Abstract:
:Ubiquitin-immunoreactive (ub-ir) neuronal cytoplasmic inclusions are characteristically found in the extramotor cortex in patients with motor neuron disease and dementia (MND-dementia) and a subset of patients with frontotemporal dementia without motor symptoms (FTD-MND type). Recently, ub-ir neuronal intranuclear inclusions have been described in a small number of patients with familial FTD-MND type. To better define the sensitivity and specificity of this pathological change, we examined postmortem tissue from 14 patients with FTD-MND type (8 familial, 6 sporadic), 10 cases of MND-dementia (5 familial, 5 sporadic), and 19 cases of MND with no history of cognitive dysfunction (2 familial, 17 sporadic). Numerous intranuclear inclusions were found in multiple anatomic sites in 6/8 cases of familial FTD-MND. Rare intranuclear inclusions were present in the hippocampal dentate granule cells in 1 case of familial MND-dementia. No sporadic cases had intranuclear inclusions. These findings suggest that intranuclear inclusions are specific for familial FTD and may identify a subset of families with a common molecular pathogenesis. Although intranuclear inclusions are most characteristic of families in which the clinical presentation is pure FTD, they may also be found in some pedigrees with both FTD and MND; further supporting the hypothesis that FTD-MND type and MND-dementia represent a clinicopathological spectrum of disease.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Mackenzie IR,Feldman Hdoi
10.1007/s00401-003-0678-1subject
Has Abstractpub_date
2003-06-01 00:00:00pages
543-8issue
6eissn
0001-6322issn
1432-0533journal_volume
105pub_type
杂志文章abstract::The ultrastructural features of SOD1-positive aggregates were determined to clarify whether these aggregates are associated with the pathogenesis of SOD1 mutant mice. We examined the spinal cord of transgenic mice expressing a G93A mutant human SOD1 gene with fewer copies (gene copy 10). At the early presymptomatic st...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-004-0939-7
更新日期:2005-03-01 00:00:00
abstract::Experimental neuropathy, characterized by endoneurial edema and demyelination, was induced by inoculating rabbits with a combination of Freund's complete adjuvant (FCA), gangliosides, lecithin and cholesterol. A less severe demyelinating neuropathy could be induced by treatment with FCA alone but no significant change...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00695496
更新日期:1987-01-01 00:00:00
abstract::Glycerol (an atoxic alcohol) and phenol (a toxic monohydroxybenzene) are currently used as neurolytic blocking agents to relieve pain or spasticity. In the present study we compared the endoneurial response of anhydrous glycerol and 7% phenol-aqua after intraneural injection into rat sciatic nerve, using electron micr...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010000349
更新日期:2001-07-01 00:00:00
abstract::Little information is available regarding the morphological changes in the mitochondria in amyotrophic lateral sclerosis (ALS). In particular, mitochondrial changes in dorsal root ganglion cells have not yet been examined. We therefore conducted an electron microscopic examination of the mitochondria in dorsal root ga...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-007-0299-1
更新日期:2007-12-01 00:00:00
abstract::It was earlier shown that bicuculline-induced status epilepticus gives rise to profound acute changes in the rat cerebral cortex, i.e. edema and neuronal alterations. In the present study, we explored to what extent interruption of the seizure activity reverses the changes observed. To that end, status epilepticus of ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00684924
更新日期:1983-01-01 00:00:00
abstract::The pattern of recovery of myelinated axons in the posterior tibial nerve after crushing was studied in rats chronically intoxicated with 2,5-hexanedione. It was given for 2 weeks before crushing (200 mg/kg i.p. 5 times a week) or additionally for two further weeks after the nerve crush. Two animals were examined from...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00703206
更新日期:1983-01-01 00:00:00
abstract::Gliomas induced in the rat by transplacental administration of ethylnitrosourea (ENU) are intensely immunoreactive for vimentin and scarcely for glial fibrillary acidic protein (GFAP). Since tumoral transformation takes place during the late fetal and early postnatal period, the sequential expression of the two glial ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00227665
更新日期:1992-01-01 00:00:00
abstract::Amyloid-beta (Abeta) deposition in cerebral blood vessel walls is one of the key features of Alzheimer's disease (AD). Abeta(1-40) carrying the "Dutch" mutation (DAbeta(1-40)) induces rapid degeneration of cultured human brain pericytes (HBP). To study the mechanisms of this Abeta-induced toxicity, a comparative cDNA ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-002-0585-x
更新日期:2002-11-01 00:00:00
abstract::Impaired axonal transport of the fast or slow component has been reported in patients with sporadic amyotrophic lateral sclerosis (ALS), animal models for ALS, and familial ALS-linked mutant Cu/Zn superoxide dismutase (SOD1) transgenic mice. However, little is known about the impairment of axonal transport in mutant S...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-005-1021-9
更新日期:2005-07-01 00:00:00
abstract::Activation of the MAPK signaling pathway has been shown to be a unifying molecular feature in pilocytic astrocytoma (PA). Genetically, tandem duplications at chromosome 7q34 resulting in KIAA1549-BRAF fusion genes constitute the most common mechanism identified to date. To elucidate alternative mechanisms of aberrant ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-011-0817-z
更新日期:2011-06-01 00:00:00
abstract::An unusual tumor of the pituitary gland is reported. The most characteristic finding of the present tumor was the presence of numerous "colloid" and mucoid secretions. The cells were stellate in shape and there were intra- or intercellular lumina which were lined by many microvilli and occasional cilia. There were als...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687617
更新日期:1984-01-01 00:00:00
abstract::Emerging evidence has demonstrated the neuroprotection of estrogen in Alzheimer's disease (AD). The hippocampus, an important target of estrogen action, is severely affected in the Alzheimer process. The aim of present study was to detect the distribution of estrogen receptor-alpha (ER-alpha) and the relationship betw...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0720-3
更新日期:2003-09-01 00:00:00
abstract::Apoptosis of neurons and glial cells has been shown to occur in the brain of patients with the acquired immune deficiency syndrome (AIDS) and was postulated as contributing to brain atrophy and white matter damage in these patients. Since apoptotic events may be induced by the Fas-Fas ligand (FasL) system, we analyzed...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051094
更新日期:1999-10-01 00:00:00
abstract::Multiple system atrophy (MSA) is characterized by the presence of distinctive glial cytoplasmic inclusions (GCIs) within oligodendrocytes that contain the neuronal protein alpha-synuclein (aSyn) and the oligodendroglia-specific phosphoprotein TPPP/p25α. However, the role of oligodendroglial aSyn and p25α in the format...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-019-02014-y
更新日期:2019-09-01 00:00:00
abstract::The expression of glial fibrillary acidic protein (GFAP) in continuous glioma and non-glioma cell lines was evaluated by molecular hybridization and immunocytochemistry. RNA transcripts from the GFAP gene were detected in two of six cell lines by Northern blot analysis of cellular RNAs using a cloned mouse GFAP cDNA p...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688264
更新日期:1987-01-01 00:00:00
abstract::Clinical and morphological findings in an 8-year-old Jewish girl with spongy leukodystrophy are presented. Ultrastructural changes indicated that this may be a form of Canavan's disease with some atypical features. Biochemical measurements indicated that synaptosomal membranes prepared from the striatum but not from t...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691321
更新日期:1981-01-01 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::"Diffuse neurofibrillary tangles with calcification" (DNTC) is a rare form of slowly progressive dementia characterized by temporal or fronto-temporal atrophy with neuronal loss and astrocytosis, neurofibrillary tangles and Fahr-type calcification, but no senile plaques in the cerebral cortex. In patients with DNTC, w...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010100411
更新日期:2001-12-01 00:00:00
abstract::A total of 66 skin biopsies from persons with Alzheimer's disease (AD) or Down's syndrome (DS) and from persons without AD were used in this study. The age range was from 7 to 89 years. Positive immunoreactivity of skin biopsies to monoclonal antibody 4G8, which is reactive to amino acid residue 17-24 of synthetic amy...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00293394
更新日期:1994-01-01 00:00:00
abstract::A murine model of Tay-Sachs disease, the prototype of the GM2 gangliosidoses, was produced through the targeted disruption of the Hexa gene encoding the subunit of alpha-hexosaminidase A. The mice were completely devoid of beta-hexosaminidase A activity and accumulated GM2 ganglioside in the CNS in an age-dependent ma...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00309622
更新日期:1995-01-01 00:00:00
abstract::Transient forebrain ischemia produces a spatially and temporally selective pattern of neuronal degeneration in the hippocampal formation of the Mongolian gerbil. Ischemic neuronal death has been suggested to depend on the activation of excitatory hippocampal pathways that project to the vulnerable neurons. This idea w...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687758
更新日期:1989-01-01 00:00:00
abstract::Matrix metalloproteinases (MMPs) are endoproteases that have been implicated in the pathogenesis of inflammatory and vasculitic neuropathies. In systemic lupus erythematosus (SLE), a peripheral neuropathy is frequently seen that is thought to be caused by ischemic nerve damage due to vasculopathy and/or vasculitis of ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-002-0653-2
更新日期:2003-04-01 00:00:00
abstract::In spite of several cases reported in the literature, the exact pathogenetic mechanism of neuropathic changes in porphyric neuropathy remains uncertain. Various authors have ascribed the neuropathologic findings to either a dying-back axonal degeneration or segmental demyelination. In recent years, the hypothesis of a...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00697191
更新日期:1984-01-01 00:00:00
abstract::Numerous families exhibiting both frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) have been described, and although many of these have been shown to harbour a repeat expansion in C9ORF72, several C9ORF72-negative FTD-ALS families remain. We performed neuropathological and genetic analysis of a la...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-013-1078-9
更新日期:2013-04-01 00:00:00
abstract::The original version of this article unfortunately contained a typesetting error in Fig 3c. The corrected Fig. 3 is given in the following page. ...
journal_title:Acta neuropathologica
pub_type: 杂志文章,已发布勘误
doi:10.1007/s00401-019-02115-8
更新日期:2020-02-01 00:00:00
abstract::This paper examines the neuropathology of oxygen-glucose deprivation uncomplicated by stagnant conditions. Rabbit vagus nerves were pulled into a multi-compartment perfusion chamber, stimulated five times per second and deprived of energy by substituting nitrogen and deoxyglucose for oxygen and glucose in the Locke's ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00685378
更新日期:1982-01-01 00:00:00
abstract::The neurone-centred view of the past disregarded or downplayed the role of astroglia as a primary component in the pathogenesis of neurological diseases. As this concept is changing, so is also the perceived role of astrocytes in the healthy and diseased brain and spinal cord. We have started to unravel the different ...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-015-1513-1
更新日期:2016-03-01 00:00:00
abstract::Thirty-one squirrel monkey fetuses were exposed to lead acetate given to the mothers perorally during the last three-fourths or two-thirds of pregnancy. The mean maternal blood lead concentration of the group was 37 micrograms/100 ml and the individual means ranged from 22-82 micrograms/100 ml. Examination of the cent...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687421
更新日期:1988-01-01 00:00:00
abstract::Respiratory chain dysfunction leads to reactive oxygen species (ROS) generation with following oxidative stress and cellular damage. A histochemical and immunohistochemical study was performed on muscle biopsies from 17 patients with mitochondrial disease [chronic progressive external ophthalmoplegia (CPEO), mitochond...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010100455
更新日期:2002-03-01 00:00:00
abstract::A newly developed in vitro labeling method with bromodeoxyuridine (BrdU) identifies S phase cells in situ in freshly obtained surgical tissue of human brain tumors which is subsequently fixed and embedded in paraffin for BrdU immunovisualization. For the first time, the BrdU labeling index (LI) is successfully compare...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687579
更新日期:1989-01-01 00:00:00