Abstract:
:A quantitative study was performed on spinal cord lesions in seven patients with X-linked recessive spinal and bulbar muscular atrophy. The myelinated fiber density of the lateral corticospinal tracts at the T7 cord level was well preserved for both large and small myelinated fibers. On the other hand, neurons in the L4 ventral horn were markedly depleted; marked loss was noted of the large alpha and medium-sized gamma motor neurons located in the lateral and medial nuclei as well as the small neurons in the intermediate zones of the ventral horn. These results suggest that myelinated fiber density and fiber-size distribution in the corticospinal tract are well preserved and that neuronal loss in the ventral horns is not restricted to alpha and gamma motoneurons but also involves small interneurons.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Terao S,Sobue G,Li M,Hashizume Y,Tanaka F,Mitsuma Tdoi
10.1007/s004010050575subject
Has Abstractpub_date
1997-01-01 00:00:00pages
1-6issue
1eissn
0001-6322issn
1432-0533journal_volume
93pub_type
杂志文章abstract::Chronic traumatic encephalopathy (CTE) is a neurodegenerative disease associated with repetitive mild impact traumatic brain injury from contact sports. Recently, a consensus panel defined the pathognomonic lesion for CTE as accumulations of abnormally hyperphosphorylated tau (p-tau) in neurons (neurofibrillary tangle...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-016-1649-7
更新日期:2017-03-01 00:00:00
abstract::Pelizaeus-Merzbacher disease (PMD) is an untreatable and fatal leukodystrophy. In a model of PMD with perturbed blood-brain barrier integrity, cholesterol supplementation promotes myelin membrane growth. Here, we show that in contrast to the mouse model, dietary cholesterol in two PMD patients did not lead to a major ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-019-01985-2
更新日期:2019-07-01 00:00:00
abstract::Synemin is a member of the intermediate protein superfamily. Previous studies in avian and rodent skeletal and cardiac muscles have demonstrated that synemin localises at the Z-band, where it associates with desmin and alpha-actinin. In the present study, the distribution of synemin was examined using immunohistochemi...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0695-0
更新日期:2003-07-01 00:00:00
abstract::The brain tissue obtained from ninety-five cognitively unimpaired subjects, with ages ranging from 22 to 50 years upon death, were immunohistochemically assessed for neurodegenerative changes, i.e., hyperphosphorylated tau (HPτ) and β-amyloid (Aβ) pathology in predilection neuroanatomical areas. HPτ pathology was obse...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-011-0906-z
更新日期:2012-01-01 00:00:00
abstract::Charcot-Marie-Tooth disease type 1 B (CMT1B) is a demyelinating neuropathy caused by mutations in the myelin protein zero (P0) gene (MPZ). A few cases of CMT1B were recently found to be characterized by focally folded myelin sheaths in nerve biopsy specimens; the significance of this association is unknown. Here, we d...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004019900175
更新日期:2000-09-01 00:00:00
abstract::A quantitative technique involving serial sectioning and semiautomatic morphometric analysis was used to assess the severity of the reduction in size of the major brain structures in cerebral hemispheres of children congenitally infected with HIV-1. Cerebral hemispheres from 12 children (18-48 months of age) who died ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050594
更新日期:1997-02-01 00:00:00
abstract::A 64-year-old woman developed septicemia and a generalized peripheral neuropathy while being ventilated postoperatively. No cause for the neuropathy could be found during life. At autopsy she was found to have infective endocarditis and multifocal inflammatory lesions in the central and peripheral nervous systems, con...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687409
更新日期:1989-01-01 00:00:00
abstract::A complex neuropathological study of two cases of Niemann-Pick disease (NPD) type C (NPDC) revealed some novel features in the chemical pathology of the neuronal storage. Lipid histochemistry showed the presence of a lipid which met the criteria of a neuronal glycosphingolipid. Sphingomyelin (SM) was not detected in t...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690966
更新日期:1985-01-01 00:00:00
abstract::Progressive supranuclear palsy (PSP) is a 4R-tauopathy predominated by subcortical pathology in neurons, astrocytes, and oligodendroglia associated with various clinical phenotypes. In the present international study, we addressed the question of whether or not sequential distribution patterns can be recognized for PS...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-020-02158-2
更新日期:2020-08-01 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::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::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::Psammoma bodies in meningocytic whorls were investigated by electron microscopy. In some whorls, connective tissue fibers were seen and membrane-bound vesicles were contiguous to degenerated cells. Some small vesicles, 0.1 to 0.5 micron in diameter, were outlined by plasma membrane (matrix vesicles), other larger ones...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00686081
更新日期:1986-01-01 00:00:00
abstract::Canine distemper virus (CDV) causes severe immunosuppression and neurological disease in dogs, associated with demyelination, and is a model for multiple sclerosis in man. In the early stage of the infection, demyelination is associated with viral replication in the white matter. In acute demyelinating lesions there i...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-004-0958-4
更新日期:2005-01-01 00:00:00
abstract::To determine the role of advanced glycation endproducts (AGE) in the pathogenesis of familial amyotrophic lateral sclerosis (ALS) with superoxide dismutase-1 (SOD1) mutation, we investigated the immunohistochemical localization of N(epsilon)-carboxymethyl-lysine (CML), one of the major AGE structures, in spinal cords ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050980
更新日期:1999-03-01 00:00:00
abstract::Collections of human postmortem brains gathered in brain banks have underpinned many significant developments in the understanding of central nervous system (CNS) disorders and continue to support current research. Unfortunately, the worldwide decline in postmortem examinations has had an adverse effect on research ti...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-008-0360-8
更新日期:2008-05-01 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::We have developed a functional vegetative model by an 18-min clamping of the ascending aorta combined with a bypass formation between the aorta to right atrium and the aorta to femoral vein. Complete global brain ischemia (CGBI) induced for 18 min with this model provided the following distinct advantages: cardiopulmo...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294614
更新日期:1990-01-01 00:00:00
abstract::VM mice were inoculated by intracerebral and intraperitoneal routes with brain homogenates containing the 87V strain of scrapie. The distribution and numbers of plaques were found for the parietal cortex, cingulate cortex, corpus callosum and hippocampus/dentate in coronal sections cut at the level of the thalamus and...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688311
更新日期:1986-01-01 00:00:00
abstract::Three weeks after inoculation of 24-day-old gnotobiotic dogs with Snyder-Hill canine distemper virus, white matter samples were taken from the primary predilection sites for canine distemper virus-associated demyelination. The plasmalogenase activity in extracts was nearly 6-fold greater than control values for a dog ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00692214
更新日期:1980-01-01 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
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::In hereditary myopathy of the diaphragmatic muscles in Holstein-Friesian cattle, the largest number of acidophilic intracytoplasmic inclusions was found in the myocardium. These inclusions, which were oval and measured 12-15 microm in the transverse sections, were characterized by a dense, amorphous zone, and a relati...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050972
更新日期:1999-02-01 00:00:00
abstract::We have found large eosinophilic bodies in the pericapillary regions of the gray and white matter in the human spinal cord. These are entirely different from the previously reported pericapillary inclusion bodies (PIB). We have designated them pericapillary rosettes (PR), since they consist of clusters of round or ovo...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00299408
更新日期:1992-01-01 00:00:00
abstract::Morphometric observations have been made on the medial plantar division of the tibial nerve (MPD) and on the motor branches of the tibial nerve to the calf muscles (MBC) in rats ranging in age from weaning (3 weeks) to 12 months. Axon size, assessed by measurements of circumference and cross-sectional area, increased ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00308712
更新日期:1990-01-01 00:00:00
abstract::Cerebral capillaries in "overgrown" neural tissue of chick embryo brains ranging in age from 5-18 days of incubation were studied by means of electron microscopy. The vessels in the abnormal tissue showed more extensive and prolonged interdigitations and overlapping of adjacent endothelial cells than did those in norm...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00684800
更新日期:1979-04-12 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 evolvement of amyloid beta (Abeta) deposition in the frontal cerebral cortex of 24 patients of increasing age with Dutch-type hereditary cerebral hemorrhage with amyloidosis (HCHWA-D) was studied using end-specific monoclonal antibodies to Abetax-42 (Abeta42) or Abetax-40 (Abeta40) and markers for degenerating neu...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051143
更新日期:2000-04-01 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 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