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Biomedical subjects

W Schlote

Publications and source records attributed to W Schlote.

At least 73 records · Page 4Linked to original sources
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↗
PubMed1984

Acid phosphatase activity in human neuronal and glial lipofuscin.

The ultrastructural demonstration of acid phosphatase activity in neuronal and glial lipofuscin of the aged human brain indicates lysosomal activity. Contrary to the constantly high enzyme activity of astroglial lipofuscin and low activity of oligodendroglial lipofuscin, the enzyme activity of neuronal pigment differs in different locations, i.e., in different neuronal populations. The activity is probably based on the importation of lysosomal enzymes into the pigment component of lipofuscin. These facts indicate the possibility of a continuous autophagosomal function of lipofuscin in neuronal and glial cell physiology. Therefore, the findings are arguments against the view that lipofuscin is a wear-and-tear material only and claim for it an active role in cell metabolism.

Acid Phosphatase↗
PubMed1984

Diffuse-disseminated sclerosis combined with partial arylsulfatase A (ASA) deficiency. Mixed heterozygosity of ASA- and pseudo-ASA-deficiency?

In a four and a half-year-old boy with a progressive cerebral process in his last year of life the arylsulfatase A (ASA) and cerebroside sulfatase activity was lowered to values only slightly above those of metachromatic leukodystrophy (MLD) homozygotes. Morphologically, however, the brain showed a diffuse-disseminated sclerosis without any findings of MLD. The enzyme findings including the reduced ASA values in both parents suggested that our proband was a mixed heterozygote both of MLD and pseudo-ASA-deficiency, but casually suffered from that inflammatory demyelinating process. An additional biochemical finding was the moderate increase of brain sulfatides which can be seen in relation to the mixed heterozygosity for ASA deficiency.

Brain↗
PubMed1983

[Brain changes in parathion poisoning: observations in 42 cases].

A total of 42 cases were examined neuropathologically to determine possible toxic changes occurring in the brain after parathion intoxication. Sporadic anoxic alterations were observed in 7% of 41 cases in which the cause of death was acute intoxication. Nearly all cases (93%), however, showed marked hyperemia, often coupled with small reactionsless, periventricular hemorrhages which occurred in 40% of the cases. In one third of the cases (33%) there was moderate swelling of the oligodendroglia. Whereas histological evidence of edema was found in nearly one third of the cases (30%), comparison of the brain weights in these subjects with those of a large comparative collective showed definite pathologic brain weights in only five cases (12%) with 95% confidence limits. It is true that in 18 cases (42%) the brain weight was above the normal value if the confidence limit is ignored. Pronounced anoxic alterations were observed in only one case in which the individual survived the acute intoxication for 4 weeks after initial respiratory arrest. Predominantly toxic changes, however, could not be detected in any of the cases examined. This negative morphologic finding does not agree with the physiologic alterations reported by other investigators using animal models; they considered the cause of death in cases of parathion intoxication to be the result of toxic paralysis of the respiratory center. The literature was discussed.

Adolescent↗
PubMed1982

[Ultrastructural findings in 9 fetuses following prenatal diagnosis of neurolipidoses].

After positive prenatal enzymic diagnosis of different neurolipidoses therapeutic abortion was carried out in the 19th to 25th week of pregnancy. Ten delivered fetuses were studied ultrastructurally and in nine of them positive results were obtained, although in some cases one had to accept relatively poor structural conservation of fetal tissues. The ultrastructure of the quantitatively small lipid storage effects qualitatively resembled that of the postnatal stages with some exceptions of localization. In fetal GM2-gangliosidosis type 2 (variant 0) concentric membranous cytoplasmic bodies were detected in the brain cortex. In Krabbe's disease the myelinated regions of the spinal cord showed scattered storage (globoid) cells, sometimes closely related to blood vessels, which contained isolated or stranded tubular or spicular inclusions. In GM1-gangliosidosis type 1 neurons of the brain stem showed lamellar inclusions structured as zebra bodies, and splenic histiocytes exhibited numerous almost clear cytoplasmic vacuoles. In fetal metachromatic leukodystrophy the CNS including myelinated regions was essentially free of morphologic lipid storage effects. However, many kidney tubules cells contained great numbers of irregular or roughly parallel stacks of membranes. These inclusions may be equivalent to "tuffstone" bodies. In one fetus the bodies were restricted to tubular cells bearing microvilli. Fluorescent microscopy of arcus of the kidney tubule showed excess amounts of metachromatic material. Less of this material was demonstrable in the envelope layer of hepatic Glisson triangles. In the fetus with Niemann-Pick disease type C large neurons of the basal ganglia and the spinal cord were filled with membranous inclusions that were similar to myelin-shaped bodies rather than to solid membraneous bodies. The 19-weeks-old fetus with enzymically proven Gaucher disease was free of ultrastructural lipid storage effects. Most but not all of the morphological findings in the fetuses with neurolipidoses were in accordance with published results.

Adult↗
PubMed1982

Slowly progressive congenital myopathy with cytoplasmic bodies--report of two cases and a review of the literature.

Observations on two patients from unrelated families are reported. Both patients were 32 years old and suffered from a congenital affection of the skeletal muscles. In one (case 2) congenital paresis of the dilator muscle of pupil and cardiomyopathy were also present. In both cases muscle biopsy showed pathologic variations of muscle fiber caliber, and in case 1 atrophic fibers were grouped in some places. Inclusions consisting of dense granular and fine filamentous components were found in skeletal muscle fibers, in about a third of the fibers in case 1 and in about a tenth of the fibers in case 2. The shape and submicroscopic structure of these inclusions were consistent with so-called cytoplasmic bodies. Surrounding or apart from the inclusions, the streaming of Z disks or their disintegration were observed. In addition, pure filamentous bodies occurred in the subsarcolemmic position in both cases. Although cytoplasmic bodies are a nonspecific type of inclusion, they were the outstanding structural anomaly in both cases. A basic defect of myofilament biosynthesis is considered to be the cause of the disorder. It is presumably evoked by a neurogenic factor affecting the trophic influence of the neuron on the muscle cell. The course of the disease may (case 1) but need not (case 2) terminate in severe impairment of skeletal muscle function.

Adult↗
PubMed1981

Ionic shifts in myelinated nerve fibers during early stages of Wallerian degeneration.

The distribution and relative mass fraction of the diffusible ions Na, K, Cl and Ca were determined by X-ray microanalysis in the axons of rat sciatic nerve 18 h and 36 h after crush and in control nerves. The investigations were performed in freeze-dried ultrathin cryosections after shock freezing of the nerves in liquified propane. 18 h after crush, no definite alteration of the ions were found compared to the control nerves. In electron micrographs of routinely processed nerves, no ultrastructural changes were seen. 36 h after crush, two types of ionic imbalance were found, the first characterized by decreased K and slightly increased Na in the axon, the second by concurrently increasing axonal Na and Cl, accompanied, in some fibers, by accumulating Ca. These types of ionic imbalance presumably represent two stages of axolemmal permeability alteration corresponding to early structural changes of axoplasm in electron micrographs of routinely processed nerves at that time.

Animals↗
PubMed1981

[Morbus Gerstmann-Sträussler-Scheinker. The Sch. family-a report of three cases (author's transl)].

The clinical symptoms from three cases and histological findings from two patients suffering from morbus Gerstmann-Sträussler-Scheinker (M-GSS) are reported. This disease belongs to the group of subacute spongiform encephalopathies. It is extremely rare and so far has only been observed in 52 members of four large families, in which the symptoms begin between the age of 33 and 50 and lead to death in 4-5 years. In the family reported here, cerebellar symptoms including myoclonia and later dementia, bulbar, and pyramidal symptoms were typical; two patients also had deterioration of vision and hearing. CSF and other biochemical data were normal. The EEGs showed progressive general slowing without periodic dysrhythmia. Evoked potential gave no evidence of demyelinization. The disease may safely be distinguished from morbus Creutzfeldt-Jakob (M-CJ) and Alzheimer's disease by histology, which reveals kuru plaques in most cases and invariably multicentric plaques as well as cortical spongiform changes of varying degree with loss of nerve cells and glial proliferation; however, only minor degenerative alterations in the cortical vessels are seen. The transmission to monkeys and histological similarities to M-CJ and kuru suggest a slow virus related to that causing scrapie. Alternatively, the genetically determined susceptibility of the patient may decide the type of reaction to the slow virus. The disposition to M-GSS is autosomally dominant.

Adult↗
PubMed1980

Gerstmann-Sträussler-Scheinker's disease. Electron-microscopic observations on a brain biopsy.

Light and electron microscopic observations are reported on a brain biopsy of a man of 59 with a rare familial disease of the CNS and a 5-year clinical course. Electron micrographs of the frontal biopsy reveal plaque-like deposits composed of amyloid cores, often multicentric in the cortex and subcortical white matter. They are localized between enlarged astrocytic processes. In the neuropil they are sometimes associated with abnormal neuritic processes, in the white matter with processes of fibrous astroglia and basement membranes. There are no signs of primary neuritic or synaptic involvement in the plaque formation which is more obviously associated with altered astrocytic processes. Moreover, degenerative alterations in the cortical vessels and slight astroglial spongiform changes as well as oligodendroglial proliferation can be found. Plaques are considered to be mainly of the kuru type; the relationship with transmissible spongiform encephalopathies is discussed.

Astrocytes↗
PubMed1980

[Subacute spongiform encephalopathy with multiform plaque formation. "Peculiar familial-hereditary disease of CNS [spinocerebellar atrophy with dementia, plaques, and plaque-like deposits in cerebellum and cerebrum" (Gerstmann, Sträussler, Scheinker)] (author's transl)].

Report of two unrelated cases of a rare familiar disease of degenerative nature, from a clinical point of view belonging to the spinocerebellar atrophies, combined with dementia. According to the pedigrees, the disease can be followed up to 3--4 generations. Microscopic study reveals glioneuronal dystrophy with spongiform changes together with kuru plaques and atypical plaque-like formations, hitherto only described in this disease. Nosological aspects of these findings are discussed.

Atrophy↗
PubMed1980

[Influence of 2-ethyl-3-(4-hydroxybenzoyl)-benzofuran (benzarone) on experimental atheromatosis (author's transl)].

Local transmural electrical stimulation by chronically implanted electrodes of a carotid artery in rabbits was daily repeated. The area in direct contact to the electrodes covered 0.5 x 5 mm of adventitia. The carotid walls received DC impulses (10 ms/imp, 10 Hz, 100 microA) 2 x 1/2 h daily. Within 4 weeks a proliferate of smooth muscle cells develops in the intima at the spot where the anode contacts the outside of the artery. If the animals were fed 2% cholesterol in normal food the proliferate developed to a typical atheroma. The other parts of the arterial system did not develop plaques within this time. At the luminal side of the plaques no significant adhesions of platelets could be seen. Addition of 300 mg 2-ethyl-3-[-4-hydroxybenzoyl]-benzofuran (benzarone, Fragivix) to 100 g of animal food (containing 2% cholesterol) caused a less pronounced increase of serum cholesterol than in the controls which received cholesterol-containing food without benzarone. The development of plaques could not be prevented by benzarone, however, the growth of the atheromatous plaque was inhibited and the incorporation of lipids into the plaques was less pronounced than in those animals which received a diet with cholesterol but without benzarone. In those animals which received benzarone more lipid-laden foam cells were seen moving through the endothelium out of the plaque into the lumen of the stimulated artery. These foam cells were of mononuclear origin.

Animals↗
PubMed1980

Cryoultramicrotomy, electron probe microanalysis and STEM of myocardial tissue.

Heart muscle preparations (papillary muscles and trabeculae) were frozen at 4.2 K on metal plate under vaccum after their length-tension relationships showed that no damage had occurred during dissecting and mounting the strips on the holder. Freeze substitution of some preparations directly after freezing demonstrated that no important cell damage due to ice crystals occurred in superficial cell layers during freezing. Ultrathin cryosections obtained at -130 degrees C were freeze dried and analyzed, in the STEM mode. The better the freezing procedure, the poorer was the contrast of the sections under electron microscopy. Preliminary approaches to increasing contrast after sublimation of tissue water show that a small increase in contrast is generally obtained at the cost of the peak/background ratio due to pronounced mass loss. The results of our analyses show that C1 content in heart muscle cells is high and distributed throughout the cytoplasm. Ca is detectable and quantitable in resting muscle in SR cisternae. The Ca amount in cytoplasm is low and just at the limit of detectability under our current analysis conditions. Preliminary experiments on papillary muscles subjected to caffeine contracture showed that calcium is not detectable in the cisternae as is the case in control experiments. Only a moderate amount of Ca is detectable in mitochondria, whereas the concentrations in cytoplasm, as in resting cardiac muscle, is too low to be quantitated.

Animals↗
PubMed1979

Ultrastructural and biochemical changes of human papillary heart muscle during different methods of induced cardiac arrest.

To determine the protective effects of different methods of cardioplegia, studies on ATP/lactate levels and ultrastructure were performed in human papillary muscles obtained during mitral valve replacement. In group I (n = 5), plain ischemic arrest in hypothermia (systemic venous temperature = 24 degrees C) was accomplished. In group II (n =12), the heart was arrested by injection cardioplegia using magnesium-aspartate-procaine at systemic venous and myocardial temperatures of 24 degrees C. In group III (n = 12) Bretschneider infusion cardioplegia at systemic venous and myocardial temperatures of 26 degrees C and 19 degrees C respectively was applied. With regard to ultrastructural changes there were no clearcut differences in the three methods of hypothermic cardiac arrest after 60 minutes of ischemia. Ischemic changes tended to be slightest in group III (infusion cardioplegia). ATP decay and lactate increase were significant in group I and moderate to minimal in groups II and III after the same period of time. It is concluded that for aortic cross-clamp times up to 60 minutes, body hypothermia and injection cardioplegia using magnesium-aspartate-procaine at a myocardial temperature of 24 degrees C provide adequate protection of the myocardium. For ischemia times beyond 70 minutes, profound myocardial hypothermia below 20 degrees C is preferred.

Adenosine Triphosphate↗