[Treatment of dysimmune neuropathies with intravenous polyvalent human immunoglobulins].
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Humans↗
Biomedical subjects
N Baumann
Publications and source records attributed to N Baumann.
Ganglioside GD1b is the target antigen for a biclonal IgM in a case of sensory-motor axonal polyneuropathy: involvement of N-acetylneuraminic acid in the epitope.
We report on a 54-year-old man with a sensory-motor polyneuropathy associated with a biclonal IgM-kappa gammopathy, which reacted with the ganglioside GD1b. Examination of nerve biopsy specimens showed some reduction in the density of myelinated fibers and axonal degeneration with a loss of large fibers and a relative increase in the density of small fibers. Immunodetection on thin-layer chromatography of the glycolipid antigens showed strong reactivity of the patient's serum IgM-kappa with GD1b ganglioside and weak binding to GD1a. biclonal IgM antibodies did not react with GM1, asialo-GM1, GT1b, GD2, or GD3. Indirect immunofluorescence staining showed binding of IgM-kappa mainly in a crescent-like pattern on the internal side of myelin sheaths, which could correspond either to an enlarged periaxonal (adaxonal) space or to the internal mesaxon or to both. The immunostaining was abolished after absorption of the serum with GD1b.
Antigens↗
Antiganglioside antibodies in motor-neuron diseases and peripheral neuropathies: study by ELISA technique and immunodetection on thin-layer chromatography.
We report here our studies on IgM reactivity towards peripheral nervous system gangliosides, in motor-neuron diseases (MND) without IgM gammopathies, and in peripheral neuropathies with IgM gammopathies. We showed by enzyme linked immunosorbent assay technique, that anti-GM1 IgM antibodies were often present at a low level in normal controls in contrast to anti-GD1b antibodies, which were never detected in control sera. We evidenced that several steps of the ELISA technique were critical such as the nonaddition of detergent in buffer solutions used for dilutions and for washing and the choice of the ELISA plates. We studied 50 cases of motor-neuron diseases, among which 40 typical cases of Amyotrophic Lateral Sclerosis, only a few had high anti-GM1 antibodies levels, which were always confirmed by immunodetection on thin-layer chromatography. These antibodies were generally directed against the oligosaccharide epitope present also in asialoGM1. No correlation has been as yet established in relation to the clinical state of the patients. In a few cases of polyneuropathies associated with IgM gammopathies, antiganglioside antibodies have been reported. We have found anti-GD1b antibodies to be present in a sensory-motor axonal neuropathy; axonal involvement was evidenced by electrophysiological study.
Autoantibodies↗
Regulation of a beta-galactoside-binding lectin and potential ligands during postnatal maturation of rat brain.
Variations in expression and activity of a beta-galactoside-specific lectin were quantified during postnatal maturation of the rat brain. Lectin expression, estimated from enzymatic immunoassay data, and lectin activity, evaluated by rabbit erythrocyte agglutination, are higher in the brains of young animals (5 days after birth) than in older ones (2 months of postnatal life). Concurrently, modifications in glycosylation during cellular differentiation were detected by affinoblotting using a biotinylated derivative of a beta-galactoside-specific brain lectin. This study shows that membrane and cytosolic glycoconjugates of the rat brain bear appropriate beta-galactoside moieties which may be recognized by an endogenous lectin expressed in the tissue itself in relation to brain maturation. The observed variations in the expression of lectin and complementary glycoconjugates can be correlated with significant events of rat brain development.
Aging↗
Adult-onset metachromatic leukodystrophy presenting as isolated peripheral neuropathy.
A 38-year-old man presented with weakness of the lower limbs. Electrophysiology revealed a pronounced demyelinating neuropathy. Nerve biopsy disclosed de- and remyelinating lesions and characteristic lamellar inclusions in Schwann cells and macrophages. There was no familial history of neurologic disorder, and impairment of motor evoked potentials was the only sign of CNS involvement. Arylsulfatase A and cerebroside sulfate sulfatase activities in leukocytes and cultures of the patient's fibroblasts were low. The sulfatide loading test also revealed abnormal sulfatide accumulation. This may be the first reported case of adult metachromatic leukodystrophy presenting as peripheral neuropathy.
Adult↗
Anticonvulsant effect of yohimbine in quaking mice: antagonism by clonidine and prazosine.
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Animals↗
[Topography of soluble gliofibrillar protein (GFA) in aged human brain].
A great variation was observed in the amount of soluble GFA depending on the brain area. Large amounts were found in the chiasma and in the pons whereas the cortical grey matter was poor. It is suggested that soluble GFA represents a potentiality of defense of the nervous tissue.
Aged↗
[Electron microscopic and neurochemical study of Alexander's disease (author's transl)].
The authors report the results of a cerebral and of a neuromuscular biopsies and of the autopsy findings in another infantile case of Alexander's disease in a girl. They review the 17 previously reported cases of this disease and the various etiopathogenic hypotheses mentioned. The presence of numerous, sometimes abnormal enlarged mitochondria and of abundant membranous cytoplasmic bodies in the astrocytic cytoplasm seems to be unreported elsewhere. Peripheral nerve changes are mentioned for the first time.
Astrocytes↗
Analysis of the major lipid classes in human peripheral nerve biopsies. Age group differences and abnormalities of ganglioside level in perhexiline maleate therapy.
We report here the results of a simple and reproducible technique which can be used in semi-routine analysis of peripheral nerve biopsy specimens, so as to have a quantitative analysis of the major lipid classes, i.e. cholesterol, cerebrosides, ethanolamine phospholipids, phosphatidyl-choline, phosphatidyl-serine + phosphatidylinositol, sphingomyelin and gangliosides. Glycolipid hexoses, cholesterol and total phospholipids have been compared in different age groups. Although all lipid classes increased from the younger to the older age group, the molar ratio of cholesterol to phospholipid differed less than the glycolipid to phospholipid ratio. Both increased significantly, even between age group 10--16 and older patients (36, 54, 61, 68, 72 and 73 years old). Although individual variations in lipid content are noteworthy, it must be emphasized that evolution with age of the lipid composition must be taken into account. Furthermore, this study confirms and extends earlier findings of increased ganglioside levels in some cases of peripheral neuropathies observed during perhexiline maleate therapy where characteristic lipid-like polymorphous inclusions have been demonstrated.
Adolescent↗
In vivo incorporation of exogenous [1-14C]stearic acid into neurons and astrocytes.
Exogenous stearic acid is needed to synthesize the membranes of neurons and astrocytes. Subcutaneously injected [1-14C]acid is taken up through the 'blood brain barrier' and incorporated into lipids of both cell types, the specific radioactivity being higher in astrocytes as compared to neurons (2200 and 800 cpm/mg proteins, respectively), 20 h after injection. Phospholipids contain high amount of radioactivity (80% in astrocytes, 65% in neurons); glycosphingolipids contain low quantities of label in the two cell types. The injected acid is partly metabolized in the brain by elongation and desaturation (thus providing very long chains, saturated mono-unsaturated and poly-unsaturated); it is also partly degraded into acetate units (utilized for synthesis of palmitic acid).
Animals↗
Absence of the major dense line in myelin of the mutant mouse "shiverer".
The myelin of the central nervous system (CNS) of the mutant mouse Shiverer is characterized by the absence of the major dense line (MDL). The intraperiod line, as seen in conventional electron micrographs and in freeze-fractured replicas, appears normal. Peripheral myelin, as seen in ventral and dorsal roots of spinal cord, is unaffected by the mutation. During the period of active myelination, the cytoplasm of most oligodendrocytes (ODs) is packed with electron-lucent vacuoles in continuity with the Golgi apparatus and with bundles of microtubules. It is concluded that a metabolic pathway possibly involving the Golgi apparatus, and contributing to the formation of the MDL is selectively affected in this mutant.
Animals↗
Immunochemical studies of myelin basic protein in shiverer mouse devoid of major dense line of myelin.
The myelin-deficient mutant Shiverer (Shi/Shi) lacks basic protein (MBP) in the myelin of its central nervous system (CNS). Less than 3% of the normal content in MBP is present in a brain extract of Shi/Shi as determined by radioimmunoassay. Indirect immunofluorescence is negative when using specific anti-MBP serum. The importance of Shi/Shi (as compared to other hypomelinating mutants) stems from the specificity of this genetic lesion, i.e. the lack of basic protein.
Animals↗
GFA and S 100 protein levels as an index for malignancy in human gliomas and neurinomas.
Human glia-specific proteins S 100 and GFA were quantitated by use of a rocket immunoelectrophoresis technique with monospecific antisera. No relation was found between the S 100 protein content of an astrocytoma and its degree of neoplasia. However, the lower the GFA protein content of the astrocytoma, the more malignant it was. Similarly, the more malignant a neurinoma was, the lower was its S 100 protein content. Therefore, the levels of these proteins might be used as indexes of neoplastic dedifferentiation.
Astrocytoma↗
Infantile form of so-called neuronal ceroid lipofuscinosis: lipid biochemical studies, fatty acid analysis of cerebroside sulfatides and sphingomyelin, myelin density profile and lipid composition.
The biochemical analysis of a case of infantile neuronal ceroid lipofuscinosis, as determined by clinical and neuropathological findings, is presented. A diminished amount of solids is found, the amount of lipids is 30% of the normal as expressed in lyophilized tissue. The yield of myelin isolated by the density gradient is 1.8% of the normal. Phospholipid patterns show a reduction in ethanolamine phosphoglyceride, N-acetylneuraminic acid is extremely low and sphingolipids are largely reduced, cerebrosides being most affected (2.5% of the normal). In cerebrosides and sulfatides the decrease in very long chain fatty acids is important, but the deficiency in any type (including hydroxy compounds) is not too dramatic. According to the aspect under electron microscopy, the density profile, and the biochemical composition of the subfractions, isolated myelin is close to normal. The loss of the myelin sheath appears to reflect a Wallerian degeneration in the CNS: myelin loss is a secondary effect. This disease, from a biochemical point of view, seems to be the ideal control for leukodystrophies.
Atrophy↗
[Mini-review on the properties of glial cells of the central nervous system (author's transl)].
During the past 15 years, increasing studies have been devoted to glial cells, i.e. oligodendrocytes, astrocytes and microglial cells of the central nervous system. Although most of their functions remain mysterious, the concept of intracellular interactions between neurons and neuroglia becomes more and more substantiated. This review describes some aspects of phylogeny of glia, recent technical approaches to the study of these cells, their major morphological and biochemical characteristics, their maturation as well as their pathology.
Animals↗
Bismuth intoxication: bismuth level in pig brain lipids and in subcellular fractions.
Experimental intoxications of pigs were performed. It seems to be confirmed that bismuth crosses the blood-brain barrier. An organic derivative, trivinyl-bismuth, is more active than the inorganic salt of bismuth. In the brain, bismuth is preferentially found in synaptosomes. Bismuth is found in brain lipids of control pigs at very low levels. In intoxicated pigs, the level of bismuth is increased mainly in cerebellum and then in thalamus. Bismuth is partly associated to lipids. There is not a correlation between the level of bismuth in blood and in brain lipids. However, in the trivinyl intoxicated pig, a high level of blood bismuth is concomittant to a high level of brain lipid bismuth. The high content of this metal in cerebellum lipid extract may be of functional significance.
Animals↗
[Extraction of plasma lipids preserving antigenic properties of proteins and allowing quantitation of gangliosides by neuraminic acid determination (author's transl)].
A technique described by Cham and Knowles allows the extraction of plasma phospholipides, triglycerides, cholesterol and free fatty acids without denaturing the proteins. The method has been slightly modified by us. Under our conditions, the yield of lipid extraction is comparable to the method of Folch. It has the advantage of not extracting mineral phosphorous and blood glucose. Thus gangliosides can be also easily determined. We confirmed by immunoelectrophoresis that the antigenic properties of plasmatic proteins remain after lipid extraction.
Blood Proteins↗
[Study of gangliosides in normal (C57B1/6J) and quaking mice. Presence and characterization of a labile ganglioside in alkaline solution].
In the adult Quaking mutant, there is an increase in some polysialogangliosides (GT1 and Gq) and a 50% decrease in BM1 content. Thus, the latter ganglioside, which is a constituent of mature myelin, could be linked to one of the late steps of the myelination process. A novel ganglioside ALG1, which appears during the postnatal development of brain, has been isolated and purified; it contains an alkali labile linkage and after alkaline treatment yields GT1b. Comparison between normal Mice and Quaking mutants does not show any significant quantitative differences, under our conditions. At the stage at which it appears, it could be linked to steps of brain maturation other than myelimation.
Animals↗