Abstract:
:We investigated whether the brainstem is affected by the pathologic process of sporadic Creutzfeldt-Jakob disease (sCJD), with particular attention to brainstem atrophy, neuronal loss, pyramidal tract degeneration, and prion protein (PrP) deposition, in 33 patients with sCJD. Brainstem atrophy, particularly in the pontine base, was relatively prominent in patients with disease of unusually prolonged duration. Neuronal loss and pyramidal tract degeneration were also identified in some but not all patients with prolonged disease. Neuronal loss was relatively prominent in the pontine nucleus and less so in the substantia nigra and inferior olivary nucleus; motor nuclei of the brainstem tegmentum were well preserved. PrP deposition was present in the brainstem in all patients, and was identified predominantly in the substantia nigra, quadrigeminal body, pontine nucleus, and inferior olivary nucleus. PrP deposition was less prominent in the red nucleus and tegmentum of the pons and medulla oblongata. PrP deposition occurred least or not at all in the pyramidal tract. The density of PrP deposition in the sCJD brainstem was not associated with disease duration or neuronal degeneration until the late stage. Our results show that atrophy, neuronal loss, and pyramidal tract degeneration occur in the sCJD brainstem, particularly in patients with an unusually prolonged disease course. These findings are not associated directly with PrP deposition and may reflect end-stage sCJD. No VV1, VV2, or MV2 cases were included in our study; however, we suggest that widespread and relatively stereotypic PrP deposition is a consistent pathologic feature of sCJD, at least in MM1 sCJD patients. Although accumulation of PrP in the brainstem appears to be an early pathologic event in sCJD, and may remain into the late disease stage, the brainstem remains relatively resistant to the pathologic process of sCJD.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Iwasaki Y,Hashizume Y,Yoshida M,Kitamoto T,Sobue Gdoi
10.1007/s00401-005-0981-0subject
Has Abstractpub_date
2005-06-01 00:00:00pages
557-66issue
6eissn
0001-6322issn
1432-0533journal_volume
109pub_type
杂志文章abstract::We report a patient who showed pathological features of both multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) at autopsy. The clinical features included severe cerebellar ataxia, autonomic failure, and rigid-akinetic parkinsonism. The clinical diagnosis was MSA. Pathological examination showed se...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010100433
更新日期:2002-01-01 00:00:00
abstract::Pediatric ependymomas are highly recurrent tumors resistant to conventional chemotherapy. Telomerase, a ribonucleoprotein critical in permitting limitless replication, has been found to be critically important for the maintenance of tumor-initiating cells (TICs). These TICs are chemoresistant, repopulate the tumor fro...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-014-1327-6
更新日期:2014-12-01 00:00:00
abstract::Striped skunks were inoculated intracerebrally with the scrapie agent (suspension of brain from a naturally infected Suffolk sheep) or intramuscularly with street rabies virus (suspension of salivary glands from naturally infected skunks). Those given the scrapie agent developed clinical signs of weakness, posterior a...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687775
更新日期:1988-01-01 00:00:00
abstract::The cholinergic basal forebrain is divided into four subregions (Ch1-4), and cholinergic neuronal loss in the nucleus basalis of Meynert (Ch4) has been correlated with cognitive impairments in both Alzheimer's disease (AD) and dementia with Lewy bodies (DLB). However, the Ch1-2 regions, which provide the major choline...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-005-0004-1
更新日期:2006-02-01 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::A 40-year-old man suffering from amyotrophic lateral sclerosis with symmetrical degeneration of the thalamus and the substantia nigra is reported. The distribution pattern of the thalamic degeneration in the present case was characteristic in that the Nucleus centralis was the severest affected of the thalamic nuclei...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691074
更新日期:1978-12-15 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::A desmoplastic primary cerebral neuroblastoma originating in the frontal lobe of a boy who died at the age of 6 years contained dense core vesicles within the cytoplasm of neoplastic cells as evidence of neuronal differentiation. Sarcomatous transformation had occurred at the time of recurrence. At autopsy, he also ha...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688540
更新日期:1980-01-01 00:00:00
abstract::Specific pathological hallmarks have been described in amyotrophic lateral sclerosis (ALS), which include motor neuronal loss, Bunina bodies (BBs) and skein like inclusions (SLIs). We investigated the relation between these three lesions in the cervical and lumbar anterior horns and the hypoglossal nuclei of 20 ALS pa...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-001-0507-3
更新日期:2002-06-01 00:00:00
abstract::Mutations in the sarcomeric protein titin, encoded by TTN, are emerging as a common cause of myopathies. The diagnosis of a TTN-related myopathy is, however, often not straightforward due to clinico-pathological overlap with other myopathies and the prevalence of TTN variants in control populations. Here, we present a...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-020-02257-0
更新日期:2021-01-15 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::Proliferation indices and mean number of silver-stained nucleolar organizer region-associated proteins (Ag-NORs) are compared in 65 brain tumors, including 34 gliomas, 8 meningiomas, 17 metastatic tumors, and 6 other tumors. Immunocytochemical investigations include labeling with the monoclonal antibody Ki-67 which id...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00308919
更新日期:1990-01-01 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::Spinal cord-notochord relationship was analyzed histologically and immunohistochemically in normal human conceptuses between the 4-8 developmental weeks and in a 8-week embryo with double spinal cord. In the early 4-week embryo, the gradual closure of the neural tube along the cranio-caudal body axis was paralleled by...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00228587
更新日期:1993-01-01 00:00:00
abstract::The brain of a 69-year-old man exhibited extensive granulomatous inflammation in the walls of arteries in the leptomeninges, associated with amyloid deposition in the media of the involved arteries. The extracranial arteries exhibited neither granulomatous inflammation nor amyloid deposition in their walls. ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00686094
更新日期:1986-01-01 00:00:00
abstract::Pleomorphic xanthoastrocytoma (PXA), a tumor most often presenting superficially over the cerebral hemisphere of young subjects, has certain morphological similarities to fibrous histiocytoma (or fibrous xanthoma) of the meninges and brain, namely the occurrence of lipid-laden neoplastic cells and, frequently, a dense...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691285
更新日期:1989-01-01 00:00:00
abstract::New pericapillary inclusion bodies were found in 17 cases of sporadic amyotrophic lateral sclerosis (ALS). The inclusion bodies consisted of paracrystalline arrays with 5-7 nm electron-dense subunits, were discernible with the light microscope and had the staining properties of protein. They were surrounded by capilla...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687970
更新日期:1986-01-01 00:00:00
abstract::Medulloblastoma (MB) represents approximately 4% of adult brain tumours, and as such is a poorly studied disease. Although many adult MB are treated using paediatric MB protocols, the reported outcomes are inferior to those observed in children. It remains unclear whether biologic differences underlie these clinical o...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-010-0780-0
更新日期:2011-02-01 00:00:00
abstract::CD44 antigen (CD44), the principle cell surface receptor for hyaluronate, is up-regulated in the human demyelinating disease multiple sclerosis on fibrous astrocytes. As astrocytes are the main target cell of canine distemper virus (CDV), the consequences of a CDV infection on the CD44 expression and distribution in b...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/pl00007417
更新日期:2000-02-01 00:00:00
abstract::Round granulated body (RGB) and eosinophilic hyaline droplets (EHDs) have been described as cytoplasmic inclusions of certain astrocytic tumors. In the previous literature, however, these inclusions have been described using various terms or regarded as nosologically the same entity. Light microscopically, RGB appeare...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00296486
更新日期:1994-01-01 00:00:00
abstract::Gerstmann-Sträussler-Scheinker disease (GSS) is a hereditary transmissible spongiform encephalopathy associated with prion protein gene mutation P102L. The age of onset is roughly restricted to around the sixth decade; however, it is unclear whether the disease-specific pathology of GSS is already evident in the pre-c...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0697-y
更新日期:2003-07-01 00:00:00
abstract::The mechanism of inflammatory demyelination in canine distemper encephalitis (CDE) is uncertain but macrophages are thought to play an important effector role in this lesion. Serum and cerebrospinal fluid (CSF), containing anti-canine distemper virus and anti-myelin antibodies from dogs with CDE were tested for their ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688176
更新日期:1989-01-01 00:00:00
abstract::Six medulloblastomas were studied by electron microscopy. Two features were found which seem to be constant and essential characteristics of medulloblastoma. First, cell junctions are abundant between tumor cells. These are mostly desmosome-like but other, closer junctions, were also seen. Second, the capillary endoth...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00685966
更新日期:1975-10-27 00:00:00
abstract::Presence and distribution of S-100 protein (S-100), neuron-specific enolase (NSE), cytokeratin polypeptides, glial fibrillary acidic protein (GFAP), vimentin, actin, lysozyme and pituitary hormones (prolactin, hGH, ACTH, beta-FSH, beta-LH, beta-TSH, alpha subunit) in folliculo-stellate cells (FSC) were studied in seve...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00689825
更新日期:1984-01-01 00:00:00
abstract::To determine the origin of the large myelinated fibers in the anterolateral funiculus (ALF) in the spinal cord of humans, myelinated fibers in the ALF of the mid-cervical spinal cord were examined quantitatively. Five groups of subjects were examined, consisting of control subjects, patients with cerebral lesions and ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051129
更新日期:1999-12-01 00:00:00
abstract::Mutations in the gene coding for Sequestosome 1 (SQSTM1) have been genetically associated with amyotrophic lateral sclerosis (ALS) and Paget disease of bone. In the present study, we analyzed the SQSTM1 coding sequence for mutations in an extended cohort of 1,808 patients with frontotemporal lobar degeneration (FTLD),...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-014-1298-7
更新日期:2014-09-01 00:00:00
abstract::Since we reported a case of acute relaxant-steroid myopathy (ARSM) in 1994, we continued histological studies and compared the findings with those in a case of corticosteroid myopathy (CM). It was revealed that (1) dystrophin, spectrin, beta dystroglycan, and sarcoglycans on the cell surface were decreased, (2) regula...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051022
更新日期:1999-05-01 00:00:00
abstract::The relationship between diabetes and the size, density and area of the ventromedial hypothalamic nucleus (VMH) was studied in the genetically diabetic Chinese hamster. Matched diabetic and non-diabetic control chinese hamsters were perfused, the hypothalamus collected, sectioned and stained for light microscopy. The ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691183
更新日期:1982-01-01 00:00:00
abstract::The character of the silver positive reticulin network was analyzed with immunofluorescence and immunoperoxidase methods in an intra vitam diagnosed case of primary brain lymphoma. The network was shown to contain connective tissue proteins rich in hexose-sugars, such as type III collagen (classical "reticulin"), basa...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690962
更新日期:1985-01-01 00:00:00
abstract::Accumulation of amyloid-β (Aβ) and neurofibrillary tangles in the brain, inflammation and synaptic and neuronal loss are some of the major neuropathological hallmarks of Alzheimer's disease (AD). While genetic mutations in amyloid precursor protein and presenilin-1 and -2 (PS1 and PS2) genes cause early-onset familial...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-012-1065-6
更新日期:2013-02-01 00:00:00