Abstract:
:Frontotemporal lobar degeneration is the term used to describe the non-Alzheimer clinical syndromes of frontotemporal dementia, semantic dementia and progressive non-fluent aphasia, regardless of the underlying neuropathological features. Considerable progress has been made in recent years in our understanding of the aetiology of this disorder, notably the identification of mutations in tau and progranulin genes, both on chromosome 17q21. Mutations in tau appear to affect the ability of tau to bind microtubules and/or increase this protein's ability to form fibrils. In contrast, progranulin mutations cause haploinsufficiency leading to TDP-43 accumulation. These genes collectively account for 10-20% of FTLD. However, it is clear that much remains to be discovered before our knowledge of this heterogeneous condition is complete.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Pickering-Brown SMdoi
10.1007/s00401-007-0241-6subject
Has Abstractpub_date
2007-07-01 00:00:00pages
39-47issue
1eissn
0001-6322issn
1432-0533journal_volume
114pub_type
杂志文章,评审abstract::The neuropathologic findings in the brain of a National Aeronautics and Space Administration (NASA) engineer, who died subsequent to nitrogen inhalation, are reported. Findings which are unusual in other forms of anoxia included relative preservation of neurons in the Sommer sector of the hippocampus and a pattern of ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688632
更新日期:1985-01-01 00:00:00
abstract::The recently identified prolactin (PRL)-releasing peptide (PrRP) is the first hypothalamic peptide hormone found to operate as a ligand of an orphan receptor that specifically stimulates PRL production from the pituitary gland. However, its other biological functions remain unknown. Using immunohistochemistry, we exam...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0753-7
更新日期:2003-11-01 00:00:00
abstract::A series of 50 human primary intracranial tumors were cultivated in vitro in an attempt to establish cell lines with the trypsinization technique. During the in vitro adaptation period, cultures were maintained at high cell density to avoid rapid over-growth by connective tissue. Five lines were established from 5 tum...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690383
更新日期:1977-07-15 00:00:00
abstract::The number and nucleolar volume of nerve cells within the nucleus basalis of Meynert, locus caeruleus, substantia nigra and dorsal raphe were examined in five patients with Huntington's chorea. No significant changes in nerve cell number were noted in any area in any patient and, although nucleolar volume was reduced ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687718
更新日期:1989-01-01 00:00:00
abstract::The nature and distribution of vascular changes in acute Wernicke's encephalopathy (WE) were analyzed in three autopsy cases. Lesions of the lateral vestibular nucleus of the medulla oblongata (three cases) and lateral ventricular wall (one case) were examined by reconstruction of 200 serial sections, and the capillar...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00307646
更新日期:1995-01-01 00:00:00
abstract::Amyotrophic lateral sclerosis (ALS) and adult onset X-linked recessive bulbospinal muscular atrophy (SPMA), constituting the category of adult onset form of motor neuron disease, were analyzed on motor nerve roots. The results of morphometric analysis on ventral spinal roots (VSR) of all spinal segments from ALS and S...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691322
更新日期:1981-01-01 00:00:00
abstract::This study reports the histological, ultrastructural, and immunocytochemical characteristics of intracytoplasmic eosinophilic inclusion bodies occurring in various types of neurons of the human central nervous system. By light microscopy, the inclusions were brightly eosinophilic, slightly birefringent, and sharply de...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00308960
更新日期:1989-01-01 00:00:00
abstract::Histochemical and electron microscopic studies of the brains inclusive of the leptomeninges containing large blood vessels from 7 patients with mucopolysaccharidosis (MPS) I, II, IIIA and V showed marked increase in mesenchymal elements and the generalized presence of characteristic lesions around cerebral veins and a...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690379
更新日期:1977-07-15 00:00:00
abstract::Three cases of spontaneous olfactory neuroblastoma (ONB) in domestic cats were morphologically and immunocytochemically characterized. Diagnostic light microscopic features included Flexner and Homer-Wright rosettes, while ultrastructurally the cells had neuritic processes, intracellular intermediate filaments, and in...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294617
更新日期:1990-01-01 00:00:00
abstract::Histopathological, immunocytochemical, and electron microscopical investigations were carried out in a man with a protracted history of spastic paraparesis, adrenal insufficiency and hypogonadism. Pathological findings were identical with those of the few previously reported cases of adrenomyeloneuropathy (AMN) includ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690750
更新日期:1980-01-01 00:00:00
abstract::In this report, we describe the clinical, topographical and immunohistochemical characteristics of neurofilament (NF) inclusion formation induced by the intracisternal inoculation of young adult New Zealand white rabbits at 28-day intervals with 100 micrograms AlCl3 over the course of 267 days. The ability to recover ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294460
更新日期:1995-01-01 00:00:00
abstract::Using a photochemical method silver was demonstrated in the brains of 1-, 14-, and 45-day-old rats which had been exposed to silver on gestational days 18 and 19. In the brain tissue of new-born rats, silver was found in the lysosomes of neurons and astroglia. Minor changes in the anatomic distribution of silver were ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691995
更新日期:1983-01-01 00:00:00
abstract::Extracellular deposition of amyloid β-protein (Aβ) in amyloid plaques and intracellular accumulation of abnormally phosphorylated τ-protein (p-τ) in neurofibrillary tangles (NFTs) represent pathological hallmark lesions of Alzheimer's disease (AD). Both lesions develop in parallel in the human brain throughout the pre...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-019-02053-5
更新日期:2019-12-01 00:00:00
abstract::We describe three cases of early- (cases 1-3, 28-39 years) and one of late-onset (case 4, 76 years) Alzheimer's disease (AD) with 'cotton wool' plaques (CWPs) but without a family history indicating autosomal dominant inheritance. The early-onset cases, but not the late-onset case, showed remarkable aggression, disinh...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0737-7
更新日期:2003-10-01 00:00:00
abstract::Neuronal accumulation of oligomeric amyloid-beta (Alphabeta) is considered the proximal cause of neuronal demise in Alzheimer disease (AD) patients. Blood-borne macrophages might reduce Abeta stress to neurons by immigration into the brain and phagocytosis of Alphabeta. We tested migration and export across a blood-br...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-008-0481-0
更新日期:2009-02-01 00:00:00
abstract::Data on the relationship between Parkinson's disease (PD) and stroke have been conflicting, some studies showing a reduced risk of stroke during life, and others indicating an increased risk of stroke-related death. Consecutive cases (n=617) of autopsy-proven idiopathic PD (Lewy body disease of the brain stem type) an...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0676-3
更新日期:2003-05-01 00:00:00
abstract::Alterations in sphingolipid metabolism are described to contribute to various neurological disorders. We here determined the expression of enzymes involved in the sphingomyelin cycle and their products in postmortem brain tissue of multiple sclerosis (MS) patients. In parallel, we investigated the effect of the sphing...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-012-1014-4
更新日期:2012-09-01 00:00:00
abstract::The present investigation examined the morphological characteristics of epiplexus macrophages following a single intracisternal injection of the antigen, bacillus Calmette-Guerin (BCG). Three days following injection of BCG (0.5 - 4.0 X 10(8) viable microorganisms), mongrel dogs were perfused with buffered aldehydes. ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690545
更新日期:1979-08-01 00:00:00
abstract::In amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA), spinal and lower brainstem motor neurons degenerate, but some motor neuron subtypes are spared, including oculomotor neurons (OMNs). The mechanisms responsible for this selective degeneration are largely unknown, but the molecular signatures of ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-020-02133-x
更新日期:2020-05-01 00:00:00
abstract::Temozolomide (TMZ) increases the overall survival of patients with glioblastoma (GBM), but its role in the clinical management of diffuse low-grade gliomas (LGG) is still being defined. DNA hypermethylation of the O (6) -methylguanine-DNA methyltransferase (MGMT) promoter is associated with an improved response to TMZ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-015-1403-6
更新日期:2015-04-01 00:00:00
abstract::The case of a child is described who at the age of 2 years showed the first evidence of a developing neurological disease. Within a couple of years, profound mental retardation and severe motor deficit with spastic tetraplegia became established. No seizures and no pigmentation of the retina were observed. The conditi...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688145
更新日期:1975-01-01 00:00:00
abstract::Flow cytofluorometric analysis was used to determine the distribution of the DNA content in cells from selected areas of normal human brain and in benign and malignant brain tumors. Propidium iodide was employed as DNA fluorochrome and the analysis was carried out on a suspension of single cells. Normal, nonstimulated...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00684802
更新日期:1979-04-12 00:00:00
abstract::We report an experimental model of Creutzfeldt-Jakob's disease (CJD) in mice leading to the formation of giant autophagic vacuoles (AV) in neurons of the cerebral cortex. These AV appear at the end of the incubation period (4-6 months postinoculation), together with spongy changes and clinical symptoms. Autophagy, a p...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688178
更新日期:1989-01-01 00:00:00
abstract::The present study investigated the expression of c-erbB-2 in 59 meningiomas, including different histological subtypes and anaplastic variants, by immunocytochemistry and molecular biological techniques. Immunohistochemistry using the monoclonal antibody FWP-51 directed against c-erbB-2-encoded oncoprotein gp185 demon...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00228582
更新日期:1993-01-01 00:00:00
abstract::A histopathological study was carried out on the brains of eight ex-boxers (ages 56 to 83) using conventional histological staining methods and immunocytochemistry with antibodies to amyloid beta-protein and the PHF-related tau protein. All cases showed a large number of tau-immunoreactive neurofibrillary tangles and ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00308813
更新日期:1991-01-01 00:00:00
abstract::Recently, 43-kDa TAR DNA-binding protein (TDP-43) was identified as a component of ubiquitinated inclusions (UIs) in sporadic amyotrophic lateral sclerosis (SALS). To clarify whether TDP-43 immunoreactivity is present in neuronal inclusions in familial ALS (FALS), we examined immunohistochemically the brains and spina...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-007-0206-9
更新日期:2007-05-01 00:00:00
abstract::In almost all of the earlier reported cases of Kufs' disease, the adult form of ceroid lipofuscinosis, the diagnosis was ascertained by cerebral tissue examination, while peripheral biopsy examination revealed an apparent poor diffusion of specific lipofuscinic deposits, the finger print profiles (FPs). We report the ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050866
更新日期:1998-07-01 00:00:00
abstract::In comparison to the levels in age and gender-matched controls, reduced levels of pathological amyloid-β protein in cerebrospinal fluid routinely precede the onset of Alzheimer's disease-related symptoms by several years, whereas elevated soluble abnormal tau fractions (phosphorylated tau, total tau protein) in cerebr...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-013-1139-0
更新日期:2013-11-01 00:00:00
abstract::Intracranial germ cell tumors (iGCTs) are the second most common brain tumors among children under 14 in Japan. The World Health Organization classification recognizes several subtypes of iGCTs, which are conventionally subclassified into pure germinoma or non-germinomatous GCTs. Recent exhaustive genomic studies show...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-017-1673-2
更新日期:2017-03-01 00:00:00
abstract::The cerebrovascular lesions of severe chronic hypertension were studied by light microscopy in perfusion-fixed, subserially sectioned brains from stroke-prone spontaneously hypertensive rats (SHRSP). The leakage and spread of plasma proteins were visualized by immunohistochemical detection of extravasated fibrinogen a...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690831
更新日期:1985-01-01 00:00:00