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Biomedical subjects

C Meier

Publications and source records attributed to C Meier.

At least 163 records · Page 9Linked to original sources
PubMed1984

Nemaline myopathy appearing in adults as cardiomyopathy. A clinicopathologic study.

We examined a 29-year-old woman with nemaline myopathy that appeared as cardiomyopathy. Clinical examination showed dilated cardiomyopathy, but no neuromuscular abnormalities of the skeletal muscles. Electromyography showed neither neurogenic nor myopathic abnormalities. A biopsy specimen from the quadripecs muscle showed typical nemaline bodies in about 50% of the muscle fibers. The patient died six months later of biventricular heart insufficiency. Autopsy revealed nemaline bodies in the working and conducting tissues of the myocardium. Earlier, the patient's mother and one of her sisters died unexplained, sudden deaths at the ages of 47 and 37 years, respectively. Sections of the myocardium taken from the sister at autopsy were available, and also disclosed nemaline bodies after restaining with trichrome.

Adult↗
PubMed1984

Polyneuropathy in Waldenström's macroglobulinaemia: reduction of endoneurial IgM-deposits after treatment with chlorambucil and plasmapheresis.

A case of progressive polyneuropathy associated with Waldenström's macroglobulinaemia is reported. A monoclonal IgM-lambda gradient was detected in the serum and cerebro-spinal fluid. By electro-immunoblot analysis antibodies against myelin-associated glycoprotein were found in the serum and cerebro-spinal fluid. The motor and sensory conduction velocities of several peripheral nerves were markedly decreased, and examination of visual evoked potentials (VEPs) revealed pathological latencies. Sural nerve biopsies before and after treatment with chlorambucil and plasmapheresis showed nerve fibre loss and demyelination. In the pre-treatment biopsy, heavy accumulations of filamentous material were found which stained positively for IgM by immuno-cytochemistry. Such accumulations had disappeared in a biopsy performed after treatment. The morphological findings were correlated with an improvement of clinical and electro-physiological findings.

Chlorambucil↗
PubMed1984

Demyelinating polyneuropathy associated with monoclonal IgM-paraproteinaemia. Histological, ultrastructural and immunocytochemical studies.

Histological, ultrastructural and immunocytochemical findings of sural nerve biopsies from 2 patients with monoclonal IgM-paraproteinaemia are presented. In both cases the pathological IgM antibodies reacted with a myelin antigen which was identified as myelin-associated glycoprotein (MAG) by immunoelectroblot . Histology and electron microscopy showed typical features of a chronic demyelinating neuropathy with accompanying axonal degeneration. Immunohistochemical studies demonstrated IgM in the vicinity of endoneurial vessels and on some of the myelinated fibres. The localisation of IgM on the myelin sheath showed a typical pattern, which was similar to that found in binding studies with the patients sera on control nerves. It resembled the characteristic immunocytochemical staining pattern of MAG. Binding studies with the patients' sera on human and canine CNS material exhibited a clear labelling of white matter and certain, as yet unidentified structures within the cerebral and cerebellar cortex. In mixed glial cell cultures, the sera of both patients bound specifically to oligodendrocytes. Our observations are interpreted as immunohistochemical evidence that the anti-MAG antibodies of the patients' sera had bound to their antigenic target in the peripheral nerves. Because the antibodies clearly react to central myelin, oligodendrocytes and other not yet identified cortical structures, CNS involvement in such disorders should be considered.

Aged↗
PubMed1984

Tetrodotoxin-resistant release of 3H-noradrenaline from the mouse vas deferens by high intensity electrical stimulation.

Vasa deferentia of mice were preincubated with 3H-noradrenaline and then superfused with a medium containing cocaine 10 microM and phentolamine 30 microM. The tetrodotoxin-resistant outflow of tritium evoked by high intensity electrical field stimulation (0.5 Hz, 200 mA current strength, 2 ms pulse width) was studied and, in some experiments, compared with the tetrodotoxin-sensitive outflow evoked by low intensity electrical field stimulation (0.5 Hz, 50 mA, 1 ms). In contrast to the outflow evoked by low intensity stimulation, the outflow evoked by high intensity stimulation was increased in Na+-free medium, and was only partly dependent on the external Ca2+ concentration. The Ca2+-dependent fraction consisted mainly of 3H-noradrenaline. Again, in contrast to the outflow caused by stimulation at low intensity, that caused by stimulation at high intensity was not reduced by Mg2+ 20 mM, Co2+ 5 mM or normorphine 40 or 100 microM, and was not enhanced by tetraethylammonium 5 mM or 4-aminopyridine 1 mM. It is concluded that high intensity electrical stimulation elicits a tetrodotoxin-resistant, calcium-dependent release of noradrenaline which differs in mechanism from the release elicited by action potentials.

4-Aminopyridine↗
PubMed1984

Neurologic disorder of vitamin E deficiency in acquired intestinal malabsorption.

Fifteen years after onset of a malabsorption syndrome, a 49-year-old man had sensory and oculomotor disorder with marked vitamin E deficiency. After 6 months of treatment with high parenteral doses of vitamin E, the neurologic signs slowly receded, but the patient died of gastrointestinal hemorrhage. Autopsy and sural nerve biopsy showed the changes in both central and peripheral nerves; these changes are considered characteristic of vitamin E deficiency.

Adult↗
PubMed1984

[Polyneuropathies and gammapathies: a form with antiglycoprotein MAG antibodies].

Two cases of polyneuropathy with IgM gammopathy are reported. Myelin associated glycoprotein is the antigen for the monoclonal antibody in both of these patients. The same antigenic specificity has now been identified in other patients having a paraproteinemia with a polyneuropathy. These findings suggest the existence of a new syndrome characterized by: a) a primary demyelinating neuropathy; b) a monoclonal IgM antibody to a specific glycoprotein component of myelin, referred to as myelin associated glycoprotein (MAG). As in other humorally mediated autoimmune diseases, it is suggested that demyelination is caused by circulating anti-MAG antibodies. The role of immunosuppressive drugs and plasmapheresis is discussed.

Aged↗
PubMed1983

The central-peripheral transition zone of cervical spinal nerve roots in Jimpy mutant and normal mice. Light- and electron-microscopic study.

Comparative morphological and ultrastructural investigations on the cervical dorsal and ventral central-peripheral transition zones (CPTZs) of Jimpys and control mice have been performed at early and advanced myelination stages. After postnatal development a characteristic cone-shaped glial outgrowth extends into the proximal part of the dorsal roots, while the ventral roots exhibit short Schwann cell and peripheral nervous tissue invaginations into the spinal cord at the ventral root-spinal cord junction in both animal groups. In Jimpys, although there is marked central myelin deficiency and absence of oligodendroglial development on the CNS side, the normal general aspect of the CPTZs is maintained. Previously postulated astrocytic and neuroaxonal abnormalities in the mutants do not alter the central-peripheral borderline, and Schwann cell migration from the spinal nerve roots into the cord does not occur.

Animals↗
PubMed1983

Hereditary motor sensory neuropathies in childhood.

Clinical data on 24 patients with hereditary motor sensory neuropathies, with onset in the paediatric period, and of their relatives, is reported. Electrophysiological studies were done in all patients and in 15 relatives. The patients were divided into two groups (Types I and II) and their hereditary trait was determined. In 11 patients a sural nerve biopsy was performed and revealed different patterns of histological alterations. The nerve biopsy always confirmed the value of conduction velocity in distinguishing between Types I and II. A genetic discordance was observed, both in regard to the phenotype and the conduction velocity, and there was increased slowing of the conduction velocity as individuals grew older. Thus the classification of these disorders in childhood can be particularly difficult. The rôle of sural nerve biopsy is discussed.

Adolescent↗
PubMed1983

Demyelinating neuropathy and monoclonal IgM antibody to myelin-associated glycoprotein.

We studied a patient with demyelinating neuropathy and monoclonal IgM kappa antibody to the major myelin-associated glycoprotein (MAG). Binding of this monoclonal antibody to the myelin antigen was demonstrated by immunoelectroblot. Binding to MAG seemed to be specific, because it was completely inhibited by MAG isolated from human myelin. Immunostaining was observed with MAG from CNS and peripheral nervous system myelin.

Antibodies, Monoclonal↗
PubMed1983

[Long-term EEG course study in patients with Creutzfeldt-Jacob disease].

In a patient with Creutzfeldt-Jakob disease subsequently confirmed by autopsy, 34 EEGs were carried out in a 14 months period. 6 weeks after the beginning of the prodromal stage of the disease a triphasic, periodic activity was recorded for the first time. This activity was maximally in evidence at 15th week. A synchrony occurring between repetitive complexes and myoclonic jerks could between repetitive complexes and myoclonic jerks could only be observed during one recording. From the 5th month onwards the intensity of the periodic EEG pattern, seen longitudinally, gradually decreased. In the time-span from the 10th to the 13th month considerable fluctuations of periodic activity were found, this during the course of one as well as between different EEG recordings. These could reach from a pronounced typical pattern to complete disappearance of periodic triphasic complexes. As possible causes for these fluctuations, we discussed a variable driving by the subcortical pacemaker as well as a decreased capability of cerebral cortex--gradually loosing so many neurons--to react to subcortical stimuli. From the 14th month onwards the periodic activity no longer could be put in evidence. In the last EEG recorded 3 days before death isoelectric periods alternated with paroxysmal delta waves respectively sharp and slow wave complexes.

Cerebral Cortex↗
PubMed1982

Non-competitive interaction between normorphine and calcium on the release of noradrenaline.

The interaction between calcium and magnesium or normorphine was studied on the electrically evoked outflow of previously incorporated [3H]noradrenaline in the isolated mouse vas deferens. The stimulation-evoked outflow of [3H]noradrenaline increased with increasing concentrations of Ca2+ ions in the medium. Mg2+ counteracted the effect of Ca2+ in a manner compatible with competitive antagonism. In contrast, normorphine interfered with the effect of Ca2+ non-competitively, thus suggesting that its mode of action differed from that of Mg2+.

Animals↗