Abstract:
:Following maternal chicken pox in the 14th week of pregnancy, a male infant was born with low birth weight, muscle wasting and limb contractures, hypotonia and areflexia. A rising titre of varicella-zoster-specific IgM (by enzyme-linked immunoabsorbent assay) confirmed congenital infection, and electromyogram showed widespread denervation. Death occurred at 8 days. Neuropathological examination revealed destructive and inflammatory lesions of cerebral cortex and white matter, thalamus, brain stem and spinal cord. In addition there were cerebellar heterotopias and bilateral polymicrogryic malformations of the insular cortex.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Harding B,Baumer JAdoi
10.1007/BF00687781subject
Has Abstractpub_date
1988-01-01 00:00:00pages
311-5issue
3eissn
0001-6322issn
1432-0533journal_volume
76pub_type
杂志文章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::A 24-year-old woman was found comatose after 2 days of cephalalgia and vomiting. An immediate diagnosis of carbon monoxyde poisoning was disclaimed when blood carbon monoxyde was found to be 1.75 ml/100. A diagnosis of acute intracranial hypertension led to trephination with ventricular punction and brain biopsy on th...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00685010
更新日期:1978-08-07 00:00:00
abstract::A significant reduction in the myelinated nerve fiber population was observed during quantitative electron-microscopic examination of peripheral nerves in chronic alloxan diabetic rats. Dystrophic axonal abnormalities and regenerating fibers were more numerous in diabetics than age-matched controls. Schwann cells show...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690466
更新日期:1984-01-01 00:00:00
abstract::Aging and neurodegeneration are often accompanied by a functionally impaired ubiquitin-proteasome system (UPS). In tauopathies and polyglutamine diseases, a mutant form of ubiquitin B (UBB(+1)) accumulates in disease-specific aggregates. UBB(+1) mRNA is generated at low levels in vivo during transcription from the ubi...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-012-1003-7
更新日期:2012-08-01 00:00:00
abstract::Neurological signs were observed in 3 lambs at approximately 1 month of age, in a flock of 1 ram and 29 ewes with 43 lambs. Deterioration occurred such that the lambs had either died or been killed by 4 months of age. Necropsies of two of these lambs revealed a diffuse encephalopathy in which the most prominent featur...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00309632
更新日期:1995-01-01 00:00:00
abstract::Uniform neuropathological changes are described in eight cases of the progressive encephalopathy syndrome with edema, hypsarrhythmia and optic atrophy (PEHO syndrome). Two of the autopsied patients were sisters and two other cases were familial. Macroscopically, cerebral and pronounced cerebellar atrophy was seen, the...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00227717
更新日期:1993-01-01 00:00:00
abstract::Basal ganglionic lesions in three young infants were found in a prospective search for early stages of status marmoratus. The lesions had formed and had passed into glial scars well before myelination of the basal ganglia commenced. The myelinating fibers crossing the scarred tissue were disoriented; however, fiber st...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688558
更新日期:1977-05-16 00:00:00
abstract::A total of 66 skin biopsies from persons with Alzheimer's disease (AD) or Down's syndrome (DS) and from persons without AD were used in this study. The age range was from 7 to 89 years. Positive immunoreactivity of skin biopsies to monoclonal antibody 4G8, which is reactive to amino acid residue 17-24 of synthetic amy...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00293394
更新日期:1994-01-01 00:00:00
abstract::We evaluated by in situ nick end labeling the presence of apoptotic glial cells in the spinal cord of rats which have sustained a moderate and severe compression injury at the level of T8-9, resulting in a severe but reversible paraparesis and irreversible paraplegia, respectively. In a previous investigation we found...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051112
更新日期:1999-11-01 00:00:00
abstract::Damage to axons in acute multiple sclerosis (MS) lesions is now well established but the mechanisms of this damage remain obscure. Here we have applied a panel of antibodies that identify cell populations and proteins contained in them with a view to detecting those cells and proteins that are localised particularly c...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-006-0045-0
更新日期:2006-04-01 00:00:00
abstract::Focal cortical dysplasia (FCD) is a localized malformation of cortical development and is the commonest cause of severe childhood epilepsy in surgical practice. Children with FCD are severely disabled by their epilepsy, presenting with frequent seizures early in life. The commonest form of FCD in children is character...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-013-1135-4
更新日期:2013-08-01 00:00:00
abstract::Multiple sclerosis (MS) is the most frequent demyelinating disease in young adults and despite significant advances in immunotherapy, disease progression still cannot be prevented. Promotion of remyelination, an endogenous repair mechanism resulting in the formation of new myelin sheaths around demyelinated axons, rep...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-020-02217-8
更新日期:2020-11-01 00:00:00
abstract::The mechanism of antibody-mediated central nervous system (CNS) demyelination in vivo was studied using rabbit eyes. Injection of anti-galactocerebroside (Gal C) antiserum alone into the normal rabbit vitreous body induced demyelination in the epiretinal myelinated fibers. This activity of the antiserum disappeared af...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687890
更新日期:1989-01-01 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
abstract::Disturbance of intracellular trafficking plays a major role in several neurodegenerative disorders including Alzheimer or Parkinson's disease. The Chediak-Higashi syndrome (CHS), a life-threatening autosomal recessive disease with frequent mutations in the LYST gene, and its animal model, the beige mouse, are both cha...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-006-0092-6
更新日期:2006-09-01 00:00:00
abstract::Vascular smooth muscle cells are involved in deposition of amyloid in brain blood vessels. Accumulation of amyloid-beta peptide (Abeta) in cultured brain vascular smooth muscle cells that overexpress human amyloid-beta precursor protein (APP) Swedish, is strongly enhanced by exposure to iron ions. We studied cellular ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-009-0497-0
更新日期:2009-05-01 00:00:00
abstract::To investigate the pathogenetic role of human T lymphocyte virus type I (HTLV-I) in central nervous system disease, a rat model for HTLV-I-associated myelopathy/tropical spastic paraparesis, designated as HAM rat disease, has been established. Wistar-King-Aptekman-Hokudai strain rats with induced HTLV-I infection deve...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050962
更新日期:1999-02-01 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::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::In search of novel genes associated with glioma pathogenesis, we have previously shown frequent deletions of the KIAA1797/FOCAD gene in malignant gliomas, and a tumor suppressor function of the encoded focadhesin impacting proliferation and migration of glioma cells in vitro and in vivo. Here, we examined an associati...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-019-02067-z
更新日期:2020-01-01 00:00:00
abstract::Respiratory chain dysfunction leads to reactive oxygen species (ROS) generation with following oxidative stress and cellular damage. A histochemical and immunohistochemical study was performed on muscle biopsies from 17 patients with mitochondrial disease [chronic progressive external ophthalmoplegia (CPEO), mitochond...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010100455
更新日期:2002-03-01 00:00:00
abstract::The unfolded protein response (UPR) is a stress response of the endoplasmic reticulum (ER) to a disturbance in protein folding. The so-called ER stress sensors PERK, IRE1 and ATF6 play a central role in the initiation and regulation of the UPR. The accumulation of misfolded and aggregated proteins is a common characte...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-015-1462-8
更新日期:2015-09-01 00:00:00
abstract::Modern neuropathology serves a key function in the multidisciplinary management of brain tumor patients. Owing to the recent advancements in molecular neurooncology, the neuropathological assessment of brain tumors is no longer restricted to provide information on a tumor's histological type and malignancy grade, but ...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-010-0736-4
更新日期:2010-11-01 00:00:00
abstract::Induction of differentiation is an attractive approach to the management of infiltrative tumors such as malignant glioma. Here, we report that lovastatin and phenylacetate induce apoptosis, but fail to induce differentiation, in malignant glioma cell lines and untransformed rat astrocytes. Lovastatin and phenylacetate...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010000254
更新日期:2001-03-01 00:00:00
abstract::With increasing longevity, the number of older schizophrenic patients is growing. Previous criteria used the age of symptom onset to differentiate between the late manifestations of early-onset schizophrenia and late-onset schizophreniform disorders. Current DSM-IV or ICD 10 nomenclatures do not differentiate between ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-001-0468-6
更新日期:2002-04-01 00:00:00
abstract::Polyclonal antibodies directed towards synaptophysin were raised against a synthesised peptide corresponding to amino acids 246 to 260 of the human synaptophysin sequence. The antibodies, when applied for immunocytochemical staining, showed a staining pattern identical to that of the commercially available monoclonal ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00293325
更新日期:1994-01-01 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::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::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::Three weeks after inoculation of 24-day-old gnotobiotic dogs with Snyder-Hill canine distemper virus, white matter samples were taken from the primary predilection sites for canine distemper virus-associated demyelination. The plasmalogenase activity in extracts was nearly 6-fold greater than control values for a dog ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00692214
更新日期:1980-01-01 00:00:00