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Biomedical subjects

E Gibbels

Publications and source records attributed to E Gibbels.

At least 19 recordsLinked to original sources
PubMed1996

[Clinically pure motor variant of Guillain-Barré polyneuritis. Report of 3 cases].

We report three patients with severe Guillain-Barré syndrome (GBS). On clinical examination, no sensory deficit was elicited. Electromyography (EMG) and motor nerve conduction velocity (NCV) studies were indicative of an axonal lesion. In two patients, we found total peripheral conduction block without volitional EMG activity. Sensory NCV and cortical median nerve evoked potentials remained normal. Sural nerve biopsy revealed unequivocal alterations of sensory nerve fibers, some in the form of primary demyelination. Therefore, these cases must bei classified as combined motor-sensory syndromes despite the clinical and electrophysiological findings. Although total denervation of the extremity muscles occurred, causing tetraparalysis, this manifestation appears to be attributable to the secondary axonal variant. In contrast to positive reports in literature, immunomodulatory treatment proved ineffective in all three cases.

Aged↗
PubMed1994

Unmyelinated fibers in human greater auricular and sural nerves: a comparative morphometric study.

Since normal structural details of human greater auricular nerve (GAN) have not as yet been studied with modern techniques, light and electron microscopic findings of seven presumably normal GANs, obtained from five patients during radical neck dissection, were compared with those of normal sural nerves (SNs). In GANs there was a tendency to higher densities per mm2 and a larger number of small-diameter fibers in myelinated fibers (MFs) and unmyelinated fibers (UFs) without obvious signs of de- or regeneration. UF histograms were unimodal in both groups, with mean UF diameters being somewhat smaller in GANS than in SNs. Schwann cell complexes containing several or even numerous UFs were more frequent in GANs than in SNs. In GANs, UF often occurred focally in great numbers within large Schwann cell complexes (polyaxonal complexes), not commonly seen in normal SNs. It is concluded that these structural peculiarities in GANs reflect natural conditions in short sensory nerves irrespective of any specific function.

Adult↗
PubMed1992

Follow-up studies in a case of unusual congenital myopathy, suggestive of nemaline type.

A 20-month-old boy--offspring of consanguinous parents, whose mother presumably had subclinical myopathy--presented with clinical signs of congenital non-progressive myopathy, neurogenic-myogenic electromyographic findings and normal motor conduction velocity. Biopsy of quadriceps muscle showed fiber-type disproportion with hypotrophic type 1, hypertrophic 2A and absent 2B fibers. Subsarcolemmal segmental foci of abnormally, in part regularly arranged bundles of mostly thin myofilaments were found in 13% of hypotrophic type 1 fibers. Rods were seen in only 1 fiber out of 20 tissue blocks. Reexamination 6 years later revealed slightly increased muscle force, myopathic EMG pattern and borderline motor and sensory nerve conduction velocities. Biopsy specimen from deltoid muscle consisted of untypable fibers of varying diameters with jagged Z-lines and increased variability of myofibrillar diameters. Multiple rods were present in 1% of the fibers, the formerly seen segmental foci in 0.1% only. Several intramuscular nerves were normal. The case contributes some new features to the spectrum of congenital myopathies of the nemaline type and suggests different stages of arrested maturation of type 1 fibers at least in this particular case.

Biopsy↗
PubMed1992

Natural course of acute and chronic monophasic inflammatory demyelinating polyneuropathies (IDP). A retrospective analysis of 266 cases.

The present study analyses one of the largest IDP series published as yet: 266 cases, hospitalised between 1950 and 1983, conventionally treated and uniformly supervised. There was GBS in 84%, chronic IDP in 13%, polyneuritis cranialis in 1.5%, Miller Fisher and predominantly sensory neuropathy in 0.8% each. Numerous features including preceding events, course and outcome have been investigated. Latencies between onset and maximal deficit lacked (expected) bimodality. Sensory involvement was severe, mortality (18%) and maximal disability (20% requiring ventilation) high, autonomic dysfunction (71%) prominent. Statistical comparison of various features confirmed old age, rapid onset and need for ventilation as unfavorable predicting factors. However, preceding infection, bulbar paralysis and onset of paresis in proximal muscles evolved as unfavorable prognostic features as well.

Adolescent↗
PubMed1990

Unmyelinated fibers in sural nerve biopsies of chronic inflammatory demyelinating polyneuropathy.

Seven patients aged 29 to 76 years with various clinical subtypes of chronic inflammatory demyelinating polyneuropathy (CIDP) were investigated. Sural nerve biopsies were performed between 7 months and 19 years after onset of disease. Quantitative electron microscopy revealed involvement of primary unmyelinated fibers (UF) in all cases. When compared with age-matched controls from the literature and two controls of our own, there was an increase of degenerating primary UF in all cases, a definite decrease of density per mm2 or number per nerve after subtraction of regenerates of myelinated and unmyelinated fibers in five cases, an increase of denervated Schwann cell complexes of the unmyelinated type in three cases, and an increased incidence of a high ratio (greater than or equal to 3) of primary UF per Schwann cell complex in five cases. Presumably due to the small number and heterogeneity of cases, the results did not correlate with type and duration of CIDP, but were obviously influenced by the degree of demyelination. The possible causes of UF damage in CIDP are discussed.

Adult↗
PubMed1990

Role of therapeutic plasmapheresis in chronic inflammatory demyelinating polyneuropathy.

Therapeutic plasmapheresis is an effective therapy in the management of CIDP. A varying percentage of patients, approximately 30 to 60%, may benefit from the treatment. The optimal frequency and volume of PE need to be clarified, but, taking into account the heterogeneity of the disease, a too rigid approach should be avoided. According to our experience, neither morphological findings on sural nerve biopsy, nor conduction slowing, conduction block, or the amount of spontaneous activity on needle electromyography in a weak muscle correlated clearly with the later outcome of PE. Possibly our patient number is too small to provide any statistically significant predictor of outcome. In our opinion it is essential to combine plasmapheresis with effective immunosuppression to avoid a rebound with overshooting synthesis of putative pathogenic antibodies or factors. Finally, IA with T-PVA columns has proven effective in single, case-controlled patients with CIDP. It may be a promising supplement to PE avoiding the need and risks of protein replacement.

Autoimmune Diseases↗
PubMed1989

[Hitler's neurologic disease--differential diagnosis of Parkinson syndrome].

This paper deals with the differential diagnosis of Hitler's Parkinsonian syndrome based upon our recently published results of an analysis of Hitler's motor functions in German news-reels, upon archived materials with notes and comments of Hitler's doctors, various memories, and our own interrogations of eye-witnesses of Hitler's nearest entourage. According to these sources, Hitler's Parkinsonian syndrome developing since mid-1941 cannot be attributed to any of the symptomatic forms of Parkinsonism. In light of family history, age of manifestation and clinical features the assumption of idiopathic Parkinson's disease seems somewhat more plausible than that of postencephalitic Parkinsonism; the genetic variant is less probable. Hitler's neurological pathography is further analysed in respect to an abuse with "Antigas pills" and to some statements in the literature presuming an amphetamine-dependence, a syphilitic manifestation, an epileptic seizure in 1932, and a tremor already present in 1923.

Diagnosis, Differential↗
PubMed1988

Human immunodeficiency virus (HIV)-related chronic relapsing inflammatory demyelinating polyneuropathy with multifocal unusual onion bulbs in sural nerve biopsy. A clinicomorphological study with qualitative and quantitative light and electron microscopy.

This is obviously the first report on a case with a spontaneous sensu strictu relapsing variant of human immunodeficiency virus (HIV)-related polyneuropathy. Its manifestation preceded LAS. Intrathecal HIV-antibodies developed between the most severe third and fourth episode. Analysis of sural nerve biopsy was consistent with a multifocally accentuated chronic inflammatory demyelination, characterized by unusual onion bulb-like Schwann cell formations with irregular voluminous layers, electron density, aggregation of filaments, multiple indented nuclei, and numerous enclosed collagen pockets. A direct or immune-mediated indirect specific influence on Schwann cell morphology by HIV might be discussed. Virus-like particles and ultrastructural markers of HIV were not detectable.

Acquired Immunodeficiency Syndrome↗
PubMed1988

[Chronic polyneuritis of the Guillain-Barré type. Significance of long-term electroneurographic follow-up findings].

Nineteen patients with various forms of chronic polyneuritis of the Guillain-Barré type were attributed by uniform clinical criteria to the different subgroups and their electroneurographic findings followed during the clinical course. The follow-up studies were conducted for 1 to 12 years with a mean of 5.3 years. All patients were treated with steroids and/or Azathioprin, in addition, six patients were subjected to plasma exchange therapy. Of the 13 patients without plasma exchange four demonstrated improvement of electroneurographic parameters, four were unchanged, two deteriorated. The six patients treated with additional plasma exchange showed improvement in four cases and deterioration in two cases. No correlation was found between clinical findings and long-term electroneurographic data. Improvement of electroneurographic data was usually less impressive than the clinical recovery. Following plasma exchange treatment three patients showed remarkable, however only temporary improvement of electroneurographic data which also did not correlate to the long-term outcome. Therefore, the long-term follow-up of electroneurographic findings renders no decisive prognostic statement in chronic polyneuritis.

Adolescent↗
PubMed1988

[Polyneuropathies following administration of tetanus toxoid].

Two cases are reported: one with an asymmetric polyneuropathy involving the left lower extremity, the other with a generalised chronic relapsing-remitting demyelinating polyneuropathy, hitherto not described in this context. The findings are discussed with regard to the relevant literature.

Adult↗
PubMed1987

Myelinated and unmyelinated fibers in sural nerve biopsy of a case with lepromatous leprosy--a quantitative approach.

A 64-year-old Cambodian male with lepromatous leprosy of several months' duration, with only brief previous treatment, had symmetrical patchy loss of pain and thermal sensibility including the lateral foot regions. Quantitative assessment by light and electron microscopy revealed normal counts of myelinated fibers (MF) and an increased number of unmyelinated fibers (UF). The respective abnormal histograms and the qualitative differentiation of UF-associated Schwann cells suggested a combination of segmental demyelination with Wallerian degeneration of MF, and a loss of genuine UF, concomitant with considerable regenerative activity of both fiber populations.

Humans↗
PubMed1986

[Plasma exchange treatment in chronic polyneuritis of the Guillain-Barré type. Experiences with 9 personal cases].

Following a review of pertinent literature, nine cases of chronic polyneuritis are reported, who underwent a total of 21 series of plasma exchange treatment during immunosuppressive medication. The effect of treatment was variable. Four out of 7 patients with relapsing and/or stepwise progressive disease demonstrated reproducible improvement of neurological signs. The three others responded to a lesser degree. Two patients with a chronic progressive course experienced only mild improvement. In general, the reaction to plasma exchange did not correlate with duration and severity of the previous clinical course, reaction to immunosuppressive drugs, or electrophysiological and morphological findings in muscle and nerve biopsies. Moreover, a clear correlation with pathogenic effects of patient-IgG in animal experiments ("passive transfer") was not discernible.

Adolescent↗
PubMed1985

Severe polyneuropathy in Tangier disease mimicking syringomyelia or leprosy. Clinical, biochemical, electrophysiological, and morphological evaluation, including electron microscopy of nerve, muscle, and skin biopsies.

Polyneuropathy in Tangier disease can be divided into three clinical types. The most severe form (type III) with a syringomyelia-like syndrome has been described in three cases only. Here, a fourth case of this type is presented. Because of unusual trophic disturbances even leprosy was suspected. Electrodiagnostic findings, including evoked cerebral potentials in this case, were suggestive of a generalized neuropathy with some degree of primary or secondary demyelination and implied possible impairment of central structures. Sural nerve biopsy, including electron microscopy and quantitative analysis, revealed a predominant reduction of smaller myelinated and unmyelinated fibres. The main morphological feature was the abundance of abnormal non-membrane-bound vacuoles in Schwann cells, mostly of the unmyelinated type, and in some endoneurial fibroblasts, macrophages and perineurial cells. There was no inverse relationship between lipid vacuoles and axons in Schwann cell complexes as suspected by others. An excess of endoneurial collagen as well as an increased fascicular area were obvious. In five skin biopsy specimens of different regions typical vacuoles were noted in Schwann cells, histiocytes, nevus cells, and rarely in perineurial cells.

Adult↗
PubMed1984

Chronic inflammatory polyneuropathy. Reduction of nerve conduction velocities in monkeys by systemic passive transfer of immunoglobulin G.

In chronic (relapsing) inflammatory polyneuropathy (CRIP), successful treatment with plasma exchange has led to the concept of pathogenic humoral factors. In 6 patients with CRIP, 5 of whom improved after plasma exchange, the potential pathogenic role of circulating immunoglobulin (Ig) fractions was tested by applying the systemic passive transfer model to marmoset monkeys. After continuous treatment with intramuscular injections for 2-8 weeks, monkeys injected with the crude immunoglobulin fractions or with purified IgG from 5 of the 6 patients showed a significant and partially reversible reduction of the motor nerve conduction velocity (mean 34%, P less than 0.001) when compared with pre-treatment values. In control animals the reduction was 4%. Morphological examination revealed only minor ultrastructural changes of the myelin sheath. Immunocytochemistry revealed that human IgG was able to cross the blood-nerve barrier. It is concluded that the circulating IgG-fraction of patients with CRIP contains a factor that may contribute to the disordered nerve function after crossing the blood-nerve barrier. It may be the removal of this particular factor which is responsible for the rapid recovery of nerve conduction in patients after plasma exchange.

Animals↗
PubMed1981

[Results of muscle biopsies in diphtheritic polyneuropathy. Light- and electron-microscopic examinations of muscle fibers, intramuscular nerves, motor endplates, and intramuscular vessels].

Muscle biopsies from the lower extremities of four patients with severe tetraplegic form of diphtheritic polyneuropathy were examined by modern techniques including histochemistry, electron microscopy and morphometric procedures. Until now comparable studies have not been published. The biopsies were removed during the acute stage of the polyneuropathy. We found scattered small angulated muscle fibers beside a more generalized slight atrophy predominantly of type 2 B fibers and targetoid-phenomenons or cores in type 1 fibers. Beside this neurogenic pattern there also were, corresponding with the results of electromyography, primary myogenic alterations with different degenerative phenomenons, suspicious of toxic origin as in cardiac muscle. The intramuscular vessels showed no abnormalities except some perivascular predominantly mononuclear cellular reactions with a remarkable number of cerebriform lymphoid cells, probably T-lymphocytes. No specific pathological alterations could be detected in 11 intramuscular nerves and two motor endplates. This may reflect the more proximal demyelination of human peripheral neurons by the diphtheria toxin as found in experimental diphtheria of the rabbit in contrast to the more distal type of the guinea pig.

Adolescent↗