Abstract:
:Frontotemporal dementia (FTD) is a prevalent neurodegenerative disease of heterogeneous histopathology. Neuropathological subtypes are identified on the basis of the presence or absence of tau- or ubiquitin-positive neuronal inclusions. Our recent work has established four disease stages that are independent of neuropathological subtype and reflect the clinical and degenerative progression observed in FTD. The variability in the extent of neuronal loss, astrogliosis, and microvacuolation are, therefore, more likely to reflect disease stage with potentially predictable differences between cases at early versus late disease stages. Understanding the variability in these parameters may assist in determining the importance of diverse disease subtypes in FTD. We examined 21 cases of sporadic, behavioural variant FTD and quantified the progression of histopathological change. The neuropathology of early disease was marked by severe astrogliosis of both the frontal and temporal cortices and neuronal loss, which was more evident in upper cortical layers of the frontal lobe. In late disease, neuronal loss was evident from both layer III and V in frontal and temporal cortices, and particularly the CA1 sector of the hippocampus. In addition, we compared the neuropathology of Pick's disease, dementia lacking distinctive histopathology and FTD with motor neuron disease, and found no difference in these pathological subtypes on the basis of neuronal loss, astrogliosis or microvacuolation. These results show that the earliest cellular changes in FTD occur in glia, and that disease stage rather than FTD subtype determines the pattern and extent of neuronal degeneration.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Kersaitis C,Halliday GM,Kril JJdoi
10.1007/s00401-004-0917-0subject
Has Abstractpub_date
2004-12-01 00:00:00pages
515-23issue
6eissn
0001-6322issn
1432-0533journal_volume
108pub_type
杂志文章abstract::The long-term survival of oligodendrocytes in the absence of axons in adult animals was studied following Wallerian degeneration of the optic nerves of adult rats for periods up to 22 months. In contrast to the findings in development and in young animals, large numbers of oligodendrocytes survived during this time pe...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00308922
更新日期:1990-01-01 00:00:00
abstract::The present study used cocultures of rat dorsal root ganglia (DRG) and peritoneal macrophages to define the role of activated complement components during demyelination. The complement cascade was activated in vitro by treatment of the cultures with natural rat serum and lipopolysaccharides. Complement activation was ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00318572
更新日期:1995-01-01 00:00:00
abstract::We report two sporadic cases of tauopathy with unusual neuropathological features. The ages of the patients at death were 86 and 74 years, and the disease durations were 4 and 3 years, respectively. The former patient showed progressive dementia and amyotrophy (autopsy revealed that severe cervical spondylosis was res...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-006-0093-5
更新日期:2006-09-01 00:00:00
abstract::It has been suggested that delayed development in either the peripheral or central nervous system could underlie the sudden infant death syndrome (SIDS). We studied the phrenic nerve in an attempt to find if maturation in this nerve was delayed in SIDS, and to see if fiber size differences could explain the paucity of...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050445
更新日期:1996-01-01 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::We describe features of a patient that broadens the clinical and pathological spectrum of neurofilament inclusion disease (NFID). The patient was a 52-year-old man with a 5--6 year history of progressive, asymmetrical spastic weakness of the upper and lower extremities; L-DOPA-unresponsive parkinsonism; and SPECT evid...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-004-0974-4
更新日期:2005-04-01 00:00:00
abstract::Thirty-three rectus superior extraocular muscles from 23 autopsy cases, all over 60 years, were examined. Eosinophilic inclusions (Hirano bodies) were observed in all the muscles; they were stained deep red by Masson's trichrome stain and were positive for protein stain. They consisted of a collection of filamentous s...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691883
更新日期:1983-01-01 00:00:00
abstract::The Norrbottnian type of Gaucher disease is characterized by infantile or juvenile onset and variable degrees of neurological symptoms, some of which develop only after splenectomy. A full neuropathological description of this type of Gaucher disease has not been reported previously. The brains of five patients were e...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690463
更新日期:1984-01-01 00:00:00
abstract::We report the case of an 11-year-old male who developed subacute diffuse polyradiculoneuropathy, associated with digestive symptoms and Epstein-Barr virus infection. Parental consanguinity was present. The laboratory findings including bone marrow smear were consistent with hemophagocytic lymphohistiocytosis (HLH). El...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-004-0897-0
更新日期:2004-10-01 00:00:00
abstract::In order to further evaluate the parameters whereby intracerebral administration of recombinant α-synuclein (αS) induces pathological phenotypes in mice, we conducted a series of studies where αS fibrils were injected into the brains of M83 (A53T) and M47 (E46K) αS transgenic (Tg) mice, and non-transgenic (nTg) mice. ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-014-1268-0
更新日期:2014-05-01 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::Pathways for transfer of horseradish peroxidase (HRP) across the cerebral microvasculature were studied in Mongolian gerbils after inducing either unilateral carotid-artery ligation or intracarotid air embolism. Electron microscopy on samples from both ipsilateral and contralateral brain hemispheres showed the reactio...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00717032
更新日期:1979-07-13 00:00:00
abstract::Low-grade neuroepithelial tumors (LGNTs) are diverse CNS tumors presenting in children and young adults, often with a history of epilepsy. While the genetic profiles of common LGNTs, such as the pilocytic astrocytoma and 'adult-type' diffuse gliomas, are largely established, those of uncommon LGNTs remain to be define...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-016-1539-z
更新日期:2016-06-01 00:00:00
abstract::Accumulation of the tau protein in fibrillar intracellular aggregates is a defining feature of multiple neurodegenerative diseases collectively referred to as tauopathies. Despite intensive study of tau, there is limited information on the formation and clearance dynamics of tau inclusions. Using rAAV vectors to media...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-021-02264-9
更新日期:2021-01-26 00:00:00
abstract::Histochemical and electron microscopic studies were performed in an attempt to clarify the muscle pathology in an 18-year-old man with Fabry disease, showing proximal limb muscle atrophy, and his 52-year-old mother, who is a Fabry carrier with hypertrophic cardiomyopathy. Despite the relatively mild myopathic changes ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00296520
更新日期:1995-01-01 00:00:00
abstract::The spinal cord of 20 patients with amyotrophic lateral sclerosis (ALS) and 5 patients with lower motor neuron disease (LMND) were investigated immunohistochemically using anti-human excitatory amino acid transporter 1 (EAAT1) and EAAT2 antibodies which are the astrocytic transporters. The purpose of the study was to ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004019900159
更新日期:2000-08-01 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::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 ...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-007-0241-6
更新日期:2007-07-01 00:00:00
abstract::The isolated paired helical filaments (PHF) that occur in the neurofibrillary tangles of Alzheimer's disease were assayed to determine if they contained N-acetyl-glucosamine and N-acetyl-galactosamine residues. The enzyme-linked lectin assay was used to detect their total content in the PHF preparation. The assay empl...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294242
更新日期:1990-01-01 00:00:00
abstract::Multiple different pathological protein aggregates are frequently seen in human postmortem brains and hence mixed pathology is common. Mixed dementia on the other hand is less frequent and neuropathologically should only be diagnosed if criteria for more than one full blown disease are met. We quantitatively measured ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-015-1406-3
更新日期:2015-05-01 00:00:00
abstract::Granulovacuolar degeneration (GVD) is a common feature in Alzheimer's disease (AD). The occurrence of GVD is closely associated with that of neurofibrillary tangles (NFTs) and GVD is even considered to be a pre-NFT stage in the disease process of AD. Currently, the composition of GVD bodies, the mechanisms associated ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-020-02261-4
更新日期:2021-01-25 00:00:00
abstract::We have studied microtubule-associated protein 2 (MAP2) expression in anterior horn neurons in the cervical and lumbar spinal cords of 19 cases of adult-onset sporadic amyotrophic lateral sclerosis (ALS) using immunohistochemistry. Specimens from 7 patients without neurological disease served as controls. MAP2 express...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050950
更新日期:1999-01-01 00:00:00
abstract::Flurothyl-induced status epilepticus was studied by light and electron microscopy (LM, EM) to determine the time course and structural features of neuronal necrosis in the vulnerable brain regions in epilepsy. The cerebral cortex, hippocampus and thalamus were examined after closely spaced recovery periods of up to 1 ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687789
更新日期:1988-01-01 00:00:00
abstract::Spinal and bulbar muscular atrophy (SBMA) is a neuromuscular disease caused by the expansion of a polyglutamine tract in the androgen receptor (AR). The mechanism by which expansion of polyglutamine in AR causes muscle atrophy is unknown. Here, we investigated pathological pathways underlying muscle atrophy in SBMA kn...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-016-1550-4
更新日期:2016-07-01 00:00:00
abstract::Modern neuropathology serves a key function in the multidisciplinary management of brain tumor patients. Owing to the recent advancements in molecular neurooncology, the neuropathological assessment of brain tumors is no longer restricted to provide information on a tumor's histological type and malignancy grade, but ...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-010-0736-4
更新日期:2010-11-01 00:00:00
abstract::The distribution of apolipoprotein E (ApoE) was studied in the brain tissue of cases of the amyotrophic lateral sclerosis-parkinsonism-dementia complex of Guam, locally known as lytico bodig disease (LB), and compared with cases of Alzheimer's disease (AD) and normal brain tissue. In both LB and AD, strong ApoE immuno...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050533
更新日期:1996-10-01 00:00:00
abstract::When 22 members of the BrainNet Europe (BNE) consortium assessed 31 cases with alpha-synuclein (alphaS) immunoreactive (IR) pathology applying the consensus protocol described by McKeith and colleagues in 2005, the inter-observer agreement was 80%, being lowest in the limbic category (73%). When applying the staging p...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-009-0523-2
更新日期:2009-06-01 00:00:00
abstract::We have previously reported a pathological investigation of peripheral neuropathy in a horse with knuckling. This report describes details of the muscle and peripheral nerve lesions in two additional cases of light horse yearlings with knuckling. The skeletal muscles showed neurogenic atrophy characterized by scattere...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050916
更新日期:1998-10-01 00:00:00
abstract::Dysferlin is a newly identified sarcolemmal protein related to Miyoshi myopathy and limb-girdle muscular dystrophy. Although its function is still unknown, it is inferred from the presence of C2 domains and a transmembrane domain in its sequence that dysferlin may be expressed or located not only at the sarcolemma but...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0686-1
更新日期:2003-06-01 00:00:00
abstract::Psammoma bodies at the perivascular area in five cases of meningioma were examined with the electron microscope. In general, meningocytic cells invest the outer aspect of blood vessels, which are constituted by multilayered basal laminae, collagen fibers, microfibrils, and pericytes. Remnants of degenerated cells are ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688693
更新日期:1985-01-01 00:00:00