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Biomedical subjects

R Heipertz

Publications and source records attributed to R Heipertz.

At least 19 recordsLinked to original sources
PubMed1981

A rapid gas-chromatographic method for the quantitative determination of clomethiazole in human serum.

A rapid method without elaborate instrumentation for the determination of clomethiazole serum concentrations is described. This method is suitable for a routine clinical laboratory, and the results, from 37 patients investigated by this method which are presented, are comparable to results described by other authors. A relationship between dose ingested and serum concentration can be established. The determination of clomethiazole serum concentrations is useful as an adjunct to overall clinical assessment, and helpful in establishing a suitable dosage regimen of clomethiazole for the individual patient; it can also be used to verify suspected clomethiazole abuse or overdose.

Adult↗
PubMed1980

Cerebrospinal fluid lipids in demyelinating disease. I. Sphingolipids.

The lipid composition of CSF, serum and lymphocytes from patients without demyelinating disease (controls) as well as from patients with acute and chronic MS is analyzed. Individual lipid fractions are isolated by TLC and their fatty acid composition determined. Lipid and fatty acid composition of normal CSF resembles the results found in lymphocytes and it is deduced that CSF lipids are derived mainly from lymphocytes rather than white matter (myelin) or serum. There is an increase of CSF sphingomyelin in patients with acute MS (showing pleocytosis) which is apparently derived from disintegrated lymphocytes; there is also an increase of linoleic acid concentration which could come from serum because of dysfunction of the blood-brain barrier. The role of the CNS in contributing to CSF lipids is considered negligible both in controls and in patients with demyelination.

Demyelinating Diseases↗
PubMed1980

Cerebrospinal fluid lipids in demyelinating disease. II. Linoleic acid as an index of impaired blood-CSF barrier.

The absolute linoleic acid concentration in CSF was determined and the findings of MS patients (n = 10) and controls (n = 12) were compared. The linoleic acid content of control CSF (1.6 +/- 0.8 nMol/ml) is considerably lower than the corresponding serum value (2.5--4.1 muMol/ml). Although CSF from MS patients contains a significantly higher linoleic acid concentration than controls the close correlation between CSF linoleic acid and CSF albumin is maintained. The high CSF concentration of cholesterol esters rich in linoleic acid, which are abundant in serum but represent only traces in CNS lipids, points towards an impaired BBB function as the cause of CSF linoleic increase. We are able to show that both albumin and linoleic acid are suitable as "serum markers" and also as reference parameters for the overproportional IgG concentration in the CSF of MS patients. On the basis of these results it can be assumed that changes in CSF linoleic acid content are an expression of dysfunction of the blood-CSF barrier in MS and not, as had previously been postulated, the result of altered myelin metabolism.

Adult↗
PubMed1980

Adult metachromatic leukodystrophy. III. Clinical course, final stages and first biochemical results.

Continuing the previously published clinical development of a case of adult metachromatic leukodystrophy (MLD), we now describe the terminal phase and death (at 46 years of age) of our patient. The final phase was characterized clinically by progression of generalized peripheral neuropathy, advanced extrapyramidal and pyramidal tract symptomatology, dementia and brainstem dysfunction. First biochemical results show a moderate relative increase (3- to 5-fold) of sulfatides in the frontal lobe white matter but not in the cortex. The analysis of fatty acids in total lipid extract shows a decrease of long-chained fatty acids in favor of short-chained fatty acids, this change is more pronounced in white matter in the cortex. The clinical course and biochemical results are discussed in relation to previous cases analyzed by us. Epidemiological aspects especially emphasize routine serach for MLD amongst patients with neuropsychiatric symptomatology showing unusual psychoses, presenile dementias or unspecific disturbance of motor coordination possibly with electroneurographic evidence of peripheral neuropathy.

Age Factors↗
PubMed1980

Adult metachromatic leukodystrophy. IV. Ultrastructural studies on the central and peripheral nervous system.

Ultrastructural studies on the central and peripheral nervous system of 2 patients with adult onset metachromatic leukodystrophy (MLD), dead at the ages of 46 and 51 years, showed MLD-specific inclusions, tufaceous and prismatic structures, a wide spectrum of membranous arrangements within lysosomal residual bodies, and the intimate admixture of sulfatides and other membranous material with lipopigments. Oligodendrocytes and Schwann cells were foremost affected but membranous inclusions could also be verified in neuronal perikarya and astrocytes. The varying ultrastructural spectrum of lysosomal residual bodies in adult onset MLD and the association with lipopigments, chiefly in nerve cells, exceed the fine structural observations on late infantile and juvenile MLD and may reflect morphological differences between these subtypes of MLD that are also known from clinical and biochemical observations.

Adipose Tissue↗
PubMed1979

[Water intoxication and brain edema in psychogenic polydipsia (author's transl)].

A case of psychogenic polydipsia is presented that showed psychic decompensation and compulsive drinking under the acute stress of an imminent operation for ovarian cyst. Without any indication of an underlying organic disease process the patient developed acute water intoxication due to the uncontrolled intake of water from the tap, this caused hyponatremia, brain edema, coma and status epilepticus. The physiology of water intoxication is reviewed in relation to this case, which is also remarkable for the acute onset and the shortness of the polydipsic state.

Adult↗
PubMed1979

Basic findings and current developments in sphingolipidoses.

Sphingolipidoses are caused by recessively inherited deficiencies of lysosomal hydrolases. The clinical backgrounds of and current biochemical and genetic approaches to the different forms and variants of gangliosidoses, trihexosylceramidosis (Fabry's disease), galactosylceramidosis (Krabbe's disease), sulfatidoses (metachromatic leukodystrophies), glucosylceramidosis (Gaucher's disease), sphingomyelinoses (Niemann-Pick disease) and ceramidosis (Farber's disease) are presented.

Fabry Disease↗
PubMed1979

The activity of 2',3'-cyclic nucleotide 3'-phosphohydrolase in human cerebrospinal fluid.

A method for the determination of cyclic nucleotide phosphatase (CNP) activity in cerebrospinal fluid is presented. In normal CSF the activity of CNP is very low. Comparing CSF from patients with multiple sclerosis to control CSF no significant difference is found, although a small proportion of MS patients has an elevated has an elevated CNP activity in their CSF. It is postulated that similar to other substances the CNP activity in CSF probably does not derive from the central nervous system.

Central Nervous System↗
PubMed1979

Primidone metabolism in renal insufficiency and acute intoxication.

Primidone (PRIM) is metabolized into phenobarbital (PB) and phenylethylmalonamide (PEMA). During anticonvulsant therapy with PRIM under normal conditions PB represents by fat the largest portion of the total concentration of all three components (PRIM + PB + PEMA). In combined therapy with diphenylhydantoin (DPH), and during chronic PRIM overdosage, the relative concentration of PB is even higher. A case of renal insufficiency while on PRIM therapy and a case of acute PRIM intoxication are presented. In both cases PRIM and PEMA are elevated while PB is relatively low. The mechanisms involved in this phenomenon are discussed. Excluding young children with chronic PRIM overdosage, and the endogenous and exogenous intoxication described here, a relative PB concentration below 40% indicates a lack of patient compliance if a steady treatment schedule has been maintained for at least 3 weeks.

Acute Disease↗
PubMed1979

Magnesium and inorganic phosphate content in CSF related to blood-brain barrier function in neurological disease.

In normal controls and in a large number of neurological patients divided into certain disease groups both Mg and PO4 were determined in cerebrospinal fluid (CSF) and serum. For both Mg and PO4 there was a marked concentration gradient between CSF and serum in normals where Mg was higher and PO4 content lower in CSF. Comparison of CSF values with serum values of patients showed pathological changes only in CSF, serum values always being within the control range. A number of disease processes associated with a disturbance of blood-brain barrier (BBB) function such as inflammatory CNS disease or CNS tumors showed significant alterations of PO4 concentrations in CSF which are interpreted as an approximation of serum values. A similar decrease of Mg did not reach statistical significance. Both Mg and PO4 in CSF showed a correlation with CSF protein concentrations, but no relationship with cells in CSF. Patients with cerebrosvascular disease were not significantly different from controls as regards their Mg and PO4 in CSF, but a small subgroup consisting of patients with an intracranial hemorrhage showed elevation of both Mg and PO4 which could signify cell necrosis rather than BBB dysfunction. Patients with disc protrusion or peripheral neuropathy did not demonstrate any abnormality of CSF Mg and PO4. In the multiple sclerosis group individual patients had elevated CSF concentrations of PO4 but the group as a whole is not different from the controls.

Blood-Brain Barrier↗
PubMed1979

Determination of IgG subgroups in cerebrospinal fluid of multiple sclerosis patients and others.

IgG subgroups (IgG1, IgG2, IgG3, IgG4) were determined by radioimmunoassay (RIA) in cerebrospinal fluid (CSF) of controls, multiple sclerosis (MS), infectious diseases (ID) and other neurological diseases (OND). The proportion of IgG1 in the total IgG subgroup concentration was significantly higher in the MS group compared to the other groups while the IgG2 proportion was significantly lower; IgG3 and IgG4 did not show any consistent change. The inverse relationship between IgG1 and IgG2 was similar in all diagnostic groups: high concentration of IgG1 was associated with low concentrations of IgG2 and vice versa. Patients with a high relative concentration of IgG1 in their CSF have a seven to eight times higher statistical risk to be suffering from MS than ID or OND. In the MS group only the IgG1 concentration correlated with the total IgG concentration determined by radial immunodiffusion, while in controls, ID and OND each IgG subgroup correlated significantly with the IgG concentration. This demonstrates that in MS a selective increase of IgG1 subgroup is mainly responsible for the increase of total IgG, while all subgroups are involved in OND and ID showing an increased total IgG concentration.

Albumins↗
PubMed1979

Urinary metabolites of clomethiazole. Detection and structural analysis by gas chromatography-mass spectrometry.

As the result of a renewed extensive investigation of clomethiazole (Distraneurin) metabolism five previously unknown metabolites could be isolated from human urine. Their structures were elucidated by mass spectrometry. Whereas previous investigations on the metabolism of clomethiazole had demonstrated changes only of the ethyl group, we now found metabolites attached to the methyl group, too. The newly isolated compounds 5-(1-hydroxy-2-chloroethyl)-4-methylthiazole (9) and 5-(2-hydroxyethyl)-4-thiazole carboxylic acid lactone (5) were found to be more abundant in human urine than 4-methyl-5-thiazole acetic acid previously considered as the main metabolite.

Biotransformation↗
PubMed1978

Interaction of nitrofurantoin with diphenylhydantoin.

Left-sided motor seizures in a patient with an operated brain tumor were controlled with 300 mg/d DPH. The introduction of antimicrobial therapy with nitrofurantoin caused a fall of serum DPH levels and the recurrence of seizures, at the same time serum gammaGT values were increased. These changes were reversible after nitrofurantoin treatment was terminated. Both increased DPH metabolism or impaired absorption could be responsible for this effect. The concomittant increase of gammaGT could be interpreted as indirect evidence of hepatic enzyme induction with increased metabolism of DPH. It is important to note the possibility of such interaction so that better anticonvulsant control can be achieved.

Humans↗
PubMed1978

Determination of immunoglobulin content of CSF based on light chain characteristics.

The immunoglobulin light chain (types kappa and lambda) content of normal cerebrospinal fluid is similar to that of normal serum. In inflammatory diseases of the central nervous system a shift in the k/l ratio in comparison to serum values, usually a relative increase of Igk, can be observed. This increase of the k/l ratio, though not specific for any disease, is most commonly found in multiple sclerosis. There is no correlation between the IgG content and the k/l ratio. The methods described here measure bound and free light chains simultaneously. The calculations show that free light chains are present in both normal and inflammatory CSF and that they appear to be of polyclonal origin. The detection of light chain abnormalities in CSF can be taken as an indicator of endogenous immunoglobulin production in the CNS and is of significance for the diagnosis of inflammatory CNS processes, especially when other signs of endogenous immunoglobulin production are absent.

Central Nervous System Diseases↗
PubMed1978

Determination of k/l immunoglobulin light chain ratios in CSF from patients with multiple sclerosis and other neurological diseases.

Using antisera against Bence-Jones protein, the concentration of light chains type k and l can be determined in CSF. The calculation of the ratio of type k to type I light chains in CSF represents a sensitive measure for the evaluation of immunological processes involving the CNS. Our results demonstrate that an increase k/l ratio is encountered in 48% of CSF specimen from multiple sclerosis (MS) patients, but also in 50% from patients with other inflammatory diseases involving the CNS, in contrast to only 18% from other neurological diseases. In none of the MS or inflammatory cases is the altered k/l ratio the only indicator of a CNS inflammation, most commonly it is accompanied by an overproportional CSF-IgG elevation (increased QG ratio), an increased cell count or both. For these reasons determination of CSF k/l ratios is helpful in the differentiation of MS and other neurological diseases, but not for the differentiation of other inflammatory CNS diseases from MS.

Central Nervous System Diseases↗