Abstract:
:Amyotrophic lateral sclerosis (ALS) and adult onset X-linked recessive bulbospinal muscular atrophy (SPMA), constituting the category of adult onset form of motor neuron disease, were analyzed on motor nerve roots. The results of morphometric analysis on ventral spinal roots (VSR) of all spinal segments from ALS and SPMA revealed the following three findings: (1) the large-myelinated alpha-motoneuron fibers were markedly decreased in number throughout all segments; (2) thin-myelinated autonomic preganglionic fibers were almost completely preserved; (3) small-intermediate-myelinated fibers which are considered to correspond to gamma-motoneuron fibers were generally well preserved in ALS, but decreased by one-half to one-third in SPMA. However, all the components of the nerve roots of the oculomotor, trochlear, and abducent nerves were completely preserved in both ALS and SPMA. Moreover, the teased-fiber study showed that the regenerating-sprouting process rarely occurred in the VSR of ALS and SPMA. The present study suggested that the site of the primary lesion seems to be in the alpha-motoneuron fibers in motor neuron diseases, such as ALS or SPMA. However, the marked discrepancy in the pathologic change in the alpha-motoneuron fibers in the VSR and the nerve roots innervating the external ocular muscles was noteworthy.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Sobue G,Matsuoka Y,Mukai E,Takayanagi T,Sobue I,Hashizume Ydoi
10.1007/BF00691322subject
Has Abstractpub_date
1981-01-01 00:00:00pages
227-35issue
3eissn
0001-6322issn
1432-0533journal_volume
55pub_type
杂志文章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::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::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::A neuropathological study is presented of a case showing the association of tuberous sclerosis of the brain and dysplasia of the corpus callosum as well as omphalocele and malrotated colon. No signs of tuberous sclerosis were found in the internal organs. From a review of the literature this appears to be the fourth c...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF01273269
更新日期:1978-04-26 00:00:00
abstract::Changes in immature rats in motor neurones after axotomy were studied by enzyme-histochemical methods. Increased activity of dehydrogenases in these neurones demonstrates enhanced metabolism and there was also increase of acid phosphatases. Decreased activity of acetylcholinesterase and indoxylacetate esterase in the ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00686163
更新日期:1975-12-30 00:00:00
abstract::During corticogenesis, neurons adopt different migration pathways to reach their final position. The precursors of pyramidal neurons migrate radially, whereas most of the GABA-containing interneurons are generated in the ventral telencephalon and migrate tangentially into the neocortex. Then, they use a radial migrati...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-010-0692-z
更新日期:2010-10-01 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::The extent of DNA fragmentation analysed using the TUNEL technique was evaluated in post-mortem human brain tissue. Twenty-four patients with clinical and histopathological diagnosis of Alzheimer's disease (AD) and a short post-mortem delay were analysed. We report an increase in the count of TUNEL-labelled cells as t...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010000228
更新日期:2000-12-01 00:00:00
abstract::Three female cats, littermates born from clinically normal parents, were examined at 8 to 10 weeks of age because of a slowly progressive posterior ataxia. Another cat from a previous litter from the same parents suffered from similar neurological symptoms. Histopathological examination of the nervous tissues of these...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00293957
更新日期:1991-01-01 00:00:00
abstract::Gliomas induced in the rat by transplacental administration of ethylnitrosourea (ENU) are intensely immunoreactive for vimentin and scarcely for glial fibrillary acidic protein (GFAP). Since tumoral transformation takes place during the late fetal and early postnatal period, the sequential expression of the two glial ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00227665
更新日期:1992-01-01 00:00:00
abstract::In spite of several cases reported in the literature, the exact pathogenetic mechanism of neuropathic changes in porphyric neuropathy remains uncertain. Various authors have ascribed the neuropathologic findings to either a dying-back axonal degeneration or segmental demyelination. In recent years, the hypothesis of a...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00697191
更新日期:1984-01-01 00:00:00
abstract::We report two sporadic cases of tauopathy with unusual neuropathological features. The ages of the patients at death were 86 and 74 years, and the disease durations were 4 and 3 years, respectively. The former patient showed progressive dementia and amyotrophy (autopsy revealed that severe cervical spondylosis was res...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-006-0093-5
更新日期:2006-09-01 00:00:00
abstract::This report describes three brothers belonging to a consanguineous family suffering from a progressive neurological disorder associated with L-2-hydroxyglutaric aciduria. Clinically this disorder is characterized by childhood onset, pyramidal signs, cerebellar and pseudobulbar syndromes and epilepsy. Pathological exam...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00310381
更新日期:1994-01-01 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::Cyclooxygenases (COX, prostaglandin endoperoxide synthases, PGG/H synthases) are potent mediators of inflammation. While COX-1 is constitutively expressed in a wide range of tissues, COX-2 is cytokine inducible. Although COX-1 expression is observed in normal tissue, enhanced COX-2 expression has been attributed a key...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051075
更新日期:1999-09-01 00:00:00
abstract::In an autopsy case of the sporadic juvenile ALS (a 17-year-old girl) intracytoplasmic inclusions are found in the upper and lower motor neurons and in nerve cells of the dentate nucleus, pontine nucleus, brain stem reticular formation, substantia nigra, thalamus, globus pallidus and others. Histochemically they contai...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691069
更新日期:1978-12-15 00:00:00
abstract::Amplification of the epidermal growth factor receptor (EGFR) gene is found in about 40% of glioblastomas (GBMs) but is rarely detected in GBM cell lines. We confirmed that the exceptional SKMG-3 GBM cell line retained amplified EGFR genes in vitro, and found that these sequences were concentrated on extra-chromosomal ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010000332
更新日期:2001-06-01 00:00:00
abstract::A 1-year-old male chinchilla with a 2-week history of conjunctivitis suffered subsequently from neurological signs comprising seizures, disorientation, recumbency and apathy. After 3 weeks of progressive central nervous disease the animal was killed in view of the poor prognosis. A non-suppurative meningitis and polio...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-002-0597-6
更新日期:2002-12-01 00:00:00
abstract::Huntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by an expanded trinucleotide CAG repeat in the gene coding for huntingtin. Deregulation of chromatin remodeling is linked to the pathogenesis of HD but the mechanism remains elusive. To identify what genes are deregulated by trimethyla...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-013-1103-z
更新日期:2013-05-01 00:00:00
abstract::Cerebrotendinous xanthomatosis (CTX), a rare autosomal-recessive lipid storage disease, has been well characterized clinically and biochemically, and recently also from the molecular biological aspect. However, only a very few publications deal with its neuropathology, and views on its pathogenesis vary. Based on a re...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294324
更新日期:1995-01-01 00:00:00
abstract::Neurofibromas represent one of the hallmarks of neurofibromatosis 1 (NF1) patients. Tumor progression of neurofibromas to malignant peripheral nerve sheath tumors (MPNST) is a frequent and life threatening complication. To learn more about processes involved in malignant transformation, we evaluated differential gene ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0797-8
更新日期:2004-02-01 00:00:00
abstract::The dynamics of cell-associated Concanavalin A (Con A) in astrocytes of the newborn rat (RNA), the rat glioma (AC), and the human glioblastoma (GB) were studied in vitro by fluorescence and electron microscopy. Con A receptors on the cell surface were seen usually as a continuous thin layer, and Con A accumulations in...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690804
更新日期:1982-01-01 00:00:00
abstract::Considering the possibility of a paracellular route for edema resolution we studied the microvasculature of the subependymal and subcortical white matter in hydrocephalic rats. Normal adult rats were used as controls. After injection of kaolin suspension into the cisterna magna, the animals were killed at intervals of...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00695575
更新日期:1984-01-01 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 gross and histopathological findings in the brain and spinal cord of five Alaskan Husky dogs with a novel incapacitating and ultimately fatal familial and presumed hereditary neurodegenerative disorder are described. Four dogs presented with neurological deficits before the age of 1 year (7-11 months) and one anim...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051192
更新日期:2000-07-01 00:00:00
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::Flurothyl-induced status epilepticus was studied by light and electron microscopy (LM, EM) to determine the time course and structural features of neuronal necrosis in the vulnerable brain regions in epilepsy. The cerebral cortex, hippocampus and thalamus were examined after closely spaced recovery periods of up to 1 ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687789
更新日期:1988-01-01 00:00:00
abstract::Pediatric glioblastomas (GBM) including diffuse intrinsic pontine gliomas (DIPG) are devastating brain tumors with no effective therapy. Here, we investigated clinical and biological impacts of histone H3.3 mutations. Forty-two DIPGs were tested for H3.3 mutations. Wild-type versus mutated (K27M-H3.3) subgroups were c...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-012-0998-0
更新日期:2012-09-01 00:00:00
abstract::Chronic wasting disease (CWD), a progressive neurological disorder of captive mule deer, black-tailed deer, hybrids of mule deer and white-tailed deer and Rocky Mountain elk, is characterized neuropathologically by widespread spongiform change of the neuropil, intracytoplasmic vacuolation in neuronal perikarya and ast...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00310125
更新日期:1991-01-01 00:00:00
abstract::This report presents the largest series of consecutive, neuropathologically confirmed cases of frontotemporal degeneration (FTD). Prior studies have found dementia lacking distinctive histology (DLDH) to be the most common pathology underlying the clinical diagnosis of FTD. In this series of 76 cases, 29 (38%) were fo...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-004-0900-9
更新日期:2004-11-01 00:00:00