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 previously. We investigated pyramidal signs, including hyperreflexia, Babinski sign, and spasticity, and involvement of the primary motor cortex and pyramidal tract, focusing on the astrocytosis of the fifth layer of the primary motor cortex. Pyramidal signs were observed in six (60%) of the ten cases. Hyperreflexia was evident in six patients (60%), with spasticity being observed in three patients (30%). Loss of Betz cells associated with prominent astrocytosis and presence of ballooned neurons in the fifth layer of the primary motor cortex was observed in all ten cases. In all cases, involvement of the pyramidal tract was obvious in the medulla oblongata, without involvement of the pyramidal tract in the midbrain. Constant and severe involvement of the fifth layer of the primary motor cortex, including the Betz cells, has not previously been reported in CBD. We suggest that the pyramidal signs in CBD have been disregarded.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Tsuchiya K,Murayama S,Mitani K,Oda T,Arima K,Mimura M,Nagura H,Haga C,Akiyama H,Yamanouchi H,Mizusawa Hdoi
10.1007/s00401-004-0966-4subject
Has Abstractpub_date
2005-04-01 00:00:00pages
353-66issue
4eissn
0001-6322issn
1432-0533journal_volume
109pub_type
杂志文章,评审abstract::Laquinimod (LAQ) is a new oral immunomodulatory compound that reduces relapse rate, brain atrophy and disability progression in multiple sclerosis (MS). LAQ has well-documented effects on inflammation in the periphery, but little is known about its direct activity within the central nervous system (CNS). To elucidate ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-012-1009-1
更新日期:2012-09-01 00:00:00
abstract::Severe hypoxic-ischemic cerebral damage was produced in 8-day-old rats following permanent bilateral carotid artery occlusion and 15 min of ischemia. Cellular damage consisted of early necrosis and appearance of cells with apoptotic-like morphology (karyorrhectic cells) and cells with granular chromatin degeneration i...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050753
更新日期:1997-12-01 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::Neuropeptides have turned out to be promising new parameters, in addition to the routinely performed histochemical diagnosis, of Hirschsprung's disease (HD). Studies of the peptidergic innervation of the affected intestinal segment of patients with HD have demonstrated a marked reduction in the density of several neur...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-002-0613-x
更新日期:2003-01-01 00:00:00
abstract::Histochemical and electron microscopic studies of the brains inclusive of the leptomeninges containing large blood vessels from 7 patients with mucopolysaccharidosis (MPS) I, II, IIIA and V showed marked increase in mesenchymal elements and the generalized presence of characteristic lesions around cerebral veins and a...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690379
更新日期:1977-07-15 00:00:00
abstract::Mice develop a fatal encephalomyelitis after infection with the Trinidad donkey strain of Venezuelan equine encephalitis (VEE) virus. Adult mice were inoculated intraperitoneally with VEE virus and the brains were examined at different time points. Morphological changes were assessed by histological staining. VEE viru...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050626
更新日期:1997-04-01 00:00:00
abstract::Recent studies have shown that Cerebrolysin can enhance synaptic function and ameliorate synaptodendritic alterations in animal models of neurodegeneration, suggesting a synaptotrophic effect. We hypothesize that Cerebrolysin might exert this effect, in part, by regulating the expression of amyloid precursor protein (...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051012
更新日期:1999-05-01 00:00:00
abstract::Virtually all phases of spinal cord injury pathogenesis, including inflammation, cell proliferation and differentiation, as well as tissue remodeling, are mediated in part by infiltrating monocyte-derived macrophages. It is now clear that these infiltrating macrophages have distinct functions from resident microglia a...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-019-01992-3
更新日期:2019-05-01 00:00:00
abstract::An unusual case of cerebellar neurocytoma with rhabdomyomatous differentiation in a 6-year-old boy is reported. Immunocytochemical and ultrastructural features of the tumour were studied. Abortive synapse formation, the presence of clear vesicles and synaptophysin immunoreactivity of the tumour cells indicated its int...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050805
更新日期:1998-03-01 00:00:00
abstract::Frontotemporal lobar degeneration is the term used to describe the non-Alzheimer clinical syndromes of frontotemporal dementia, semantic dementia and progressive non-fluent aphasia, regardless of the underlying neuropathological features. Considerable progress has been made in recent years in our understanding of the ...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-007-0241-6
更新日期:2007-07-01 00:00:00
abstract::Pontocerebellar hypoplasia type 2 (PCH-2; MIM 277470), an autosomal recessive neurodegeneration with fetal onset, was studied in six autopsies with ages at death ranging between 1 and 22 years. Three patients were distantly related. A case of olivopontocerebellar hypoplasia (OPCH; MIM 225753) was studied for compariso...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-007-0263-0
更新日期:2007-10-01 00:00:00
abstract::A case of classic medulloblastoma that metastasized, despite the absence of local recurrence, to extraneural sites 7 years after treatment is reported. The metastases were, in contrast to the primary tumor, of large cell type and displayed abortive myogenic and, in one site, also rhabdoid differentiation. The primary ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-005-0986-8
更新日期:2005-05-01 00:00:00
abstract::A critical appraisal is presented of nearly two decades of research publications and review articles advocating the bidirectional transcytosis of fluid-phase molecules, most notably native horseradish peroxidase (HRP), through the normal and experimentally modified blood-brain barrier (BBB). Extracellular routes circu...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/BF00294368
更新日期:1989-01-01 00:00:00
abstract::The pro-inflammatory and blood-brain barrier (BBB) effects of intratumoral (IT) injection of human recombinant tumor necrosis factor-alpha (rTNF-alpha) were studied in the Fischer rat RT-2 glioma model. Animals received a single stereotaxic injection of either 6 x 10(4) U rTNF-alpha or excipient (vehicle) into the cen...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/pl00007398
更新日期:1997-01-01 00:00:00
abstract::This report concerns an immunocytochemical and ultrastructural study of the motor cortices of 11 patients with amyotrophic lateral sclerosis (ALS). Specimens from 12 normal individuals served as controls. Antibodies against phosphorylated neurofilament (PNF; 200 kDa), ubiquitin, glial fibrillary acidic protein (GFAP) ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00293318
更新日期:1994-01-01 00:00:00
abstract::Low-grade neuroepithelial tumors (LGNTs) are diverse CNS tumors presenting in children and young adults, often with a history of epilepsy. While the genetic profiles of common LGNTs, such as the pilocytic astrocytoma and 'adult-type' diffuse gliomas, are largely established, those of uncommon LGNTs remain to be define...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-016-1539-z
更新日期:2016-06-01 00:00:00
abstract::The long-term survival of oligodendrocytes in the absence of axons in adult animals was studied following Wallerian degeneration of the optic nerves of adult rats for periods up to 22 months. In contrast to the findings in development and in young animals, large numbers of oligodendrocytes survived during this time pe...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00308922
更新日期:1990-01-01 00:00:00
abstract::The blood-brain barrier (BBB) was studied in rats with electrolyte-induced demyelination (EID), an experimental model for central pontine myelinolysis. Intravenously injected peroxidase was extravasated at 3 h post hypertonic saline injection (PHS) into regions frequently involved in EID. Increased pinocytotic activit...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00310371
更新日期:1994-01-01 00:00:00
abstract::The current classification of human sporadic prion diseases recognizes six major phenotypic subtypes with distinctive clinicopathological features, which largely correlate at the molecular level with the genotype at the polymorphic codon 129 (methionine, M, or valine, V) in the prion protein gene and with the size of ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-012-1002-8
更新日期:2012-10-01 00:00:00
abstract::Ubiquitin-immunoreactive (ub-ir) neuronal cytoplasmic inclusions are characteristically found in the extramotor cortex in patients with motor neuron disease and dementia (MND-dementia) and a subset of patients with frontotemporal dementia without motor symptoms (FTD-MND type). Recently, ub-ir neuronal intranuclear inc...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0678-1
更新日期:2003-06-01 00:00:00
abstract::The left and right recurrent laryngeal nerves and peroneal nerves from two groups of foals, one less than 1 month of age and the other 6 months of age, were examined by light and electron microscopy. While there was no evidence of fiber loss on light microscopy, occasional onion bulbs, regenerating clusters and swolle...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00227825
更新日期:1992-01-01 00:00:00
abstract::Activation of the MAPK signaling pathway has been shown to be a unifying molecular feature in pilocytic astrocytoma (PA). Genetically, tandem duplications at chromosome 7q34 resulting in KIAA1549-BRAF fusion genes constitute the most common mechanism identified to date. To elucidate alternative mechanisms of aberrant ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-011-0817-z
更新日期:2011-06-01 00:00:00
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::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::The idiopathic inflammatory myopathies (IIM) are a heterogeneous group of neuromuscular disorders subdivided into polymyositis (PM), sporadic inclusion body myositis (sIBM) and dermatomyositis (DM). Chemokines play an essential role in sustained inflammation associated with IIM. We studied the distribution of the alph...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-005-0989-5
更新日期:2005-06-01 00:00:00
abstract::Lysolecithin has been used in many studies to induce demyelination in peripheral nerves. In the present investigation lysolecithin (lysophosphatidyl choline) was injected into rat sciatic nerves at a dose of 2-3 microns of a 10 mg/ml solution in order to study the effects of this lipid on cellular elements other than ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690634
更新日期:1982-01-01 00:00:00
abstract::Clinical and morphological findings in an 8-year-old Jewish girl with spongy leukodystrophy are presented. Ultrastructural changes indicated that this may be a form of Canavan's disease with some atypical features. Biochemical measurements indicated that synaptosomal membranes prepared from the striatum but not from t...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691321
更新日期:1981-01-01 00:00:00
abstract::The present study used cocultures of rat dorsal root ganglia (DRG) and peritoneal macrophages to define the role of activated complement components during demyelination. The complement cascade was activated in vitro by treatment of the cultures with natural rat serum and lipopolysaccharides. Complement activation was ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00318572
更新日期:1995-01-01 00:00:00
abstract::We examined the effect of lactic acid on cultured human glioma cell lines expressing glial fibrillary acidic protein (GFAP), vimentin and neuron-specific enolase (NSE). The growth of the cells was inhibited by the lactic acid in a dose-dependent manner. At 56 mM of lactic acid, the surviving cells of the KNS-42-c2 cel...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00296110
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