Abstract:
:Histochemical investigations were carried out on skeletal muscle biopsies from ten patients with chronic progressive external ophthalmoplegia with ragged-red fibers (RRF). In addition to the RRF, mild myopathic change consisting of variation in size of both type 1 and 2 fibers was seen in all patients, as well as neuropathic change in eight. Scattered fibers with absent cytochrome c oxidase (CCO) activity (focal deficiency) were seen in all patients. In serial sections, CCO deficiency did not always occupy the entire length of a fiber but was localized segmentally to regions measuring several hundred micrometers in length, suggesting the heterogeneity of CCO activity even in the same fiber.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Yamamoto M,Nonaka Idoi
10.1007/BF00689593subject
Has Abstractpub_date
1988-01-01 00:00:00pages
558-63issue
6eissn
0001-6322issn
1432-0533journal_volume
76pub_type
杂志文章abstract::In search of novel genes associated with glioma pathogenesis, we have previously shown frequent deletions of the KIAA1797/FOCAD gene in malignant gliomas, and a tumor suppressor function of the encoded focadhesin impacting proliferation and migration of glioma cells in vitro and in vivo. Here, we examined an associati...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-019-02067-z
更新日期:2020-01-01 00:00:00
abstract::Intracerebral C6 glioma xenografts spontaneously develop centrally located necrotic regions that are bordered by densely packed neoplastic cells. Proliferative and metabolic heterogeneity in these perinecrotic regions were assessed by bromodeoxyuridine (BrdU) incorporation, by immunocytological and by histochemical an...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0803-1
更新日期:2004-03-01 00:00:00
abstract::A 65-year-old woman was admitted to our hospital for forgetfulness, depression and eccentric behavior that had been first noticed 2 years prior to admission. She showed memory impairment, perseveration and repeated violent actions, but no limb-kinetic apraxia. She died 12 years after the onset of symptoms. At autopsy,...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010100460
更新日期:2002-03-01 00:00:00
abstract::We have previously characterized three human leucine-rich repeats and immunoglobulin-like domains (LRIG) genes and proteins, named LRIG1-3 and proposed that they may act as suppressors of tumor growth. The LRIG1 transmembrane protein antagonizes the activity of epidermal growth factor receptor family receptor tyrosine...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-006-0032-5
更新日期:2006-03-01 00:00:00
abstract::A 50-year-old carpenter died in Western Pennsylvania of rabies on January 4, 1979. He had been hospitalized in an intensive care unit for 28 days. The diagnosis was made postmortem from light and electron microscopic examination of central nervous system tissue. Immunofluorescence studies confirmed the diagnosis later...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687393
更新日期:1980-01-01 00:00:00
abstract::Cryostat sections of 12 gliomas and of 3 peritumoral brain tissue samples were investigated for mononuclear cell infiltration by immunohistochemistry, concentrating on cells expressing monocyte/macrophage markers. Only low numbers of T cells were detected in the tumors, whereas in average 20%-30% of all cells present ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294647
更新日期:1990-01-01 00:00:00
abstract::Frontotemporal lobar degeneration with TAR DNA-binding protein 43 inclusions (FTLD-TDP) is the most common pathology associated with frontotemporal dementia (FTD). Repeat expansions in chromosome 9 open reading frame 72 (C9ORF72) and mutations in progranulin (GRN) are the major known genetic causes of FTLD-TDP; howeve...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-015-1436-x
更新日期:2015-07-01 00:00:00
abstract::In looking for a possible influence of nuclear inclusions (NIs) on neurodegeneration in human brains, we quantified morphological features of pontine neurons of three unrelated cases of neuronal intranuclear inclusion disease (NIID) and five control cases. Cross-sectional area of each neuronal nucleus and the indices ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-002-0614-9
更新日期:2003-02-01 00:00:00
abstract::Interstitial cells are isolated neurons located in the infracortical white matter that are known to express neuropeptides. Twenty-four cases selected for the absence, slight (Braak stages I-II), moderate (Braak stages III-IV), or serious degree (Braak stages V-VI) of cortical neurofibrillary pathology were studied for...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-002-0551-7
更新日期:2002-10-01 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::Focal cortical dysplasia (FCD) is a localized malformation of cortical development and is the commonest cause of severe childhood epilepsy in surgical practice. Children with FCD are severely disabled by their epilepsy, presenting with frequent seizures early in life. The commonest form of FCD in children is character...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-013-1135-4
更新日期:2013-08-01 00:00:00
abstract::This report concerns a clinicopathological study of three additional patients with corticobasal degeneration (CBD), described here for the first time, and a clinicopathological correlation between pyramidal signs and upper motor neuron involvement, in ten autopsy cases of CBD, including seven cases reported by us prev...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-004-0966-4
更新日期:2005-04-01 00:00:00
abstract::Missense mutations of the V600E type constitute the vast majority of tumor-associated somatic alterations in the v-RAF murine sarcoma viral oncogene homolog B1 (BRAF) gene. Initially described in melanoma, colon and papillary thyroid carcinoma, these alterations have also been observed in primary nervous system tumors...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-011-0802-6
更新日期:2011-03-01 00:00:00
abstract::A 79-year-old woman had a 10-year history of dementia, initially presenting as non-fluent aphasia. Magnetic resonance imaging showed frontal atrophy (left greater than right) and hyperintense foci within white matter. Neuropathologically, there was severe frontal atrophy due to cortical neuronal loss with spongy chang...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0719-9
更新日期:2003-08-01 00:00:00
abstract::Immunohistochemical studies of monoamine neurons were performed to evaluate toxic effects of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) on young adult mice and compare them with those of their offspring. Mice, 9-11 weeks old (C57BL/6J), injected subcutaneously with a large dose of MPTP (17 mg/kg per day) duri...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294662
更新日期:1989-01-01 00:00:00
abstract::Intracranial solitary fibrous tumors (SFTs) are typically dural-based, CD34-positive neoplasms of uncertain histogenesis. We examined ten cases of meninges obtained at autopsy from patients with no history of neurological illness, head trauma, or neurosurgical intervention, and ten cases of typical meningiomas with at...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010100389
更新日期:2001-10-01 00:00:00
abstract::Neuronal intracytoplasmic inclusions were found in the ventrolateral and posterior nuclei of the thalamus of 3-month-old thiamine-deficient mice and quaking mice. An electron-microscopic study revealed that these inclusions were composed of condensation of parallel rectilinear 10-nm filaments with interconnecting cros...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691499
更新日期:1983-01-01 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::Oral administration of N-[4-(3-ethoxy-2-hydropropoxy)phenyl] acrylamide (EHA) induced selective granule cell destruction in the granular layer of the cerebellar cortex together with neurological signs, such as delayed righting reflex, gait or truncal ataxia, and convulsion. Neuropathologically, it caused multifocal gr...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051133
更新日期:2000-04-01 00:00:00
abstract::A human glioma cell line, SA146, was initiated on precoated extracellular matrix from a stereotactic biopsy of a glioblastoma. We report modulation in the expression of glial fibrillary acidic protein (GFAP) by SA146 passed in vitro before or after xenogenic transplantation into nude mice. Immunofluorescence data show...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050722
更新日期:1997-10-01 00:00:00
abstract::A description is given of three cases of ganglioneuromas, which originated from ganglia of the sympathetic chain of hamsters. Other reports of such tumors in this species are reviewed and the literature relative to hamster ganglioneuromas presented. In addition, the possible genesis of origin from adult ganglion cells...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00696794
更新日期:1975-10-01 00:00:00
abstract::Muscle fiber abnormalities are described in three cases of neonatal maple syrup disease. There were important variations in fiber diameters. Lesions consisted in focal or diffuse destruction of myofibrils. In view of recent biochemical and clinical data, a direct relation between elevated branched-chain amino acid lev...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00685251
更新日期:1984-01-01 00:00:00
abstract::The expression of members of the Jun family of transcription factors was examined by immunohistochemistry, Western blotting, in situ hybridization and Northern blotting in the developing and adult rat brain following colchicine administration. Apoptotic cells, as revealed by their typical morphology and positive stain...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051059
更新日期:1999-08-01 00:00:00
abstract::Relatively little work has been done on the structural effects of organic lead in the central nervous system (CNS), although this form of lead may be a significant fraction of total brain lead. We tested a number of easily measured light-histological parameters of neuronal development in rats for sensitivity to (a) no...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687338
更新日期:1984-01-01 00:00:00
abstract::Using immunohistochemical methods optimized to detect herpes simplex virus type 2 (HSV-2) antigen, paraffin sections from human central nervous system tissues from 31 cases pathologically diagnosed as multiple sclerosis (MS), 34 cases of other neurological diseases, 4 adult cases of HSV encephalitis, and mouse brains ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00686969
更新日期:1988-01-01 00:00:00
abstract::A 77-year-old man suffered intermittent hemiconvulsions of unknown etiology on the left side for a period of about 5 weeks. At the autopsy, there was marked neuronal loss, severe proliferation of astrocytes and spongiform changes in the right cerebral cortex. The cerebral white matter showed loosening with astroglial ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687782
更新日期:1988-01-01 00:00:00
abstract::The close correlation between abnormally low pre-mortem cerebrospinal fluid (CSF) concentrations of amyloid-β1-42 (Aβ(1-42)) and plaque burden measured by amyloid imaging as well as between pathologically increased levels of CSF tau and the extent of neurodegeneration measured by MRI has led to growing interest in usi...
journal_title:Acta neuropathologica
pub_type: 杂志文章,多中心研究
doi:10.1007/s00401-011-0808-0
更新日期:2011-05-01 00:00:00
abstract::A 17 year-old male, who was mentally and bodily retarded from the age of 7 years. At the age of 16 years, several neurological signs appeared. Pathologically, there was widespread myelin loss and tissue sponginess in the white matter of the cerebrum and cerebellum, bilaterally and symmetrically. A great many Alzheimer...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:
更新日期:1976-01-01 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::Solanezumab and Crenezumab are two humanized antibodies targeting Amyloid-β (Aβ) which are currently tested in multiple clinical trials for the prevention of Alzheimer's disease. However, there is a scientific discussion ongoing about the target engagement of these antibodies. Here, we report the immunohistochemical s...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-015-1489-x
更新日期:2015-11-01 00:00:00