Abstract:
:A 56-day-old infant with alpha-neuraminidase deficiency, whose clinical features included severe edema of extremities and ascites which resembled those in severe infantile sialidosis, was autopsied. Perforation, whose pathogenesis was unclear, was found on the descending portion of the duodenum. Light and electron microscope studies showed that neurons in the cerebral and cerebellar corticies, and the thoracic spinal cord contained membrane-bound vacuoles but no membranous cytoplasmic bodies. Zebra bodies were found only in the neurons of the spinal cord. The neurons in the paraganglion and in the Auerbach's myenteric plexus were also distended with numerous membrane-bound vacuoles. Hepatocytes, endothelial cells and Kupffer cells in the liver and glomerular and tubular epithelial cells in the kidney were swollen with a number of vacuoles, although the patient showed none of the clinical features of renal involvement. These pathological changes were similar to those in nephrosialidosis reported by Le Sec et al. [Arch Fr Pediatr 35:819-829 (1978)].
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Yamano T,Shimada M,Matsuzaki K,Matsumoto Y,Yoshihara W,Okada S,Inui K,Yutaka T,Yabuuchi Hdoi
10.1007/BF00688050subject
Has Abstractpub_date
1986-01-01 00:00:00pages
278-84issue
3-4eissn
0001-6322issn
1432-0533journal_volume
71pub_type
杂志文章abstract::A monoclonal antibody, raised against extracts from Alzheimer brain, that recognizes a phosphorylated epitope in high molecular weight neurofilament proteins and tau proteins also immunostains Alzheimer neurofibrillary tangles, neurites in senile plaques and granulovacuolar degeneration. This result suggest that granu...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00686619
更新日期:1987-01-01 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::Altered levels of cerebrospinal fluid (CSF) peptides related to Alzheimer's disease (AD) are associated with pathologic AD diagnosis, although cognitively normal subjects can also have abnormal levels of these AD biomarkers. To identify novel CSF biomarkers that distinguish pathologically confirmed AD from cognitively...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-010-0667-0
更新日期:2010-06-01 00:00:00
abstract::A patient with mixed gonadal dysgenesis showed glove and stocking-type sensory impairment and slowing of motor and sensory nerve conduction. Sural nerve biopsy revealed minifascicular formation with decreased density of myelinated fibers. As far as we are aware, this is the first report of polyneuropathy with minifasc...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051085
更新日期:1999-09-01 00:00:00
abstract::A case of secretory meningioma with numerous meningothelial rosettes is reported. A 66-year-old man with moyamoya disease gradually developed skull deformity, and underwent surgery for the skull tumor overlying the hemisphere. Histological examination disclosed numerous meningothelial rosettes quite similar to those i...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00227831
更新日期:1992-01-01 00:00:00
abstract::Using a 3H-labelled virion DNA probe applied to tissue sections, we have previously identified the precise microscopic anatomical location of herpes simplex virus (HSV) during the acute and latent stages of infection of the mouse trigeminal ganglia and central nervous system (CNS). In the present investigation, we com...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00692842
更新日期:1987-01-01 00:00:00
abstract::Chronic traumatic encephalopathy (CTE) is a progressive degenerative disorder associated with repetitive traumatic brain injury. One of the primary defining neuropathological lesions in CTE, based on the first consensus conference, is the accumulation of hyperphosphorylated tau in gray matter sulcal depths. Post-morte...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-017-1686-x
更新日期:2017-03-01 00:00:00
abstract::An 18 month old cat had a 6 month history of abnormal high stepping gait, ataxia, muscle twitching and loss of sensory perception. The clinical course was interrupted by several episodes of temporary remission. The essential pathological features were those of a polyradiculoneuritis with segmental demyelination, focal...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00689561
更新日期:1978-01-19 00:00:00
abstract::A macrosomic male infant with multiple malformations survived for 4 days. His external dysplasias comprised macrocephalus, cheilopalatoschisis, auricular anomalies, and unilateral hexadactyl; his internal dysplasias included cysts of kidneys and pancreas, and a patent foramen ovale. The child had frequent generalized ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690449
更新日期:1980-01-01 00:00:00
abstract::It is well established that the binding of pathogenic aquaporin-4 (AQP4)-specific autoantibodies to astrocytes may initiate a cascade of events culminating in the destruction of these cells and in the formation of large tissue-destructive lesions typical for patients with neuromyelitis optica spectrum disorders (NMOSD...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-018-1950-8
更新日期:2019-03-01 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::Glycerol (an atoxic alcohol) and phenol (a toxic monohydroxybenzene) are currently used as neurolytic blocking agents to relieve pain or spasticity. In the present study we compared the endoneurial response of anhydrous glycerol and 7% phenol-aqua after intraneural injection into rat sciatic nerve, using electron micr...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010000349
更新日期:2001-07-01 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::APO2 ligand (APO2L)/TRAIL is a novel member of the tumor necrosis factor cytokine family and a potent inducer of apoptosis in tumor cell lines. We recently reported that APO2L is consistently expressed in low-grade astrocytomas, anaplastic astrocytomas, glioblastomas, and cell lines derived thereof, and that malignant...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/pl00007399
更新日期:2000-01-01 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::A limited cortical resection including the rolandic fissure and the pre- and postcentral cortical regions was carried out in a patient suffering from epilepsia partialis continua resistant to antiepileptic drugs. The histological examination revealed several foci of very large neurons distributed with no laminar organ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00299415
更新日期:1992-01-01 00:00:00
abstract::The pathological changes in the nucleus basalis of Meynert (nbM) in 10 autopsied cases with Pick's disease were studied in comparison with 15 age-matched controls. Both the number and density of nerve cells and the degree of fibrillary gliosis were examined at the anterior, intermediate, and posterior divisions of the...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687402
更新日期:1989-01-01 00:00:00
abstract::Progression of neuritic dystrophy is a histological hallmark of Alzheimer's disease (AD) in addition to amyloid deposition and neurofibrillary tangle formation. Dystrophic neurites (DNs) are abnormal neurites, and are closely associated with amyloid deposits. To clarify the process of DN formation, we immunohistochemi...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-004-0907-2
更新日期:2004-11-01 00:00:00
abstract::Plaque-type deposition of prion protein (PrP) in the brain has been extremely rare in sporadic Creutzfeldt-Jakob disease patients with methionine homozygosity at polymorphic codon 129 of the PrP gene and type 1 abnormal isoform of PrP (sCJD-MM1). Here we report three sCJD-MM1 patients who showed prominent PrP-positive...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-008-0425-8
更新日期:2008-11-01 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::The microtubule-associated protein tau accumulates as cytoplasmic inclusions in Alzheimer's disease (AD), Pick's disease (PiD), progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD). We investigated the immunoreactivity of tau-positive structures using a panel of antibodies to epitopes spanning the ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0671-8
更新日期:2003-05-01 00:00:00
abstract::The formation of oedema in peripheral nerves was studied in rats at intervals varying from 6 h to 14 days after transection of the right sciatic nerve. Samples were removed proximal and distal to the injury, and the degree of oedema was determined by a microgravimetric method and by measurements of the water content a...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00686069
更新日期:1986-01-01 00:00:00
abstract::The conventional concept of Pick's disease does not distinguish Pick's disease with Pick bodies (Pick body disease, PBD) from Pick's disease without Pick bodies [lobar atrophy without Pick bodies, LA-PB(-)]. Recently, intraneuronal ubiquitin-positive inclusions (ub-inclusions), which are thought to be a hallmark of am...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-001-0513-5
更新日期:2002-07-01 00:00:00
abstract::Abundant proliferation of cells having the histologic and tinctorial features of microglia, were seen in the brain of 34-year old man who suffered cardiac arrest 10 days before death and whose bone marrow was nearly totally depleted of white-blood-cell precursors. It is suggested, that in adult human brains there are ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691588
更新日期:1978-09-15 00:00:00
abstract::Alpha-synuclein (αSyn) preformed fibrils (PFF) induce endogenous αSyn aggregation leading to reduced synaptic transmission. Neuronal activity modulates release of αSyn; however, whether neuronal activity regulates the spreading of αSyn pathology remains elusive. Here, we established a hippocampal slice culture system ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-020-02227-6
更新日期:2020-12-01 00:00:00
abstract::To assess whether amyloid plaque accumulation in the monkey brain can account for age-related cognitive impairment that begins at about 20 years of age, we measured plaque content in the brains of 14 rhesus monkeys aged 5-30 years. We used immunohistochemistry employing the monoclonal antibody 6E10, which is specific ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050735
更新日期:1997-11-01 00:00:00
abstract::In this study, we used immunohistochemistry to screen for alpha-synuclein pathology in the brains of 241 individuals without clinical evidence of neurologic disease, and discovered 36 cases (15%) with incidental Lewy bodies (LBs) and one case, a 96-year-old woman (0.4%), with inclusions similar to those seen in multip...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-008-0398-7
更新日期:2008-09-01 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::Intermitochondrial septate structures were found in the dystrophic axons of two cases of infantile neuroaxonal dystrophy. Septate structures were previously seen in some tumors (glioblastomas and Schwannomas) and several organs of vertebrates and invertebrates, but never in human central nervous system (CNS). The stru...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688007
更新日期:1980-01-01 00:00:00
abstract::It is conventionally accepted that during the early stages of Wallerian degeneration of myelinated peripheral nerve fibres Schmidt-Lanterman incisures represent the sites at which the myelin sheath, together with enclosed axoplasm, is segmented into myelin ovoids. This mechanism is considered by some authors to be fac...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:
更新日期:1979-11-01 00:00:00