Abstract:
:Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare transmissible disease caused by accumulation of pathological prion protein in the CNS. sCJD typically affects patients in their sixties. The median disease duration in sCJD (6 months) is shorter than in variant Creutzfeldt-Jakob disease (vCJD) (14 months). The clinical diagnosis in sCJD is supported by the detection of periodic sharp and slow wave complexes (PSWC) in the electroencephalogram, 14-3-3 proteins in the cerebrospinal fluid (CSF) and hyperintense basal ganglia on magnetic resonance imaging (MRI). In contrast to sCJD, hyperintensities in the posterior pulvinar (the "pulvinar sign") are seen in vCJD. Different sCJD subtypes characterised by distinct neuropathological lesion profiles, clinical features and codon 129 genotype of the prion protein gene (PRNP) are described, together with the type with a proteinase K-resistant core of the prion protein. The sensitivity of diagnostic tests varies considerably in different sCJD subtypes. Alzheimer 's disease and Lewy body dementia are the most frequent differential diagnoses in elderly patients, while chronic inflammatory CNS disorders have to be considered in younger patients.
journal_name
Folia Neuropatholjournal_title
Folia neuropathologicaauthors
Krasnianski A,Meissner B,Heinemann U,Zerr Isubject
Has Abstractpub_date
2004-01-01 00:00:00pages
24-38eissn
1641-4640issn
1509-572Xjournal_volume
42 Suppl Bpub_type
杂志文章,评审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::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
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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::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::The analysis of qualitative changes in the locus coeruleus (LC) was performed on brains from 21 cases of Parkinson's disease. Eleven cases were selected for quantitative analysis of the loss of LC noradrenergic pigmented neurons. The qualitative studies revealed uneven dissemination of the noradrenergic cells loss of ...
journal_title:Folia neuropathologica
pub_type: 杂志文章
doi:
更新日期:1997-01-01 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::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::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::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::We describe here a variety of autophagous forms encountered in the terminal stages of two models of scrapie in hamsters. We also show that the number of autophagic vacuoles increased through the incubation period in the 263K strain scrapie model. In addition, we demonstrated for the first time the presence of aggresom...
journal_title:Folia neuropathologica
pub_type: 杂志文章
doi:
更新日期:2007-01-01 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: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::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::Neuroacanthocytosis is a rare disease of nervous system with multisystem pathology. This review presents clinical syndromes and morphological changes of sporadic and familial forms of neuroacanthocytosis and is illustrated by the case of a 27-year-old man. Progressive extrapyramidal syndrome appeared at the age of 22....
journal_title:Folia neuropathologica
pub_type: 杂志文章,评审
doi:
更新日期:1996-01-01 00:00:00
abstract::Lipopolysaccharide (LPS) is a potent immunogen when administered locally and/or systemically. The peripheral immunization with LPS could contribute to the progression of neurological diseases because a strong link between neuroinflammation and dopaminergic degeneration has been found. The switch between the survival a...
journal_title:Folia neuropathologica
pub_type: 杂志文章
doi:10.5114/fn.2020.96755
更新日期:2020-01-01 00:00:00
abstract::Autopsy material of 18 patients, who died between 66-86 years of age and of 4 ones, who died between 20 and 38 years of age was investigated. The white matter of frontal lobe, corpus callosum and cerebellum was studied using histological and biochemical methods. According to the results of neuropathological studies, t...
journal_title:Folia neuropathologica
pub_type: 杂志文章
doi:
更新日期:1994-01-01 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::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::Idiopathic hypertrophic pachymeningitis (IHPM) is a rare pathological state, with still unclear aetiopathogenesis. We present a case of a 63-year-old woman with cranial variety of that disease. The manifestations of the disease included headaches, paresis of VI, IX, X nerves and cerebellar ataxia. The disease was diag...
journal_title:Folia neuropathologica
pub_type: 杂志文章
doi:
更新日期:2007-01-01 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::The size frequency distributions of beta-amyloid (A beta) and prion protein (PrPsc) deposits were studied in Alzheimer's disease (AD) and the variant form of Creutzfeldt-Jakob disease (vCJD) respectively. All size distributions were unimodal and positively skewed. A beta deposits reached a greater maximum size and the...
journal_title:Folia neuropathologica
pub_type: 杂志文章
doi:
更新日期:2007-01-01 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::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::Forty human primary brain tumors: twelve protoplasmic, six gemistocytic, four fibrillary and ten anaplastic astrocytomas, eight glioblastomas were submitted for immunohistochemical and histochemical characterization of microglia in tumor tissue and in its surroundings. The following antibodies were used: GFAP, ferriti...
journal_title:Folia neuropathologica
pub_type: 杂志文章
doi:
更新日期:1994-01-01 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::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::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::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::The endothelial vacuolar and vesicular transports in traumatic human brain oedema have been reviewed and compared with experimental brain oedema in order to establish their role in both oedema formation and oedema resolution. Normal or "non-activated" and "activated" capillaries are found. The activated capillaries sh...
journal_title:Folia neuropathologica
pub_type: 杂志文章,评审
doi:10.5114/fn.2013.35951
更新日期:2013-01-01 00:00:00