Biomedical subjects

J Peiffer

Publications and source records attributed to J Peiffer.

At least 37 records · Page 2Linked to original sources

PubMed1989

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↗

PubMed1989

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↗

PubMed1989

[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↗

PubMed1988

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↗

PubMed1988

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↗

PubMed1988

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↗

PubMed1988

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↗

PubMed1988

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↗

PubMed1988

[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↗

PubMed1987

Adrenoleukodystrophy in an adult female. A clinical, morphological, and neurochemical study.

A 43-year-old female with adrenoleukodystrophy (ALD) is described, who developed spastic tetraparesis, suffered grand mal seizures, and became stuporous and demented during the last 5 years of her life. Computed tomography revealed symmetrical hypodense lesions in the peritrigonal regions. Adrenal insufficiency was not evident except for skin pigmentation. The ultrastructure of a rectal biopsy specimen showed inclusions with lamellae and interspersed clefts in macrophages of the submucosal layer. At autopsy, the adrenals were found to contain large foam cells filled with similar inclusions. The brain cortex and the spinal cord were histologically normal. However, cerebral white matter exhibited widespread demyelination which spared only the arcuate fibres. In regions of less severe demyelination scattered inflammatory cells were seen. On electron microscopy, aggregates of typical paired leaflets with distinct intermediate lines were demonstrated in perivascular macrophages. Histochemical study showed these cells to contain free as well as esterified cholesterol. Gas chromatographic analysis of very long chain fatty acids (VLFA) from the demyelinated cerebral white matter showed a marked increase of C26:0 fatty acid in cholesterol esters and above-normal values for C24:0 and C24:1 in gangliosides. It is suggested that the condition was a heterozygote form of X-linked ALD. Patients with neurodegenerative symptoms with or without adrenal insufficiency can easily be screened for X-linked ALD by VLFA analysis in blood or cultured fibroblasts.

Adrenal Glands↗

PubMed1987

Proliferative potential of meningiomas determined with the monoclonal antibody Ki-67.

In 30 meningiomas we investigated the proliferation rate of various subtypes with the monoclonal antibody Ki-67. Frozen sections were incubated with Ki-67 antibody using a modified Alkaline Phosphatase anti-Alkaline Phosphatase (APAAP)-technique and evaluation of proliferation rate was done by cell counting. Meningiomas of the meningiotheliomatous, fibrous and angioblastic subtype without atypical histological findings contained 1% or less proliferating cells. In recurrent tumors, in transitional and in anaplastic meningiomas there is a marked increase of proliferating cells up to 20%. The distribution of marked cells varies in recurrent tumors and anaplastic meningiomas, and a focal proliferation of tumor cells was seen in meningiomas from transitional type. Immunohistological labelling of proliferating cells in meningiomas may allow a more precise prediction of the proliferation potential of each meningioma.

Alkaline Phosphatase↗

PubMed1987

Additional biochemical criteria in the differential diagnosis of myositis.

Thirty-six biopsy specimens of human biceps and vastus lateralis muscles were examined by histometric analysis and determination of enzyme activities (phosphorylase, triosephosphate dehydrogenase, 3-hydroxacyl-CoA-dehydrogenase, lactate dehydrogenase, hexose isomerase, citrate synthetase, 6-phosphogluconate dehydrogenase). The series included 13 specimens from patients suffering from a benign form of muscular dystrophy (limb girdle and Becker type of muscular dystrophy) and 12 specimens from patients with an acute (n = 5) or chronic (n = 7) form of myositis. Muscle fibres were atrophic in myositis and hypertrophic (with an increased variation of fibre diameters) in muscular dystrophies, as has been shown previously. When myositis samples were compared with either normal or dystrophic muscles, a highly significant lowering of glycolytic enzyme activity was found in chronic myositis, while the activity of 6-phosphogluconate dehydrogenase was elevated to highly significant levels. Measurements of the latter enzyme's activity might be of additional value in differentiating chronic forms of myositis from benign muscular dystrophies.

Adolescent↗

PubMed1987

Classification of myositis. Correlations between morphological and clinical classifications of inflammatory muscle disease.

Insufficient clinical data given to pathologists often hamper the differentiation of inflammatory muscle diseases. Thus we proved the value of a classification based only upon morphological criteria. Among 160 biopsies with myositic pattern (4.9% of a series of 3264 muscle biopsies) we could distinguish the following groups: Interstitial inflammation with only few fibre necroses (17.6%), predominance of fibre necroses with few inflammatory infiltrates (38.1%), small vessel vasculitis (20.0%), arteritis (13.1%), granulomatous myositis (8.8%) and polymorphonuclear leukocytic infiltrates (2.6%). This classification is compared with the used clinical diagnoses. Some special morphological aspects (histometric and immunocytologic examinations, tissue calcification, inclusion bodies, neurogenic atrophies) are analysed and so are some clinical peculiarities (e.g. malignoma-association, differentiation of mixed connective tissue syndrome, drug-induced reactions). The diagnostic value of purely morphologic criteria is restricted. For better diagnostic work a great number of clinical informations is required. Nevertheless, certain morphological patterns give important directions, e.g. to occult malignoma.

Adult↗

PubMed1987

Encephalomyelopathies associated with extracerebral malignant tumors.

It was the aim of this study to examine the probability of pathogenetical relations between extracerebral malignant tumors and lesions of CNS. The term paraneoplastic should be questioned. Among a running series of 2,000 brain autopsies, 456 patients (22.8%) showed such tumors, 362 of these combined with lesions in brain or spinal cord. Out of these cases, 100 had metastases, meningoses blastomatosae or leukotic hemorrhages, 218 other, non-tumoral lesions, and 44 both tumoral and non-tumoral lesions. The last-mentioned 262 cases were the target of the examination. We distinguished 6 groups: a) various well-definable impacts, diseases or genetical defects (n = 18), b) unspecific terminal resp. agonal changes (n = 49), c) vascular or circulatory disorders incl. embolizations (20 non-leukotic hemorrhages, 148 anemic infarctions or selective neuronal necroses), thromboses, angiitis or calcifications, d) infections and other inflammatory alterations (n = 37), e) metabolic and toxic lesions (9 Wernicke's disease, 12 central pontine myelinolyses, f) anomalies difficult to classify (51 cases with subacute cerebellar atrophy, diffuse leukoencephalopathy, focal spongious axonopathic lesions, myelomalacia and other). After analysing the various lesions and discussing the probable pathogenesis we grouped according to the following scheme: I) tumor-unrelated (casual coincidence) (43.2%), II) therapy-dependent (3.7%), III) agony-related (10.7%), IV) homoiogenic disorders (e.g. larynx carcinoma and Wernicke's disease) (2.0%), V) nosocomial disorders (12.9%), VI) tumor-dependent (local neighbourhood effects, primary or secondary remote effects). As the central group there remain the primary remote effects (17.3%), separable into specific functional anomalies by tumorous organ destruction, remote effects of tumor cell-born (ectopic) release of hormones or hormone-like substances (n = 2), tumor antigen-dependent immune reactions (n = 20), and pathogenetically still uncleared mechanisms (n = 51). One should apply the term paraneoplastic only for the three last-mentioned conditions.

Adolescent↗

PubMed1986

Normomorphic sialidosis in two female adults with severe neurologic disease and without sialyl oligosacchariduria.

Two female patients of German origin, aged 38 and 21 years, with myoclonus epilepsy and cerebellar ataxia, but without dysmorphic signs and dementia, were found to excrete normal amounts of sialyl oligosaccharides in their urine. The younger patient showed cherry red spots in her ocular fundi. The older patient had a brother with an autopsy-proven neuronal storage disease compatible with sialidosis, and in her rectal biopsy lamellar inclusion bodies were detected. Enzyme assays in cultured fibroblasts of both patients revealed a profound but incomplete deficiency of oligosaccharide sialidase activity and normal beta-galactosidase activity. Adult sialidosis was diagnosed in both patients. In their fibroblasts, moderate elevations of bound sialic acid could also be measured. The small residual sialidase activity, which in the older patient had a normal KM value, is considered responsible for the late onset and slow clinical course of the disease. It is concluded that in adult sialidosis the extraneural storage process can be difficult to demonstrate in terms of metabolite accumulation or excretion during the course of intraneuronal storage.

Adult↗

PubMed1986

Borrelia-induced meningoradiculitis--two different forms of the disease.

The pattern of CSF cytological findings in the acute stage and after 10 days of antibiotic and steroid treatment was examined in 14 patients with meningoradiculitis due to an arthropode-transmitted infection with Borrelia duttoni (burgdorferi). At the acute onset of neurological symptoms, 12 patients revealed lymphocytic pleocytosis (cell count 50-500 X 10(6) cells/l) with numerous plasma cells (5-12% of the total cell count) and neutrophilic granulocytes (0.2-3.4%). 10-14 days after the onset of penicillin infusion and steroid treatment, cell count was reduced by at least 50% in 8 patients. Plasma cells then varied between 2 and 8%, whereas the neutrophilic granulocytes had disappeared. Two patients with slowly progressive neurological symptoms had a normal cell count, and inflammatory changes in CSF cytology were not observed.

Borrelia Infections↗

PubMed1986

[Spontaneous subarachnoid hemorrhage as a symptomatic manifestation of an acoustic neurinoma].

Subarachnoid hemorrhages (SAH) due to intracranial tumors are very rare. We report the clinical, neuroradiological and histological findings of an acoustic neurinoma, the initial and manifestating symptom of which was an acute SAH. In the literature we found only eight further cases of acoustic neurinomas with spontaneous SAH. The causes of the SAH are discussed; and we compare our findings to those in the literature.

Cerebral Angiography↗

PubMed1985

[Hypokalemic periodic paralysis provoked by "Ambene"].

The case of a 42-year-old man is reported, who on four occasions developed a hypokalaemic periodic paralysis after an intramuscular injection of "Ambene". The detailed examination of this patient shows, that it is the primary, autosomal dominant inherited form of hypokalaemic periodic paralysis, and not the secondary form, which is caused by a renal or gastrointestinal loss of potassium. Clinical and electrophysiological, as well as histopathological and electron microscopic findings are presented, showing the typical vacuolar myopathy with submicroscopic tubular structures. In the literature there is evidence for an increased sensitivity of the muscle membrane to insulin with an increased potassium-shift inside the cell in hypokalaemic periodic paralysis. "Ambene" is a combination, which contains amongst other substances dexamethasone and the local anaesthetic drug lidocain. In the present case the paresis was possibly caused by a combined effect of dexamethasone with a consequent hyperglycaemia and lidocain with a change in the excitability of the muscle membrane. The pathophysiological mechanism of hypokalaemic periodic paralysis is discussed in terms of the release by the combination of these two drugs. It has not previously been reported that "Ambene" can provoke a hypokalaemic periodic paralysis. This is a severe side effect because of the resulting cardiac and respiratory problems.

Adult↗