motor abnormalities. This disorder has both a variable course and phenotype. Gross neuropathological examination reveals brain atrophy, especially of the frontal and temporal lobes, and selective atrophy of the basal ganglia and brainstem nuclei. The major microscopic features of FTDP-17 demonstrate the presence of neurofibrillary tangles, neuropil threads and glial inclusions composed of insoluble tau protein. Distribution and amount of tau deposits vary, depending on the type of MAPT mutation. The definitive diagnosis of FTDP-17 requires a set of clinical and pathological features combined with a molecular genetic analysis. Currently, there is no known effective treatment for FTDP-17.
journal_name
Folia Neuropatholjournal_title
Folia neuropathologicaauthors
Wszołek ZK,Słowiński J,Golan M,Dickson DWsubject
Has Abstractpub_date
2005-01-01 00:00:00pages
258-70issue
4eissn
1641-4640issn
1509-572Xpii
5369journal_volume
43pub_type
杂志文章,评审abstract::Cortical biopsies of 13 patients with clinical diagnosis of congenital hydrocephalus, Arnold-Chiari malformation and hydrocephalus, and postmeningitis hydrocephalus were examined by transmission electron microscopy to study the damage of endothelial cells, basement membrane, astrocytic end-feet layer, and perivascular...
journal_title:Folia neuropathologica
pub_type: 杂志文章
doi:
更新日期:2009-01-01 00:00:00
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
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更新日期:2004-01-01 00:00:00
abstract::Tumours of astroglial origin, both malignant glioblastoma (GBM) and benign subependymal giant cell astrocytoma (SEGA), pose a serious medical problem. Casein kinase 2 (CK2), a member of the serine/threonine kinase family, has antiapoptotic properties and plays a vital role in glial tumour cell survival. It contributes...
journal_title:Folia neuropathologica
pub_type: 杂志文章
doi:10.5114/fn.2019.88452
更新日期:2019-01-01 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::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
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:INTRODUCTION:Sporadic inclusion body myositis (sIBM) is one of the most common myopathies in patients above 50 years of age. Its progressive course finally leads to immobilisation, and no effective therapy exists. Its pathogenesis includes both degenerative and inflammatory processes, however, its direct causes remain ...
journal_title:Folia neuropathologica
pub_type: 杂志文章
doi:10.5114/fn.2015.56550
更新日期:2015-01-01 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: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::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::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::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::Metallothioneins take part in the homeostasis of the ions of the metals which are necessary for the proper metabolism of the organism (zinc, copper), in biosynthesis regulation of the zinc-containing proteins and also in the detoxication of metals from the tissues. They also protect the tissue from the effects of free...
journal_title:Folia neuropathologica
pub_type: 杂志文章
doi:
更新日期:2003-01-01 00:00:00
abstract::There is an increased risk of stroke in patients with HIV infection. One of the mechanisms is production of anticardiolipin (aCL) antibodies, induced by the virus. Many studies have documented a high incidence of aCL antibodies in patients with HIV infection and the increased risk of stroke, although there is no such ...
journal_title:Folia neuropathologica
pub_type: 杂志文章
doi:
更新日期:2005-01-01 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::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::The inflammatory process is a nonspecific complex, stereotype, coordinated response of tissues to injury. This process involves vascular permeability, active migration of blood cells, and passage of plasma constituents into the injurious tissue. The molecules that mediate the initial events of inflammation are the adh...
journal_title:Folia neuropathologica
pub_type: 杂志文章,评审
doi:
更新日期:1998-01-01 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::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::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::We report the case of an 84-year-old male patient afflicted by cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) showing minimal symptoms of disease. The patient was diagnosed on the basis of ultrastructural and genetic examinations. Ultrastructurally, a typical vascu...
journal_title:Folia neuropathologica
pub_type: 杂志文章
doi:10.5114/fn.2014.47846
更新日期:2014-01-01 00:00:00
abstract::Ganglioglioma is a tumor composed of neoplastic neurons and neoplastic glial cells mixed in different proportions. Astrocytes are the essential glial component. The tumor proliferates mostly in the temporal lobe cortex, scarcely in other areas of the brain. In ganglioglioma the population of ganglionic neurons is very...
journal_title:Folia neuropathologica
pub_type: 杂志文章
doi:
更新日期:1999-01-01 00:00:00
abstract::Metformin, a type 2 anti-diabetic drug and an activator of AMP-activated protein kinase (AMPK), has been shown to reduce infarct size and pathological changes affecting astroglia in animal models of ischemic stroke. In this study, we evaluated how metformin affects cell viability, apoptosis and determined the role of ...
journal_title:Folia neuropathologica
pub_type: 杂志文章
doi:10.5114/fn.2018.80866
更新日期:2018-01-01 00:00:00
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journal_title:Folia neuropathologica
pub_type: 杂志文章
doi:
更新日期:2004-01-01 00:00:00
abstract::Cerebellar degeneration belongs to indirect effects of malignancy on the nervous system. Although the involvement of immune system is accepted as a hypothesis of its pathology, the clinical observations of ineffective immunomodulatory therapy suggest complex pathomechanisms, which await elucidation. The aim of this st...
journal_title:Folia neuropathologica
pub_type: 杂志文章
doi:
更新日期:2010-01-01 00:00:00
abstract::Cerebral ischemia injury seriously endangers human health and its molecular mechanism is still not fully understood. microRNA-223 (miR-223) has been reported to be involved in many physiological functions but the specific role of miRNA-223 in ischemic neuronal injury is still unclear. An oxygen-glucose deprivation and...
journal_title:Folia neuropathologica
pub_type: 杂志文章
doi:10.5114/fn.2018.74659
更新日期:2018-01-01 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:INTRODUCTION:Transsphenoidal surgery is the treatment of choice in Cushing's disease (CD), although even late recurrences occur in some patients. Low expression of O-6-methylguanine-DNA methyltransferase (MGMT) has been linked to a high risk of relapse in pituitary tumours, but the evidence for corticotroph adenomas is...
journal_title:Folia neuropathologica
pub_type: 杂志文章
doi:10.5114/fn.2020.102438
更新日期:2020-01-01 00:00:00
abstract::The visual cortex undergoes age related changes that have been studied mainly in rats Maccaca Mulata, and human beings. Despite the fact that there is no extensive neuronal loss in aged brains, a lot of important pathological changes are found in the morphology of the neurons. The present study describes the morpholog...
journal_title:Folia neuropathologica
pub_type: 杂志文章
doi:10.5114/fn.2012.30526
更新日期:2012-01-01 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