Immunohistochemistry with anti-prion protein 27-30 gives reactions with fungi.
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Aspergillosis↗
Biomedical subjects
J Peiffer
Publications and source records attributed to J Peiffer.
Creutzfeldt-Jakob disease with amyloid angiopathy: diagnosis by immunological analyses and transmission experiments.
It was difficult to make a definite pathological diagnosis in a 73-year-old man with Creutzfeldt-Jakob disease (CJD) due to extensive amyloid angiopathy which lacked any severe spongiform changes. Immunostaining using anti-prion protein (PrP) antibody revealed fine granular deposits in the gray matter, after hydrolytic autoclaving pretreatment on tissue sections. Western blotting also revealed an abnormal isoform of PrP, but PrP gene analysis did not show any abnormalities. The primary transmission experiments were repeated three times and induced spongiform encephalopathy in a few mice after a long incubation period.
Aged↗
Periventricular neurocytoma: a pathological entity.
Three cases of periventricular neurocytomas are presented. All patients had a large but well-circumscribed, hyperdense tumor with insignificant contrast enhancement in the lateral and third ventricle, causing hydrocephalus. Calcification was present in one patient. Angiography revealed a blush tumor enhancement in two cases. Surgical removal was complete in two patients and incomplete in one. Light microscopy showed a cell pattern that resembled either ependymoma or oligodendroglioma. However, in all cases the tumor was confirmed to be a neurocytoma by immunocytochemical analysis that showed reactivity for synaptophysin and/or neuron-specific enolase in a high percentage of neoplastic cells. With respect to the literature it is concluded that neurocytomas represent an individual pathological entity of supratentorial midline tumors. Complete surgical removal without irradiation is the recommended treatment.
Adult↗
Neuropathology in the Third Reich. [Memorial to those victims of National-Socialist atrocities in Germany who were used by medical science].
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Adolescent↗
Immunohistochemical demonstration of mitochondria in routinely processed tissue using a monoclonal antibody.
We report results obtained using the monoclonal antibody M-II 68, which recognizes inner mitochondrial membrane in routinely processed (formalin-fixed and paraffin-embedded) tissue by light microscopic immunohistochemistry. In ten normal brains, the range of immunoreactivity in various cell types and locations was defined. The most intense staining was observed in Purkinje cells, in neurons of cranial nerve nuclei, pons and substantia nigra, as well as in choroid plexus epithelial cells. By comparison with this control group, one case of primary mitochondrial encephalomyopathy exhibited increased staining of endothelial and vascular smooth muscle cells, choroid plexus epithelial cells, and neurons of various locations. Scattered ragged-red fibres were heavily labelled in one case of mitochondrial myopathy, while ten muscles without mitochondriopathy were left unstained. Our method is able to detect accumulations of mitochondria and increases in mitochondrial cristae density. It could prove useful for differential diagnosis of routine biopsy material and for clarification of cell types involved in mitochondrial cytopathies.
Adolescent↗
Intracerebral distribution of mitochondrial abnormalities in 21 cases of infantile spongy dystrophy.
Using a monoclonal antibody to an inner mitochondrial membrane antigen and light microscopic immunohistochemistry, we investigated the distribution of increased immunostaining (mitochondrial anomalies, MA) on paraffin sections from 21 brains with infantile spongy dystrophy (Leigh's disease, 8; Canavan's disease, 4; Alpers' syndrome, 2; mixed spongy dystrophy, 7). Compared with an age-matched control group, MA were present in all cases of Leigh's disease (leptomeningeal and intracerebral endothelial and vascular smooth muscle cells, choroid plexus epithelia, ependymal cells, astrocytes or some neurons), in 2 cases of Canavan's disease and the Alpers' syndrome cases (astrocytes and occasionally some neurons). The MA were restricted to spongy areas in Canavan's disease and Alpers' syndrome, whereas they were distributed throughout the brain in Leigh's disease. In mixed spongy dystrophies the Leigh histology was associated with MA, but not the Canavan histology. Brains with Wernicke's encephalopathy (3 cases), adult infarction (3), and multicystic encephalopathy (5) showed no MA, but one with methylmalonaciduria did. Our results substantiate the classification of Leigh's disease as primary mitochondrial encephalopathy.
Adolescent↗
Immunohistologic analysis of neuron-specific enolase and neuropeptides in stromal cells of cerebellar hemangioblastomas.
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Antibodies↗
Intratumoral histologic heterogeneity of gliomas. A quantitative study.
Quantitative data for intratumoral histologic heterogeneity were obtained by investigating ten small and ten large punched samples from 50 unembedded supratentorial gliomas. The 1000 samples were diagnosed according to the World Health Organization (WHO) classification and six histopathologic features associated with malignancy were evaluated (cellular density, nuclear pleomorphism, necroses, histologic architecture, vessels, and mitoses), each with defined gradations. The slides were read independently by two observers. The initially high interobserver variability (grade, 22.2%; type, 10.3%; and tumor presence/absence, 7.1%) was for the most part due to intermediate grades and types and was reduced to 1.7% after mutual review. Small samples showed lower mean grade than large samples and more often absence of tumor (7.6% versus 2.4%). Of all gliomas, 48% showed differently typed samples, 82% differently graded samples, and 62% benign and malignant grades. Intratumoral heterogeneity was higher for the necroses than for the other histopathologic features. Our results underscore the importance of extensive tissue sampling.
Brain Neoplasms↗
Ragged-red fibres detected in paraffin sections by a monoclonal antibody to inner mitochondrial membrane.
An immunohistochemical method is reported using the M-II68 monoclonal antibody, which detects mitochondrial accumulations ("ragged-red fibres") in routinely processed (formalin-fixed, paraffin-embedded) muscle tissue. Ten cases with electron-microscopically and histochemically proven mitochondrial myopathy featured 4% to 24% ragged-red fibres. In a series of 50 muscle biopsies without mitochondrial myopathy, scattered ragged-red fibres (less than 0.1%) were present in a few normal and pathological muscles. The immunohistochemical method is specific for mitochondria, does not require frozen tissue and permits rapid examination of large areas.
Antibodies, Monoclonal↗
Intracerebral malignant fibrous histiocytoma at site of a previously excised low grade glioma.
The case of a 42-year-old patient is reported who developed an intracerebral malignant fibrous histiocytoma at the site of an oligoastrocytic mixed glioma which had been excised 2 1/2 years previously. Reasons for the extreme rarity of intracranial malignant fibrous histiocytomas, the probability of a traumatic aetiology of this particular tumour, and the possible significance of intratumoural cells positive for glial fibrillary acidic protein (GFAP) are discussed.
Adult↗
Meningio-angiomatosis.
A case is described of a boy five years old who suffered from left-sided muscular weakness since the first months of life and from absences since the second year of life. He died of valproate-induced hepatic insufficiency. Autopsy of the brain revealed meningio-angiomatosis, a rare but rather benign disorder usually characterized by narrow meningothelial proliferations abutting upon cortical plaques and exhibiting proliferations of small vessels with perivascular cuffs of fibroblast-like cells. The peculiarities were that the case lacked any leptomeningeal calcification - in line with the patient's age being the lowest so far reported for pathologically verified meningio-angiomatosis - and also exhibited intracortical clusters of mesenchymal cells that did not form vessels ("free fibroblasts"). Immunohistochemically perivascular cells were negative for S100, GFAP, desmin and factor-8-related antigen and were embedded in interstitial collagen of types III and VI as well as procollagen I, while "free fibroblasts" were surrounded by deposits of basement membrane collagen type IV. The results are consistent with a meningothelial origin of perivascular cells and "free fibroblasts".
Angiomatosis↗
[Acetylcholinesterase negativity in the lamina propria in the first 8 weeks of life does not exclude Hirschsprung disease].
In a total of 34 children with Hirschsprung's disease, 7 cases showed at first biopsy a normal lamina propria mucosae without aberrant acetylcholine esterase (ACHE)-positive fibres. In further biopsies all 7 cases showed the typical pattern of abundant ACHE-positive nerve fibres in the lamina propria, the last one at the age of 8 weeks. This data suggests that in children with Hirschsprung's disease who have not yet attained their 8th week of life, approximately one-third are without the typical aberrant nerves in the lamina propria. Therefore, we emphasize that a biopsy taken before the 8th week of life showing a negative morphological result should be repeated if the clinical suspicion remains. The late sprouting of the pathological nerves should be considered especially if there are abundant ACHE-positive fibres in the lamina muscularis mucosae.
Acetylcholinesterase↗
Adult-onset rod disease with abundant intranuclear rods.
The third case of adult-onset rod disease (nemaline myopathy) with abundant myofibrillar as well as intranuclear rods is described. The 61-year-old woman suffered from progressive weakness of proximal extremities and of the neck, mimicking polymyositis. Muscle biopsy revealed a striking myopathic pattern, with intranuclear rods occurring in 31% of the fibres. On light and electron microscopy and by immunohistochemical study, the rods differed from myofibrillar rods. The absence of alpha-actinin in intranuclear rods suggests an enhanced readiness of actin filaments to bind to diverse proteins, instead of overproduction of alpha-actinin as the pathogenetic basis of the rod formation.
Actinin↗
Intrauterine development of posthaemorrhagic hydrocephalus.
Differential diagnosis of intrauterine hydrocephalus includes various types of cerebral malformations. In about 30% of cases the pathogenesis remains unknown. During 1983-1985 we diagnosed intrauterine posthaemorrhagic hydrocephalus (IPHH) in four newborns. In three of the reported cases intraventricular haemorrhage (IVH) could already be proven in the fetus. Due to these observations, IPHH has to be considered with the differential diagnosis of congenital hydrocephalus. Haemorrhagic diathesis and multiple pregnancy are discussed as possible risk factors for this severe fetal complication.
Adult↗
Does the pleomorphic xanthoastrocytoma exist? Problems in the application of immunological techniques to the classification of brain tumors.
A case of a fibrous xanthomatous tumor of the meninges is reported. This is a rare tumor of childhood in which the characteristic pleomorphic histology contrasts with the good clinical prognosis. These tumors were reclassified as pleomorphic xanthoastrocytomas (PXA) due to their glial fibrillary acidic protein (GFAP) positivity. In the present tumor, GFAP was absent from nearly all cell bodies in most of the leptomeningeal regions of the tumor but could be detected with greater frequency at the cortical-leptomeningeal border zones and in the areas in which the tumor had infiltrated the cortex. All the tumor cells expressed vimentin and in, addition, most expressed alpha-1-antitrypsin, alpha-1-antichymotrypsin, tartrate-resistant acid phosphatase, common leukocyte antigen, and OKM1. This spectrum of marker staining corresponded not only to the pattern observed in two cutaneous fibrous histiocytomas and one malignant fibrous histiocytoma, but also to the results previously published in the literature with regard to fibrohistiocytic tumors. By contrast, this spectrum of monocytic-histiocytic marker staining was not seen in gliomas. We, therefore, regard the PXA as a mesenchymal tumor of the meninges, identical to benign fibrous histiocytomas elsewhere in the body. The possible reasons why this mesenchymal tumor can show GFAP positivity in the leptomeningeal border zone are discussed.
Astrocytoma↗
Encephalomyelitis and demyelinating diseases in patients with extracerebral malignant tumors.
We examined central nervous system (CNS) lesions in 456 patients with primary extracerebral malignant tumors. Inflammatory reactions caused by viral (progressive multifocal leukoencephalopathy (PML), herpes zoster varicella), fungal, or bacterial infections could be demonstrated in 20 patients. In a further 19 patients, the brain tissue showed lymphocyte infiltrates of unknown etiology and, in four of these, autopsy revealed probable paraneoplastic, non-bacterial, endocarditis as a possible explanation for the local inflammatory reaction. The frequency of thrombophlebitis, non-arteriosclerotic thrombosis and arteritis was significantly higher than in a control group of 2052 tumor-free patients. Focal spongiform-axonopathic lesions (24 cases) as well as diffuse leukoencephalopathy (11 cases) were interpreted as probably being at least in part paraneoplastic because the same alterations could also be observed in patients who had never undergone cytostatic or radiation therapy. The possible pathogenetic conditions are discussed and a classification of these tumor-accompanying, but not always tumor-dependent, lesions suggested.
Brain↗
Mitochondrial myopathies with necrotizing encephalopathy of the Leigh type.
Two patients with mitochondrial encephalomyopathy (MEP) serve to emphasize the variability of this group of diseases. Cerebral insults, mitochondrial cardiopathy, relapsing ileus, cerebral angioma, ataxia, and myoclonic seizures characterized the first case of an adult man with similar diseases in his family, interpreted as transitional form between mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) and myoclonus epilepsy associated with ragged red fibers (MERRF). The second patient, a floppy infant with cardiomyopathy and myoclonism, statomotoric and mental retardation showed combined defects in mitochondrial respiratory chain at NADH-CoQ reductase and cytochrome c oxidase and a deficiency of carnitine. In both patients neuropathologically criteria of Leigh's syndrome could be demonstrated in the cerebral cortex, in case 2 also clinically. The classificatory problems of the relationships between KSS, MELAS, MERRF, Leigh's as well as Alpers' syndromes are discussed.
Adult↗
[Anesthesia for eye operations in mitochondrial encephalomyelopathy].
Mitochondrial encephalomyopathy involves a disturbance of the mitochondrial respiratory chain, as a result of which the blood lactate level is elevated. In stress situations a lactate acidosis can occur. The disease may be subdivided into three main syndromes: Kearns-Sayre syndrome (KKS), "myoclonus epilepsy with ragged red fibers syndrome" (MERRF), and "mitochondrial myopathy, encephalopathy, lactic acidosis and strokelike episodes syndrome" (MELAS). There are also several intermediate forms. Ophthalmological symptoms are frequent and occasionally have to be treated surgically. A 20-year-old male patient with a mixed form of these syndromes including elements of KSS and MERRF had to undergo cataract extraction. The authors decided to perform the operation under local anesthesia and sedation, with the anesthetist on standby. No problems arose. In all cases where mitochondrial encephalomyopathy is suspected the diagnosis should be confirmed by a muscle biopsy and the risk of cardiac arrest, respiratory insufficiency, and epileptic seizures ruled out prior to surgery. Local anesthesia with sedation appears to be the most favorable form of anesthesia provided the maximum dose is observed and a substance with a high convulsion threshold is chosen. Perioperative monitoring by an anesthetist and temporary provision of a cardiac pacemaker are necessary.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗