Abstract:
:Histopathological, immunocytochemical, and electron microscopical investigations were carried out in a man with a protracted history of spastic paraparesis, adrenal insufficiency and hypogonadism. Pathological findings were identical with those of the few previously reported cases of adrenomyeloneuropathy (AMN) including cytoplasmic lamellar inclusions consisting of two parallel 2.5 nm leaflets separated by a clear space of variable extent, in the brain, spinal roots, adrenal gland, and interstitial cells of the testis. No inclusions could be found in oligodendrocytes. In brain macrophages they are thought to represent breakdown products of pathological myelin stored in lysosomes, whereas in other localizations they might be an expression of the primary metabolic defect of the cell. Special attention was paid to the pseudosystematic type of fiber tract degeneration in the spinal cord. The dying-back pattern of axonal destruction was interpreted as a possible result of the multisegmental demyelination observed in these tracts. All known hormones could be localized in the pituitary by immunocytochemistry. Corticotrophs and gonadotrophs were numerous. The structural damage of the adrenal cortex and the interstitial cells of the testis is, therefore, considered to result from the inborn error of metabolism on the one hand and from an enhanced stimulation exerted by ACTH and gonadotrophins on the other.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Probst A,Ulrich J,Heitz PU,Herschkowitz Ndoi
10.1007/BF00690750subject
Has Abstractpub_date
1980-01-01 00:00:00pages
105-15issue
2eissn
0001-6322issn
1432-0533journal_volume
49pub_type
杂志文章abstract::A series of 50 human primary intracranial tumors were cultivated in vitro in an attempt to establish cell lines with the trypsinization technique. During the in vitro adaptation period, cultures were maintained at high cell density to avoid rapid over-growth by connective tissue. Five lines were established from 5 tum...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690383
更新日期:1977-07-15 00:00:00
abstract::Although clinical and electrophysiological evidence indicates that the amygdaloid body plays an important role in the pathogenesis of temporal lobe epilepsy, there are very few detailed data on histopathological changes in this nucleus in epilepsy patients. In the present study we have examined the lateral nucleus of ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050658
更新日期:1997-06-01 00:00:00
abstract::The present study investigated the expression of c-erbB-2 in 59 meningiomas, including different histological subtypes and anaplastic variants, by immunocytochemistry and molecular biological techniques. Immunohistochemistry using the monoclonal antibody FWP-51 directed against c-erbB-2-encoded oncoprotein gp185 demon...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00228582
更新日期:1993-01-01 00:00:00
abstract::It has been suggested that delayed development in either the peripheral or central nervous system could underlie the sudden infant death syndrome (SIDS). We studied the phrenic nerve in an attempt to find if maturation in this nerve was delayed in SIDS, and to see if fiber size differences could explain the paucity of...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050445
更新日期:1996-01-01 00:00:00
abstract::The pattern of recovery of myelinated axons in the posterior tibial nerve after crushing was studied in rats chronically intoxicated with 2,5-hexanedione. It was given for 2 weeks before crushing (200 mg/kg i.p. 5 times a week) or additionally for two further weeks after the nerve crush. Two animals were examined from...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00703206
更新日期:1983-01-01 00:00:00
abstract::A 17 year-old male, who was mentally and bodily retarded from the age of 7 years. At the age of 16 years, several neurological signs appeared. Pathologically, there was widespread myelin loss and tissue sponginess in the white matter of the cerebrum and cerebellum, bilaterally and symmetrically. A great many Alzheimer...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:
更新日期:1976-01-01 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 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::Adult rabbits were subjected to 4.5 Gy of whole-blody or brain alone gamma-irradiation, and their hippocampus was examined with the light and electron microscope. Pycnotic cells were found at the base of the granular layer of the dentate gyrus in the so-called subgranular zone, as soon as 3 h after irradiation, and we...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690520
更新日期:1979-12-01 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::Tumours of the neuroglia, 172 in all (50 glioblastomas, 65 fibrillar and gemistocytic astrocytomas, 26 pilocytic astrocytomas and 31 oligodendrogliomas), were studied by automated microscopic picture analysis. Thirteen morphometric and densitometric parameters of the tumour cell nuclei as well as two mitotic parameter...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00692692
更新日期:1982-01-01 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::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::A man of 75 years of age had had lightning pains in the legs for 8 years. Clinical examination demonstrated mild pyramidal signs and involvement of the posterior columns. A morphine pump was placed in the epidural space in the lumbar region to treat the pain. He died two weeks later from a massive pulmonary embolism. ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00695497
更新日期:1987-01-01 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::Alzheimer's disease (AD) is a major cause of dementia. Characteristic neuropathological features of AD include neurofibrillary tangles, senile plaques, amyloid angiopathy and microvascular atrophy. The ultra-structure of the microvascular atrophy in AD and its pathogenetic significance have not been defined. This repo...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050386
更新日期:1996-01-01 00:00:00
abstract::In spite of several cases reported in the literature, the exact pathogenetic mechanism of neuropathic changes in porphyric neuropathy remains uncertain. Various authors have ascribed the neuropathologic findings to either a dying-back axonal degeneration or segmental demyelination. In recent years, the hypothesis of a...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00697191
更新日期:1984-01-01 00:00:00
abstract::Olfactory dysfunction is a frequent and early feature of patients with neurodegenerative disorders such as Alzheimer's disease (AD) and Parkinson's disease (PD) and is very uncommon in patients with frontotemporal dementia (FTD). Mechanisms underlying this clinical manifestation are poorly understood but the premature...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-011-0830-2
更新日期:2011-07-01 00:00:00
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::We have previously characterized three human leucine-rich repeats and immunoglobulin-like domains (LRIG) genes and proteins, named LRIG1-3 and proposed that they may act as suppressors of tumor growth. The LRIG1 transmembrane protein antagonizes the activity of epidermal growth factor receptor family receptor tyrosine...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-006-0032-5
更新日期:2006-03-01 00:00:00
abstract::Aberrant peripheral nerve fiber bundles, i.e., masses of peripheral nerve fibers without perineural sheath cells, are found in the perivascular spaces of some spinal cords. The 514 spinal cords examined for these structures were separated into two groups; 507 spinal cords without definite necrotic lesions (group 1) an...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00308951
更新日期:1989-01-01 00:00:00
abstract::This report deals with the expression of stress-response (heat-shock) protein 72 (srp 72) in a series of 95 primary human brain tumors and 21 carcinoma metastases to the central nervous system (CNS). Immunohistochemical procedures were employed; cells of the human cervical cancer line HeLa S3 were used as positive con...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00227818
更新日期:1992-01-01 00:00:00
abstract::Gerstmann-Sträussler-Scheinker disease (GSS) is a hereditary transmissible spongiform encephalopathy associated with prion protein gene mutation P102L. The age of onset is roughly restricted to around the sixth decade; however, it is unclear whether the disease-specific pathology of GSS is already evident in the pre-c...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0697-y
更新日期:2003-07-01 00:00:00
abstract::A 65-year-old woman was admitted to our hospital for forgetfulness, depression and eccentric behavior that had been first noticed 2 years prior to admission. She showed memory impairment, perseveration and repeated violent actions, but no limb-kinetic apraxia. She died 12 years after the onset of symptoms. At autopsy,...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010100460
更新日期:2002-03-01 00:00:00
abstract::SNAP-25 (a synaptosomal-associated protein of 25 kDa) has been shown to be involved both in synaptic vesicle exocytosis and in axonal outgrowth. In the present study, we investigated the changes in SNAP-25 immunoreactivity in the hippocampus of the Mongolian gerbil (Meriones unguiculatus) at different time points afte...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050795
更新日期:1998-03-01 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::Knockout of genes encoding metabotropic glutamate receptor 5 (mGluR5) or its endogenous regulators, such as Norbin, induce a schizophrenia-like phenotype in rodents, suggesting dysregulation of mGluR5 in schizophrenia. Human genetic and pharmacological animal studies support this hypothesis, but no studies have explor...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-015-1411-6
更新日期:2015-07-01 00:00:00
abstract::The ultrastructure of the curly fibers was examined by the transmission and immunoelectron microscopy as well as by the rapid-freeze, deep-etch and replica method. The curly fibers consisted mainly of paired helical filaments (PHF) in the neuropils, both pre- and post-synaptic. On the deep-etch replicas, PHF in the ne...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00334498
更新日期:1990-01-01 00:00:00
abstract::Frontotemporal lobar degeneration with TAR DNA-binding protein 43 inclusions (FTLD-TDP) is the most common pathology associated with frontotemporal dementia (FTD). Repeat expansions in chromosome 9 open reading frame 72 (C9ORF72) and mutations in progranulin (GRN) are the major known genetic causes of FTLD-TDP; howeve...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-015-1436-x
更新日期:2015-07-01 00:00:00
abstract::Ectopic neurons have been detected in the hippocampus of postnatal hooded rats aged 5-24 days. These rats were exposed to methylazoxymethanol acetate (MAMac) during foetal development by injecting the mother rats with this neurotoxin. At birth, the hippocampus of rats exposed to MAMac showed a normal cytoarchitecture;...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688698
更新日期:1977-10-10 00:00:00