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 with Down's syndrome. To our surprise, similar argyrophilic deposits were found in 16 of these cases. The deposits were equally well stained by three different silver stains and had the same size, shape, and distribution in gray matter as the diffuse amyloid plaques commonly seen in adults. However these structures appeared to be amyloid negative. There were no obvious differences in the primary diagnoses amongst the group of patients with argyrophilic deposits and the group without them, and the origin, permanence, and functional significance of these plaque-like deposits are still unknown. Nonetheless, their recognition is important since they may represent subtle brain injury and since similar structures in adults might easily be misinterpreted as true diffuse amyloid plaques.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Crain BJ,McPhatter L,Croom DW 2nd,Hulette CMdoi
10.1007/BF00294258subject
Has Abstractpub_date
1995-01-01 00:00:00pages
42-9issue
1eissn
0001-6322issn
1432-0533journal_volume
89pub_type
杂志文章abstract::A case of progressive polyneuropathy associated with Waldenström's macroglobulinaemia is reported. A monoclonal IgM-lambda gradient was detected in the serum and cerebro-spinal fluid. By electro-immunoblot analysis antibodies against myelin-associated glycoprotein were found in the serum and cerebro-spinal fluid. The ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690395
更新日期:1984-01-01 00:00:00
abstract::Defects of major histocompatibility complex (MHC) class I antigen-processing machinery (APM) components have been shown to contribute to immune escape of malignant cells. We investigated the expression of APM components in astrocytomas without detectable defects in HLA class I antigen expression and correlated it with...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-007-0231-8
更新日期:2007-08-01 00:00:00
abstract::Six subtypes of sporadic Creutzfeldt-Jakob disease with distinctive clinico-pathological features have been identified largely based on two types of the abnormal prion protein, PrP(Sc), and the methionine (M)/valine (V) polymorphic codon 129 of the prion protein. The existence of affected subjects showing mixed phenot...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-009-0585-1
更新日期:2009-11-01 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::Pilocytic astrocytomas are the most common astrocytic tumors of childhood and differ clinically and histopathologically from those astrocytomas that affect adults. Studies of adult astrocytic tumors have revealed allelic losses on chromosomes 10, 17p, 19q and alterations in the epidermal growth factor receptor (EGFR) ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00454903
更新日期:1993-01-01 00:00:00
abstract::We examined whether the Golgi apparatus (GA) is fragmented in nigral neurons in 18 cases with Parkinson's disease (PD) and in 8 control cases. The nigral neurons in cases with PD showed various degrees of Lewy pathology with alpha-synuclein immunohistochemistry, and we divided the neurons into three subtypes according...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-006-0114-4
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::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::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::We have obtained a cDNA fragment to human glial fibrillary acidic protein (GFAP) by immunoscreening a lambda gt11 human brain cDNA library with antibody to bovine GFAP. The highly homologous nucleotide sequence of this clone with that of the mouse GFAP enabled the identification of this cDNA as one encoding GFAP. As t...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687396
更新日期:1989-01-01 00:00:00
abstract::To determine the origin of the large myelinated fibers in the anterolateral funiculus (ALF) in the spinal cord of humans, myelinated fibers in the ALF of the mid-cervical spinal cord were examined quantitatively. Five groups of subjects were examined, consisting of control subjects, patients with cerebral lesions and ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051129
更新日期:1999-12-01 00:00:00
abstract::Experimental neuropathy, characterized by endoneurial edema and demyelination, was induced by inoculating rabbits with a combination of Freund's complete adjuvant (FCA), gangliosides, lecithin and cholesterol. A less severe demyelinating neuropathy could be induced by treatment with FCA alone but no significant change...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00695496
更新日期:1987-01-01 00:00:00
abstract::The original version of this article unfortunately contained a mistake. The Panel A in the published figure 5 is incorrect. The corrected Figure 5 is placed in the following page. ...
journal_title:Acta neuropathologica
pub_type: 已发布勘误
doi:10.1007/s00401-019-02092-y
更新日期:2020-01-01 00:00:00
abstract::Dysferlin is a newly identified sarcolemmal protein related to Miyoshi myopathy and limb-girdle muscular dystrophy. Although its function is still unknown, it is inferred from the presence of C2 domains and a transmembrane domain in its sequence that dysferlin may be expressed or located not only at the sarcolemma but...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0686-1
更新日期:2003-06-01 00:00:00
abstract::Seven patients aged 29 to 76 years with various clinical subtypes of chronic inflammatory demyelinating polyneuropathy (CIDP) were investigated. Sural nerve biopsies were performed between 7 months and 19 years after onset of disease. Quantitative electron microscopy revealed involvement of primary unmyelinated fibers...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00307700
更新日期:1990-01-01 00:00:00
abstract::Tenascins (TNs) are a family of extracellular matrix glycoproteins. The first member of this family to be recognized, tenascin-C (TN-C), is known to be expressed in various tumors including human astrocytomas. Tenascin-X (TN-X) is the latest member of the TN family to be reported, and its expression in tumor tissues h...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050636
更新日期:1997-05-01 00:00:00
abstract::A Schwann cell can form only one internode of myelin around an axon. However, we observed the formation by a single Schwann cell of myelin around two axons of different diameters in the sural nerve of a 45-year-old man with mononeuritis multiplex. Schwann cell processes spiraled in the same direction around each axon,...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00304478
更新日期:1992-01-01 00:00:00
abstract::A retrospective study of 450 consecutive AIDS autopsy cases (397 males, 53 females; mean age at death 38.4 years) in Vienna, Austria, between 1984 and 1999 compares the central nervous system (CNS) findings in three cohorts: 1984-1992 (190 cases), 1993-1995 (162 cases) and 1996-1999 (98 cases, after introduction of tr...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010000245
更新日期:2000-08-01 00:00:00
abstract::A 77-year-old man suffered intermittent hemiconvulsions of unknown etiology on the left side for a period of about 5 weeks. At the autopsy, there was marked neuronal loss, severe proliferation of astrocytes and spongiform changes in the right cerebral cortex. The cerebral white matter showed loosening with astroglial ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687782
更新日期:1988-01-01 00:00:00
abstract::A 40-year-old man suffering from amyotrophic lateral sclerosis with symmetrical degeneration of the thalamus and the substantia nigra is reported. The distribution pattern of the thalamic degeneration in the present case was characteristic in that the Nucleus centralis was the severest affected of the thalamic nuclei...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691074
更新日期:1978-12-15 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::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::This study reports the histological, ultrastructural, and immunocytochemical characteristics of intracytoplasmic eosinophilic inclusion bodies occurring in various types of neurons of the human central nervous system. By light microscopy, the inclusions were brightly eosinophilic, slightly birefringent, and sharply de...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00308960
更新日期:1989-01-01 00:00:00
abstract::A murine model of Tay-Sachs disease, the prototype of the GM2 gangliosidoses, was produced through the targeted disruption of the Hexa gene encoding the subunit of alpha-hexosaminidase A. The mice were completely devoid of beta-hexosaminidase A activity and accumulated GM2 ganglioside in the CNS in an age-dependent ma...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00309622
更新日期:1995-01-01 00:00:00
abstract::Two young zebra siblings from consecutive pregnancies suffered from neurological disease, characterized by ventral deviation of the neck and tetraparesis which progressed to tetraplegia within a few weeks. On histological examination widespread neuronal degeneration was observed in the ventral horns of the spinal cord...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00684532
更新日期:1977-01-31 00:00:00
abstract::The finding of novel fibroblastic onion bulb-like structures in peripheral nerves is reported for the first time in avian riboflavin deficiency. Day old broiler meat chickens were fed a riboflavin deficient diet (1.8 mg/kg) and were killed on postnatal days 6, 11, 16, 21 and 31, whereas control chickens were fed a con...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-007-0215-8
更新日期:2007-08-01 00:00:00
abstract::Missense mutations of the V600E type constitute the vast majority of tumor-associated somatic alterations in the v-RAF murine sarcoma viral oncogene homolog B1 (BRAF) gene. Initially described in melanoma, colon and papillary thyroid carcinoma, these alterations have also been observed in primary nervous system tumors...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-011-0802-6
更新日期:2011-03-01 00:00:00
abstract::It was earlier shown that bicuculline-induced status epilepticus gives rise to profound acute changes in the rat cerebral cortex, i.e. edema and neuronal alterations. In the present study, we explored to what extent interruption of the seizure activity reverses the changes observed. To that end, status epilepticus of ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00684924
更新日期:1983-01-01 00:00:00
abstract::Alpha-synuclein is known to play an important role in several neurodegenerative diseases. Moreover, it is expressed in central nervous system neuronal tumors, and another member of the synuclein family, gamma-synuclein, is overexpressed in breast and ovarian carcinomas. However, the expression of alpha-synuclein has n...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010100443
更新日期:2002-02-01 00:00:00
abstract::The fine structures of two pineal parenchymal neoplasms has been described. The tumors contained a predominance of small, poorly differentiated cells with prominent nuclei and scanty cytoplasm. These cells were similar to those found in medulloblastomas, primitive cerebral neuroectodermal tumors, and fetal cerebellum ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00699239
更新日期:1981-01-01 00:00:00