Abstract:
:We described the first two unrelated Polish families with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). In the morphological examination with light microscopy, two kinds of changes were observed: (1). panarteritis nodosa-like changes with eosinophilic fibrinoid necrosis of the vessel wall and perivascular inflammatory infiltrates and (2). basophilic granular material in the tunica media characteristic of CADASIL. At electron microscopy, we found deposits of granular osmophilic material (GOM) within the wall of arteries, veins and capillary vessels. Our findings imply two questions requiring further investigation: Why in the genetically determined vascular disorder are the features of systemic inflammatory vascular disease present? Why in capillary walls deprived of smooth muscle cells are deposits of GOM present?
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Rafalowska J,Fidziańska A,Dziewulska D,Podlecka A,Szpak GM,Kwieciński Hdoi
10.1007/s00401-003-0764-4subject
Has Abstractpub_date
2003-12-01 00:00:00pages
569-74issue
6eissn
0001-6322issn
1432-0533journal_volume
106pub_type
杂志文章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::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::Recent studies of genetic abnormalities in pediatric low-grade gliomas (LGGs) have focused on activation of the ERK/MAPK pathway by KIAA1549-BRAF gene fusions in the majority of pilocytic astrocytomas (PAs) and by rare mutations in elements of the pathway across histopathologically diverse LGGs. This study reports tha...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-010-0763-1
更新日期:2010-12-01 00:00:00
abstract::3-Nitropropionic acid (NPA), a toxin which irreversibly inhibits the Krebs cycle enzyme succinate dehydrogenase, causes severe neurologic disease and a specific pattern of morphologic brain damage when given subcutaneously to rats. To determine whether hypotension or hypoxemia were necessary for development of morphol...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688340
更新日期:1987-01-01 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::Investigations of the existence and distribution of muscle spindles and tendon organs in the human voluntary anal sphincter muscle in 53 cases of autopsy material from both female and male persons (age: 27th week of gestation to 81 years) brought forward the following results: Similar to animals, muscle spindles are f...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691860
更新日期:1984-01-01 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::Stereological estimates of mean volumes, surface areas, and cortical thicknesses were obtained on formalin-fixed brains from 19 men with AIDS and 19 controls. Volumes of neocortex, white matter, central brain nuclei, ventricles and archicortex were estimated using point counting and Cavalieri's unbiased principle for ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00334671
更新日期:1993-01-01 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 mixed lymphoblastic T cell lymphoma and gonadotroph cell pituitary adenoma occurred 25 years after first resection of the adenoma. Within 1 year the lymphoma overgrew the adenoma, but was still restricted to the sellar region. Histologically, lymphoma and adenoma components were tightly admixed. Possible pathogeneti...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050990
更新日期:1999-03-01 00:00:00
abstract::We recently examined the brain from an 8-month-old infant with Down's syndrome and found argyrophilic plaque-like deposits throughout the neocortex and cerebellum. To ascertain the specificity of this observation, we examined 27 additional brains from the pediatric autopsy service, including 1 from another patient wit...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294258
更新日期:1995-01-01 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::The central distal axonopathy induced in dogs by the administration of high doses of clioquinol is contrasted with the central-peripheral distal axonopathy precipitated by intoxication with 2,5-hexanedione. Mature, pure-bred Beagle dogs received a daily oral dose of 400 mg/kg of clioquinol for up to 7 months, or 1 ml ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690549
更新日期:1979-08-01 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::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 studied a 27-year-old woman who died after a 6-year history of progressive dementia, dystonia, ataxia, apraxia, spasticity, choreoathetosis, visual and auditory hallucinations, and optic atrophy. Magnetic resonance imaging showed decreased intensity in the globus pallidus, substantia nigra, and dentate nuclei in T2...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010000224
更新日期:2000-11-01 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::An unresolved issue in the study of demyelinating disease is whether blood-brain barrier damage is dependent upon the migration of inflammatory cells into the central nervous system (CNS). In a study of experimental autoimmune encephalomyelitis (EAE) in rabbits, a freeze-dried, paraffin-embedded tissue technique was e...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00692851
更新日期:1987-01-01 00:00:00
abstract::Three cases of spinal tanycytic ependymoma are reported, a man aged 45 years and two women aged 36 and 55 years. Each patient developed gradual paraparesis over a few months prior to admission. Magnetic resonance imaging showed an enhancing, well-circumscribed tumor in the spinal cord in each case. Histologically, the...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010000265
更新日期:2001-01-01 00:00:00
abstract::The present investigation examined the morphological characteristics of epiplexus macrophages following a single intracisternal injection of the antigen, bacillus Calmette-Guerin (BCG). Three days following injection of BCG (0.5 - 4.0 X 10(8) viable microorganisms), mongrel dogs were perfused with buffered aldehydes. ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690545
更新日期:1979-08-01 00:00:00
abstract::Axons of the peripheral nervous system (PNS) are reduced in caliber in response to the experimental diabetic state. The cause of this reduced axonal size is disputed. Various theories include (a) axonal dwindling, (b) inhibition of growth, and (c) shrinkage due to serum hyperosmolarity. This study was designed to dire...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688637
更新日期:1985-01-01 00:00:00
abstract::Although it is known that deletions or mutations of the SMN1 gene on chromosome 5 cause decreased levels of the SMN protein in subjects with proximal autosomal recessive spinal muscular atrophy (SMA), the exact sequence of pathological events leading to selective motoneuron cell death is not fully understood yet. In t...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-008-0411-1
更新日期:2008-09-01 00:00:00
abstract::Two experiments were completed in which brains and spinal cords from lambs affected with spongy degeneration of the central nervous system (CNS) were analysed for water, sodium and potassium, as measures of cerebral oedema. In a third experiment, lambs with CNS spongy degeneration were tested for permeability of cereb...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687928
更新日期:1975-01-01 00:00:00
abstract::The effect of induced hypertension instituted after a 2-h delay following middle cerebral artery occlusion (MCAO) on brain edema formation and histochemical injury was studied. Under isoflurane anesthesia, the MCA of 14 spontaneously hypertensive rats was occluded. In the control group (n = 7), the mean arterial press...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00305878
更新日期:1991-01-01 00:00:00
abstract::Four of seven patients with nemaline myopathy had severe, rapidly progressing symptoms. These four showed an increase in acid phosphatase activity in muscle fibers demonstrated by histochemistry and cathepsin B&L activity by biochemical measurement. On electron microscopy, nemaline bodies, occasionally disorganized my...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687709
更新日期:1989-01-01 00:00:00
abstract::Fifteen cases of mitochondrial myopathy, three cases of hereditary motor and sensory neuropathy (HMSN) VI, and 280 cases of neuropathies of different etiologies were examined by electron microscopy for the presence of mitochondrial abnormalities in the sural nerve. Altered mitochondrial were found in most cases of mit...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/BF00293381
更新日期:1991-01-01 00:00:00
abstract::Mineralization in the wall of central nervous system blood vessels is sporadically encountered in aged horses and cattle as in man, generally as an age-related change. This phenomenon has not to date been located in the meninges in dogs or cats. The present study reports a retrospective histological examination of 50 ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-002-0641-6
更新日期:2003-03-01 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::Changes in morphology, behavior of the blood-brain barrier (BBB), regional cerebral blood flow (rCBF), and local cerebral glucose utilization (LCGU) were assessed and correlated in Mongolian gerbils following 5 min cerebral ischemia, produced by bilateral clamping of the common carotid arteries. The morphological chan...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691868
更新日期:1983-01-01 00:00:00
abstract::Considering the possibility of a paracellular pathway for edema resolution, we studied the intracerebral movement of proteins and ionic lanthanum in rats with experimental hydrocephalus. Hydrocephalus was induced by injection of kaolin suspension into the cisterna magna. After induction of hydrocephalus, horseradish p...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687004
更新日期:1985-01-01 00:00:00