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Biomedical subjects

H Bernheimer

Publications and source records attributed to H Bernheimer.

At least 55 records · Page 3Linked to original sources
PubMed1986

Anti-myelin antibodies in cerebrospinal fluid and serum of patients with meningopolyneuritis Garin-Bujadoux-Bannwarth and other neurological diseases.

Anti-myelin antibodies (AMA) of IgG, IgM, and IgA class were investigated by ELISA in CSF and serum from patients with meningopolyneuritis Garin-Bujadoux-Bannwarth (GBB), other inflammatory diseases of the nervous system (ID) comprising meningoencephalitis (ME), multiple sclerosis (MS), and Guillain-Barré syndrome (GBS), and various noninflammatory neurological diseases (NID). Anti-Borrelia antibodies (ABA) were determined by ELISA in GBB patients. In CSF, a high incidence of IgG-AMA, IgM-AMA, and IgA-AMA was found in GBB as compared with ID and NID. On average, positive AMA titers were higher in GBB than in ID and NID, IgM-AMA titers in GBB being most prominent. In serum, AMA were found in all but 2 patients investigated. On average, IgM-AMA titers were higher in GBB and MS than in other diseases; IgG-AMA titers in GBB and ME were relatively low. Antibody indices, calculated from titer values and Ig concentrations in CSF and serum, indicate intrathecal synthesis mainly of IgG- and IgA-AMA, and of IgG- and IgM-ABA. Participation of AMA in the pathogenesis of GBB may be envisaged, but needs further confirmation.

Antibodies, Bacterial↗
PubMed1985

Occurrence of IgA subclasses (IgA1 and IgA2) in the human nervous system. Correlation with disease.

The occurrence of IgA subclasses in pathological conditions of the nervous system was studied by means of monoclonal antibodies and an indirect immunofluorescence technique. IgA1- and/or IgA2-positive lymphoid (plasma) cells were found in demyelinating diseases comprising multiple sclerosis, Guillain-Barré syndrome, and adrenoleukodystrophy, in various inflammatory diseases, and in tumors, some of which exhibited labeling of tumor cells. Demyelinating and inflammatory diseases with chronic course displayed some prevalence of IgA2-, and tumors some prevalence of IgA1-positive cells. This is the first demonstration of IgA1 and IgA2 in the nervous system.

Alzheimer Disease↗
PubMed1985

The cerebrohepatorenal (Zellweger) syndrome: an improved method for the biochemical diagnosis and its potential value for prenatal detection.

The sequence of reactions involved in plasmalogen biosynthesis has been evaluated in cultured fibroblasts of patients with the cerebrohepatorenal syndrome. A double-label, double-substrate incubation using [1-14C] hexadecanol and 1-0-[9', 10'-3H]hexadecylglycerol was performed to monitor the relative rates of peroxisomal and microsomal biosynthesic steps. [14C] radioactivity associated with 1'-alkenyl groups of plasmalogens was found to be drastically reduced in fibroblasts of affected patients whereas [3H] incorporation was apparently normal. This finding is specific for cerebrohepatorenal syndrome fibroblasts since cell lines of patients with childhood adrenoleukodystrophy and neuronal ceroidlipofuscinosis utilized the lipid precursors of plasmalogen biosynthesis at normal rates. The results show that the defect in plasmalogen synthesis in the cerebro-hepato-renal syndrome is restricted to the peroxisomal steps. The finding of normal microsomal biosynthetic steps was exploited to devise a novel diagnostic assay in fibroblasts and amniocytes based on the comparison of [3H/14C] isotope ratios within aldehydes released from plasmalogens by acid hydrolysis. The procedure can be completed with a minimal amount of cells since it renders quantitative analyses unnecessary. Therefore, this technique appears ideally suited for the sensitive and safe prenatal diagnosis of the cerebro-hepato-renal syndrome.

Abnormalities, Multiple↗
PubMed1984

Serum antibodies against glycosphingolipids in chronic relapsing experimental allergic encephalomyelitis. Demonstration by ELISA and relation to serum in vivo demyelinating activity.

Sera from guinea pigs with spinal cord-induced chronic relapsing experimental allergic encephalomyelitis (crEAE) were tested for IgG antibodies against glycosphingolipids (GSL; galactocerebroside, ganglioside GM1, sulfatide) by an enzyme-linked immunosorbent assay and for in vivo demyelinating activity by infusion into the lumbosacral subarachnoid space of normal rats. In chronic stage-crEAE sera (40-200 days after sensitization) a high incidence (21/26) and high titers (up to 1:2560) of antibodies against one or more GSL coincided with a high incidence (22/26) of in vivo demyelinating activity. These results suggest an involvement of antibodies against various GSL in the process of demyelination.

Animals↗
PubMed1983

Brain tissue immunoglobulins in adrenoleukodystrophy: a comparison with multiple sclerosis and systemic lupus erythematosus.

Immunoglobulin (Ig) concentrations were investigated in white matter samples of two adrenoleukodystrophy (ALD), three multiple sclerosis (MS), two systemic lupus erythematosus (SLE), one rheumatoid arthritis, and three control brains obtained at autopsy. "Free" Igs were extracted at pH 7.4; subsequently, bound Igs were extracted at pH 2.5 and 10.8, respectively. Igs were quantified by radial immunodiffusion. In ALD material there was an increase of free IgG and IgA, in one sample also of IgM, as compared to controls. No significant amounts of Igs were detected in the pH 2.5 and 10.8 extracts of ALD brain. Similarly to ALD, an increase of free IgG and IgA was a characteristic finding in MS brain; in contrast to ALD and control material, significant amounts of bound Igs (IgG) extractable at acid or alkaline pH, respectively, were present in MS tissue. In both SLE brains increase of free IgM was conspicuous. Preliminary studies on binding of Igs extracted at pH 7.4 from brain to frozen sections of normal human and bovine brain tissue revealed different binding properties of Igs from ALD, MS, SLE, and control brains. Immunochemical findings in ALD indicating pathologic accumulation of Igs in brain tissue were paralleled by immunocytochemical observations demonstrating accumulation of lymphoid cells staining for IgG, IgA, and IgM, respectively, mainly in areas of recent demyelination. Participation of Igs in the pathogenesis of ALD lesions may be considered but needs further confirmation.

Adolescent↗
PubMed1982

Detection of adrenoleukodystrophy by increased C26:0 fatty acid levels in leukocytes.

Very long chain fatty acids of peripheral blood leukocytes were analyzed by gas chromatography in nine members of a family including two hemizygotes and one obligate heterozygote for adrenoleukodystrophy (ALD), as well as in twelve controls. Comparative investigations were done in cultured fibroblasts. Elevated C26:0 levels were observed in leukocytes and fibroblasts of ALD hemizygotes. The obligate heterozygote displayed a clear-cut increase of C26:0 concentration in leukocytes but not in fibroblasts. Determination of C26:0 in leukocytes may serve as diagnostic tool in the detection of ALD gene carriers.

Adolescent↗
PubMed1982

Neurochemical investigations of aged human brain cortex.

Neurochemical investigation of FC and TC of 10 brains of old age and 10 controls did not reveal significant differences of total ganglioside, DNA, protein, and lipid levels. With regard to individual gangliosides there was a significant decrease of the percentage of GM1 in the FC and a significant decrease of GM1, GD1a, GM2 as well as a significant increase of GD1b and GD3 in the TC of the aged brains. Our results present some neurochemical evidence for regionally different changes in neuronal processes in the aged human brain, whereas neuronal cell loss might play only a minor role in this condition.

Adolescent↗
PubMed1982

A ganglioside-dependent cellular binding mechanism in rat macrophages.

A rosetting assay was performed with rat alveolar and peritoneal macrophages and sheep red blood cells (SRBC) treated with gangliosides. SRBC incubated with GM2, GD1a, and a mixture of bovine brain gangliosides (BBG) showed binding to rat macrophages. The extent of binding was dependent on the concentration of gangliosides. Binding induced by GM2 was stronger than that induced GD1a and BBG. GM1 and GA2 did not induce rosette formation. Inhibition studies with gangliosides, sialyllactose, lactose, and neuraminic acid suggested that the macrophage binding site recognizes neuraminic acid in conjunction with neighboring carbohydrates showing highest affinity for GM2. Sodium azide, sodium fluoride, ethylenediaminetetraacetate, cytochalasin B, and colchicine did not inhibit rosette formation. The percentage of peritoneal macrophages binding ganglioside-treated SRBC was lower than that of alveolar macrophages. Proteose-peptone-stimulated peritoneal macrophages, however, showed an increase of rosette formation. Around 30% of adherent spleen cells also had binding activity for GM2-treated SRBC. Peripheral blood mononuclear cells did not bind ganglioside-treated SRBC. Phagocytosis of GM2-treated SRBC attached to alveolar macrophages could not be observed. It is suggested that macrophages may express recognition sites for certain gangliosides that might be important in cell-cell interaction.

Animals↗
PubMed1982

Adult GM2 gangliosidosis masquerading as slowly progressive muscular atrophy: motor neuron disease phenotype.

Ultrastructural and biochemical studies were performed on postmortem material of a 67-year-old woman presenting with proximal muscle weakness in the legs, slurred speech, and mental subnormality. The symptoms began at age 19 and showed extremely slow progression, mimicking progressive muscular dystrophy. A brother suffered from a similar chronic neuromuscular disease, and two sisters died at an early age from unknown "nervous" diseases. Autopsy disclosed abundant lipid accumulation in CNS neurons and severe cerebellar cortical atrophy of the granule cell type. Skeletal muscle showed a terminal stage of denervation atrophy with severe lipomatosis; intrafusal fibers of muscle spindles contained lipid deposits. Complex lamellar cytoplasmic inclusions often resembling membranous cytoplasmic bodies or stacked membranes were seen in cells of the brain. In addition, there were various lipopigment bodies, fingerprint profiles, rare polyglucosan bodies, rodlike structures, and filamentous sheaves, particularly in substantia nigra. Accumulation of gangliosides GM2 and GA2 in the cerebral cortex was demonstrated by thin-layer chromatography. Determination of hexosaminidase activity was not possible (formalin-fixed material). This observation, in addition to the cases reported by Navon et al. [1981] and Johnson [1982], is suggested to represent a new phenotype of adult-onset GM2 gangliosidosis referred to as motor neuron disease phenotype, which can be differentiated from other adult-onset lipidoses and motor neuron disorders. Our paper emphasizes the importance of ultrastructural demonstration of lamellar inclusions for the differential diagnosis of ceroid lipofuscinosis, and the value of biochemical studies in the diagnostic clarification of atypical neuromuscular disorders.

Adult↗
PubMed1981

Fractionation of spinal cord tissue affects its activity to induce chronic relapsing experimental encephalomyelitis.

Chronic relapsing experimental allergic encephalomyelitis (EAE), an animal disease closely resembling multiple sclerosis, was induced in young guinea pigs by sensitization with guinea pig spinal cord tissue together with complete Freund's adjuvant and a high amount of mycobacterium tuberculosis. Sensitization of animals with myelin basic protein leads to an acute form of EAE, but not to a chronic demyelinating disease. The present study was designed to trace the factors of spinal cord tissue responsible for chronic disease and demyelination by using fractionated spinal cord tissue for sensitization. The following preparations were tested for encephalitogenic activity: a) total guinea pig spinal cord homogenized in chloroform/methanol (C/M), b) C/M residue (protein fraction), c) C/M extract (lipid fraction) and d) bovine brain gangliosides. C/M treated spinal cord preparations were less encephalitogenic as compared to untreated spinal cord. The protein fraction showed very little encephalitogenic activity, the histological changes were limited to perivascular inflammation without demyelination. A crude lipid fraction induced some chronic inflammation. Neither the purified lipid fraction nor gangliosides produced any EAE symptoms. The conclusion can be made that protein is necessary for the induction of chronic relapsing EAE, whereas lipid or a protein-lipid complex seems to be responsible for the demyelinating component of this disease.

Animals↗
PubMed1981

Fatty acid patterns in brain, fibroblast, leukocyte and body fluid lipids in adrenoleukodystrophy.

Fatty acid patterns of certain tissue and body fluid lipids of ALD and control cases were investigated by thin layer and gas chromatography. A high percentage (55.2%) of VLFA was found in the ALD brain white matter cholesterol esters; this was mainly due to increased proportions of 26:0, 25:0, 26:1 and greater than 26 C fatty acids. The percentage of VLFA in the white matter cholesterol esters of pathological controls with myelin breakdown of different etiology was low (0.4-7.1%). In the fibroblast total lipids of an ALD patient an increase of 26:0 and 25:0 fatty acids was found. Moderately increased proportions of hexacosanoic acid were observed in the leukocyte total lipids of 2 severely affected ALD patients, but not in a slightly as well as in a possible affected case. No accumulation of VLFA was detected in cholesterol ester, triglyceride and free fatty acid fractions in the CSF of an ALD patient and in ALD serum sphingomyelin. Our results substantiate the specificity of VLFA accumulation in the white matter cholesterol esters of ALD brain and lend some further evidence to the view that a generalized impairment of VLFA metabolism seems to be present in this disease.

Adolescent↗
PubMed1981

Striatal ganglioside levels in the rat following kainic acid lesions: comparison with Huntington's disease.

Ganglioside and DNA levels were estimated in the striatum of rats 10 days and 6 weeks after lesioning by intrastriatal injection of kainic acid. There was a moderate, 21-24% decrease of the ganglioside concentration per unit protein on the side of the lesion, which can be ascribed to the loss of the intrinsic striatal neurons following the injection of kainic acid. On the other hand, there was a 131 and 60% increase of DNA per unit protein in the kainate injected side 10 days and 6 weeks after the lesions, respectively; these changes apparently reflected the gliotic reaction brought about by the neurotoxin. Qualitatively similar findings--a decrease of ganglioside and an increase of DNA levels per unit protein--were also found in the brain of patients with Huntington's disease; however, as compared with the corresponding control material, the decrease of the ganglioside concentration was more pronounced in the striatum of Huntington's disease (by 38% in the caudate nucleus and by 46% in the putamen) than in the kainate lesioned rat striatum. This difference could be due to the different proportions of the intrinsic and extrinsic neuronal plasma membranes in the striatum of the two species; however, the possibility of a more generalized affection of neuronal plasma membranes in Huntington's disease may also be envisaged.

Animals↗
PubMed1981

Accumulation of very long chain fatty acids is common to 3 variants of adrenoleukodystrophy (ALD). "Classical" ALD, atypical ALD (female patient) and adrenomyeloneuropathy.

Fatty acids of cholesterol esters were analyzed by gas chromatography in affected CNS white matter of 3 variants of ALD ("classical" ALD, atypical ALD (adult female) and AMN) and of 10 controls with myelin breakdown of an etiology other than ALD. In all 3 ALD variants a marked accumulation of very long chain fatty acids (VLFA) as compared to control material was observed. This was due to the accumulation mainly of saturated C24-C32 fatty acids, particularly of C26:0, C25:0 and, to a lesser extent, C24:0 and C27:0 fatty acids. Our results demonstrate for the first time an accumulation of VLFA in an adult female patient (atypical ALD), who probably is an ALD heterozygote rather than a variant of AMN, and confirm and extend earlier findings in classical ALD and AMN, respectively. It appears that ALD may be a single nosological entity with clinically and morphologically different variants sharing specific ultrastructural (accumulation of paired leaflets) and neuro-biochemical (accumulation of VLFA) diagnostic markers.

Addison Disease↗
PubMed1981

Susceptibility of multiply antibiotic-resistant pneumococci to the new beta-lactam drugs and rosaramicin.

Eight new beta-lactam antibiotics and rosaramicin were tested for their activity against penicillin-susceptible and multiply resistant pneumococci. All of the beta-lactam drugs were 50- to 150-fold less active against the drug-resistant pneumococci when compared with their activity against penicillin-susceptible pneumococci. Rosaramicin activity was similar to that of erythromycin.

Anti-Bacterial Agents↗
PubMed1979

Di-, mono- and nonphytanyl triglycerides in the serum: a sensitive parameter of the phytanic acid accumulation in Refsum's disease.

The relative proportions of the diphytanyl, monophytanyl and nonphytanyl triglycerides ("triglyceride pattern"), the phytanic acid content of the triglycerides and the phytanic acid levels in the serum of 3 patients with Refsum's disease (heredopathia atactica polyneuritiformis, phytanic acid storage disease) were estimated by thin-layer chromatography, densitometry and gas chromatography, respectively. The individual triglyceride patterns were clearly dependent on the phytanic acid content of the triglycerides: the more phytanic acid in the triglycerides, the higher the percentage of the diphytanyl and the lower the percentage of the nonphytanyl triglycerides. The monophytanyl triglycerides were also related to the phytanic acid content of the triglycerides, although in a complex manner. The triglyceride pattern can be taken as a parameter of the phytanic acid accumulation in Refsum's disease to be at least as sensitive as the serum phytanic acid level in the biochemical evaluation of the efficiency of the dietary treatment.

Eicosanoic Acids↗