Abstract:
:This ultrastructural study deals with the synapses of primary dendrites of the anterior horn neurons in the lower lumbar spinal cords of seven patients with amyotrophic lateral sclerosis (ALS) who had mild neuronal depletion. Specimens from seven age-matched, neurologically normal individuals served as controls. In each instance, the autopsy was performed within 6 h after death. Our results indicate a statistically significant increase in degenerative changes in the dendrite presynapses of normal-appearing neurons of the ALS patients. The alterations included aggregation of electron-dense synaptic vesicles and dark mitochondria with dense cristae, and bundles of neurofilaments. However, we found no significant difference between controls and patients with respect to cross-sectional area and length of the dendrites, number of synapses per dendrite, and lengths of individual synapses and their active zones in the normal-appearing neurons, even though the patients' neurons had a smaller cross-sectional area. In chromatolytic neurons, the number of synapses and the length of the active zone of the primary dendrites were significantly diminished. These findings suggest that despite degenerative changes of the presynapses, the synapses in the primary dendrites of the anterior horn neurons are preserved at the early stage of ALS. The preservation of these synapses may be due to their relative resistance to degenerative processes, or may represent a compensatory mechanism of the synapses for diminished synaptic function in distal dendrites.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Sasaki S,Iwata Mdoi
10.1007/s004010050426subject
Has Abstractpub_date
1996-01-01 00:00:00pages
278-83issue
3eissn
0001-6322issn
1432-0533journal_volume
91pub_type
杂志文章abstract::A massive expansion of a GGGGCC repeat upstream of the C9orf72 coding region is the most common known cause of amyotrophic lateral sclerosis and frontotemporal dementia. Despite its intronic localization and lack of a canonical start codon, both strands are translated into aggregating dipeptide repeat (DPR) proteins: ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-015-1450-z
更新日期:2015-10-01 00:00:00
abstract::Progressive myopathy developed in two women who consumed ipecac syrup containing emetine hydrochloride to induce vomiting as part of their anorexia nervosa. Muscle biopsy specimens were characterized by severe disruption of the sarcomeres. The ultrastructural spectrum extended from "Z-band streaming" to the formation ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00686202
更新日期:1988-01-01 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::This study was undertaken to elucidate morphological changes in the synaptic area of the Purkinje cell dendritic spines when granule cells were decreased in number. The mice were injected s.c. with 30 mg/kg b.w. of cytosine arabinoside on days 2, 3, and 4, and on days 7, 8 and 9, and were designated as group I and gro...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00685343
更新日期:1983-01-01 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::Neurofibrillary tangles were induced in the motor neurons of the rabbit spinal cord by the intrathecal injection of colchicine, vinblastine, and vincristine. The tangles stained intensely by immunofluorescence and by the peroxidase-anti-peroxidase procedure using neurofilament antisera raised against chicken brain ant...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690460
更新日期:1980-01-01 00:00:00
abstract::We present here an unusual case of papillary neuroepithelial tumor of the pineal region. The patient was a 29-year-old female who presented with headaches. A computed tomography scan revealed a tumorous lesion at the pineal region and hydrocephalus. The resected tumor was composed of columnar and cuboidal cells showin...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-004-0898-z
更新日期:2004-10-01 00:00:00
abstract::Immunohistochemical analysis of inflammatory cell density and infiltrate subpopulations in 42 meningiomas was performed. Evaluation of infiltrating cell density was carried out by cell counting. Meningothelial and fibroblastic meningiomas contained an average of 3% mononuclear cells; the few lymphocytes were localized...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294381
更新日期:1989-01-01 00:00:00
abstract::Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are relentlessly progressive neurodegenerative disorders with overlapping clinical, genetic and pathological features. Cytoplasmic inclusions of fused in sarcoma (FUS) are the hallmark of several forms of FTLD and ALS patients with mutati...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-012-1043-z
更新日期:2013-02-01 00:00:00
abstract::Two spontaneous neurodegenerative diseases of the horse, equine motor neuron disease (EMND) and equine degenerative myeloencephalopathy (EDM), have been associated with alpha-tocopherol deficiency, and both were characterized by prominent accumulations of endothelial lipopigment in the small vessels of the spinal cord...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00296510
更新日期:1995-01-01 00:00:00
abstract::This report illustrates a case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) masquerading as neurofibromatosis due to multifocal enlargements of spinal nerve roots. The patient initially complained of intermittent numbness of the hands and leg weakness at age 62. Nerve conduction velocities were ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-002-0616-7
更新日期:2003-02-01 00:00:00
abstract::A morphometric study of the basal nucleus of Meynert (bnM) has been performed in a 70-year-old man with a 4-year history of pathologically confirmed progressive supranuclear palsy (PSP). An important neuronal loss (52%) was demonstrated in the bnM. This finding has not been previously documented with morphometric meth...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00697397
更新日期:1983-01-01 00:00:00
abstract::Histological, ultrastructural and morphometrical observations on Clarke's column were carried out in 18 patients with sporadic amyotrophic lateral sclerosis (ALS) and 15 age-matched control subjects. Of the 18 ALS patients 6 had been on a respirator before death. Bunina bodies were found in the neuronal cytoplasm in 7...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00304464
更新日期:1992-01-01 00:00:00
abstract::The incidence of CNS lymphoma has increased significantly in the past 30 years, primarily in the elderly and immunocompromised. While T-cell lymphomas comprise 15-20% of systemic lymphomas, they comprise less than 4% of primary CNS lymphomas, suggesting that they may be under-recognized compared to their systemic coun...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-007-0338-y
更新日期:2008-03-01 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::MicroRNA (miRNA) expression was assessed in human cerebral cortical gray matter (GM) and white matter (WM) in order to provide the first insights into the difference between GM and WM miRNA repertoires across a range of Alzheimer's disease (AD) pathology. RNA was isolated separately from GM and WM portions of superior...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-010-0756-0
更新日期:2011-02-01 00:00:00
abstract::Seven patients with the typical clinical picture and muscle biopsy findings of classical Werdnig-Hoffmann disease showed Wallerian degeneration in their biopsied sural nerves. In dorsal root ganglia of one patient there were residual nodules and several chromatolytic neurons. By electron microscopy the changes of chro...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690973
更新日期:1978-05-24 00:00:00
abstract::Large cell/anaplastic (LC/A) medulloblastoma (MB) is a recently recognized variant of medulloblastoma known to be associated with an advanced stage and a poor prognosis. Although Eberhart et al. suggested histopathologic grading of medulloblastoma in 2002, no consensus has been reached in terms of determining the crit...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-006-0073-9
更新日期:2006-07-01 00:00:00
abstract::Cerebellar torpedoes, unique fusiform swellings of Purkinje cell axons within the granular layer, have been known to occur sparsely associated with diffuse cerebellar changes. This report describes, in three human autopsy cases with focal necrotic lesions in the cerebellar white matter, torpedoes which were essentiall...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00311388
更新日期:1992-01-01 00:00:00
abstract::Electron microscopy revealed the presence of cytoplasmic tubular aggregates in the capillary endothelium of a sparsely granulated growth hormone cell adenoma removed surgically from a 25-year-old female patient with acromegaly. To our knowledge, this is the second publication describing these structures in hypophysial...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00684545
更新日期:1977-01-31 00:00:00
abstract::The dynamics of cerebrospinal fluid (CSF) tau and Aβ biomarkers over time in Alzheimer's disease (AD) patients from prodromal pre-symptomatic to severe stages of dementia have not been clearly defined and recent studies, most of which are cross-sectional, present conflicting findings. To clarify this issue, we analyze...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-013-1151-4
更新日期:2013-11-01 00:00:00
abstract::In a previous study the occurrence of nerve fibre degeneration with a distribution as in dying-back neuropathies was described in young rats subjected to severe protein deprivation (Oldfors 1981). In this study the ultrastructural appearance of the degeneration of the nerve fibres at different levels of the longitudin...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688871
更新日期:1982-01-01 00:00:00
abstract::Neuregulins belong to a family of multipotent growth-promoting proteins, and have been shown to have a crucial role in accumulating acetylcholine receptor at neuromuscular junctions. A functional role of neuregulins in muscle regeneration has not yet been identified. Using reverse transcription (RT)-PCR, Western blot ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-007-0198-5
更新日期:2007-04-01 00:00:00
abstract::The cerebrovascular lesions of severe chronic hypertension were studied by light microscopy in perfusion-fixed, subserially sectioned brains from stroke-prone spontaneously hypertensive rats (SHRSP). The leakage and spread of plasma proteins were visualized by immunohistochemical detection of extravasated fibrinogen a...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690831
更新日期:1985-01-01 00:00:00
abstract::Rat sciatic nerve was assayed for total collagen and total protein both immediately post-mortem and at weekly intervals after death. No significant difference was found in collagen and protein content between corresponding nerve segments assayed fresh and up to 4 weeks post mortem. However, variation was found in peri...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00692870
更新日期:1980-01-01 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::Macroautophagy is a dynamic process whereby cytoplasmic components are initially sequestered within autophagosomes. Recent studies have shown that the autophagosome membrane can selectively recognize ubiquitinated proteins and organelles through interaction with adapter proteins such as p62 and NBR1. Both proteins are...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-012-0975-7
更新日期:2012-08-01 00:00:00
abstract::In Alzheimer's disease (AD) and other tauopathies, the cytosolic protein Tau misfolds and forms intracellular aggregates which accumulate within the brain leading to neurodegeneration. Clinical progression is tightly linked to the progressive spread of Tau pathology throughout the brain, and several lines of evidence ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-018-1911-2
更新日期:2018-11-01 00:00:00
abstract::Cornelia de Lange syndrome (CDLS) is a rare multisystemic malformative syndrome of uncertain etiology characterized by severe psychomotor and mental retardation. Here we report the neuropathological analysis of a 35-year-old patient who displayed the classical clinical symptomatology of CDLS. A congenital dysgenesis o...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-002-0562-4
更新日期:2002-09-01 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