Abstract:
:The case of a child is described who at the age of 2 years showed the first evidence of a developing neurological disease. Within a couple of years, profound mental retardation and severe motor deficit with spastic tetraplegia became established. No seizures and no pigmentation of the retina were observed. The condition remained practically unchanged for some 8 years and the patient died at 12 years of age of terminal bronchopneumonia. At autopsy there was conspicuous diffuse atrophy of the brain. The cerebral cortex was particularly involved. Most of the cortical neurons were destroyed and neuroglia showed abundant proliferation. The few remaining neurons contained inclusion material which was identified as lipofuscin. Noticeable cedifferences from the various types of amaurotic idiocies are noted and similarities to a case of lipidosis recently reported from Finland are suggested.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Jervis GA,Donahue Sdoi
10.1007/BF00688145subject
Has Abstractpub_date
1975-01-01 00:00:00pages
109-16issue
2eissn
0001-6322issn
1432-0533journal_volume
31pub_type
杂志文章abstract::Altered levels of cerebrospinal fluid (CSF) peptides related to Alzheimer's disease (AD) are associated with pathologic AD diagnosis, although cognitively normal subjects can also have abnormal levels of these AD biomarkers. To identify novel CSF biomarkers that distinguish pathologically confirmed AD from cognitively...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-010-0667-0
更新日期:2010-06-01 00:00:00
abstract::A case of cerebellar medullomyoblastoma in a young boy was investigated by electron microscopy. The neuroectodermal component shows the characteristics of a desmoplastic medulloblastoma. The mesodermal component consists of more or less differentiated cross-striated muscle cells. Undifferentiated muscle cells are very...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690521
更新日期:1979-12-01 00:00:00
abstract::An autopsy case of Nasu-Hakola's disease (membranous lipodystrophy) was reported. A 29-year-old Japanese woman whose younger sister had been affected with typical Nasu-Hakola's disease with skeletal and neuropsychiatric syndromes and membrano-cystic lesions in the bones developed forgetfulness and lack of initiative. ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00689400
更新日期:1981-01-01 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::Laminin alpha2 (merosin)-deficient congenital muscular dystrophy (CMD) patients show progressive muscle fiber necrosis and ineffective muscle regeneration. This is probably due to decreased formation of multi nucleated myotubes resulting from a myoblast fusion defect. When receiving a mechanical signal from muscle mem...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-004-0884-5
更新日期:2004-09-01 00:00:00
abstract::Silver-staining methods are helpful for histological identification of pathological deposits. In spite of some ambiguities regarding their mechanism and interpretation, they are widely used for histopathological diagnosis. In this review, four major silver-staining methods, modified Bielschowsky, Bodian, Gallyas (GAL)...
journal_title:Acta neuropathologica
pub_type: 历史文章,杂志文章,评审
doi:10.1007/s00401-007-0200-2
更新日期:2007-05-01 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::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::Numbers of dystrophic neurites, seen with the electron microscope, in CA1 of the hippocampus of either C3H mice infected with 22C or 79A strains of scrapie, or LM mice infected with strain ME7 were greater than in age-matched control mice. Vacuolation, seen by light microscopy in CA1 of the hippocampus of mice infecte...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688263
更新日期:1987-01-01 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::Accumulation of amyloid-β (Aβ) and neurofibrillary tangles in the brain, inflammation and synaptic and neuronal loss are some of the major neuropathological hallmarks of Alzheimer's disease (AD). While genetic mutations in amyloid precursor protein and presenilin-1 and -2 (PS1 and PS2) genes cause early-onset familial...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-012-1065-6
更新日期:2013-02-01 00:00:00
abstract::Cryostat sections of 12 gliomas and of 3 peritumoral brain tissue samples were investigated for mononuclear cell infiltration by immunohistochemistry, concentrating on cells expressing monocyte/macrophage markers. Only low numbers of T cells were detected in the tumors, whereas in average 20%-30% of all cells present ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294647
更新日期:1990-01-01 00:00:00
abstract::Two cases out of a sample of 41 fetuses and infants with prosencephalic malformation, observed at the Institute of Pathology and Department of Neuropathology of the University of Heidelberg, are described here in detail. These cases presented grossly with microcephaly and missing forebrain, appearing to be cases of ap...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/pl00007438
更新日期:2000-03-01 00:00:00
abstract::Focal cortical dysplasias (FCDs) are increasingly recognized as one of the most common causes of pharmaco-resistant epilepsies. FCDs were recently divided into various clinico-pathological subtypes due to distinct imaging, electrophysiological, and outcome characteristics. In this review, we will overview the internat...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-014-1304-0
更新日期:2014-07-01 00:00:00
abstract::Sporadic Creutzfeldt-Jakob disease (sCJD) is a transmissible brain proteinopathy. Five main clinicopathological subtypes (sCJD-MM(V)1, -MM(V)2C, -MV2K, -VV1, and -VV2) are currently distinguished. Histopathological evidence suggests that the localisation of prion aggregates and spongiform lesions varies among subtypes...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-020-02168-0
更新日期:2020-08-01 00:00:00
abstract::The immunohistochemical distribution of alpha and beta subunits of S-100 protein (S-100 alpha, S-100 beta, respectively) in 138 cases of human brain tumors was investigated by the avidin-biotin immunoperoxidase method. Brain tumors can be divided into four groups: group 1 [S-100 alpha (+) and/or S-100 beta (+)]; astro...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00296376
更新日期:1991-01-01 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::The neuropathologic findings in the brain of a National Aeronautics and Space Administration (NASA) engineer, who died subsequent to nitrogen inhalation, are reported. Findings which are unusual in other forms of anoxia included relative preservation of neurons in the Sommer sector of the hippocampus and a pattern of ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688632
更新日期:1985-01-01 00:00:00
abstract::We have found large eosinophilic bodies in the pericapillary regions of the gray and white matter in the human spinal cord. These are entirely different from the previously reported pericapillary inclusion bodies (PIB). We have designated them pericapillary rosettes (PR), since they consist of clusters of round or ovo...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00299408
更新日期:1992-01-01 00:00:00
abstract::A case of 14-year-old girl is reported in whom an alveolar rhabdomyosarcoma occurred in the soft tissues of the left forearm 4 years prior to death. Despite extensive surgery as well as chemotherapy and radiotherapy the tumor recurred locally and produced extensive metastases including a metastasis to the brain. Cereb...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687824
更新日期:1985-01-01 00:00:00
abstract::We report a sporadic tauopathy of 6-year duration in a 76-year-old woman. Her initial symptoms were asymmetrical parkinsonism and muscle weakness, with apraxia appearing 2 years later. The brain showed frontal and temporal cerebral atrophy; severe neuronal loss and gliosis were observed in the precentral cortex (loss ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-005-1086-5
更新日期:2005-12-01 00:00:00
abstract::A strong immunoreactivity for ferritin was observed in the neuritic (senile) plaques in Alzheimer's disease hippocampus. The ferritin accumulation was almost exclusively associated with the microglia, which appeared to have proliferated greatly. These cells were also positive for HLA-DR, a putative marker for reactive...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00334497
更新日期:1990-01-01 00:00:00
abstract::Excitotoxic stimulation of NMDA receptors results in the activation of a variety of cellular responses. The inducible transcription factor NF-kappaB is known to be involved in excitotoxic responses by neurons. Here, we show that NF-kappaB activation occurs in a biphasic manner in hippocampal slices following a 20-min ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-004-0876-5
更新日期:2004-09-01 00:00:00
abstract::The distribution of apolipoprotein E (ApoE) was studied in the brain tissue of cases of the amyotrophic lateral sclerosis-parkinsonism-dementia complex of Guam, locally known as lytico bodig disease (LB), and compared with cases of Alzheimer's disease (AD) and normal brain tissue. In both LB and AD, strong ApoE immuno...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050533
更新日期:1996-10-01 00:00:00
abstract::Using immunohistochemical methods optimized to detect herpes simplex virus type 2 (HSV-2) antigen, paraffin sections from human central nervous system tissues from 31 cases pathologically diagnosed as multiple sclerosis (MS), 34 cases of other neurological diseases, 4 adult cases of HSV encephalitis, and mouse brains ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00686969
更新日期:1988-01-01 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::Histochemical and electron microscopic studies were performed in an attempt to clarify the muscle pathology in an 18-year-old man with Fabry disease, showing proximal limb muscle atrophy, and his 52-year-old mother, who is a Fabry carrier with hypertrophic cardiomyopathy. Despite the relatively mild myopathic changes ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00296520
更新日期:1995-01-01 00:00:00
abstract::Psammoma bodies at the perivascular area in five cases of meningioma were examined with the electron microscope. In general, meningocytic cells invest the outer aspect of blood vessels, which are constituted by multilayered basal laminae, collagen fibers, microfibrils, and pericytes. Remnants of degenerated cells are ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688693
更新日期:1985-01-01 00:00:00
abstract::The kidney of a 7-month-old male Cairn terrier with globoid cell leukodystrophy (GLD) was investigated with light and electron microscopes. A few tubular epithelial cells in the inner medulla as well as some exfoliated cells in the lumina revealed PAS-positive cytoplasm in which needle-like structures were to be seen ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687036
更新日期:1986-01-01 00:00:00
abstract::The accumulation of calcium in the hippocampal and thalamic vascular endothelium and the perivascular space was detected histochemically by means of the pyroantimonate technique 30, 60 and 120 min after systemic kainic acid administration. An increased number of calcium pyroantimonate deposits was found in the endothe...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00685971
更新日期:1986-01-01 00:00:00