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  • 1
    ISSN: 1433-0407
    Keywords: Schlüsselwörter Guillain-Barré-Syndrom ; N.-suralis-Biospie ; EMG ; Key words Guillain-Barré syndrome ; N. suralis biopsy ; EMG
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary 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.
    Notes: Zusammenfassung Wir beobachteten 3 Patientinnen mit schwer verlaufendem Guillain-Barré-Syndrom (GBS). Bei klinischer Untersuchung waren keine sensiblen Störungen zu eruieren. EMG und motorische Elektroneurographie sprachen für eine axonale Läsion. Bei 2 Patientinnen bestand ein kompletter Leitungsblock motorischer Fasern, dabei war im EMG keine Willküraktivität nachweisbar. Sensible Nervenleitgeschwindigkeiten und kortikale Medianus-SEP blieben auch im Verlauf unauffällig. Die Biopsie des N. suralis konnte aber jeweils eine eindeutige leichte Beteiligung sensibler Nervenfasern, z. T. in Form von primärer Demyelinisierung, belegen. Somit handelt es sich bei diesen Fällen trotz der klinischen und elektrophysiologischen Befunde um gemischt motorisch-sensible Läsionen. Obwohl es im Verlauf zu einer kompletten Denervierung der Extremitätenmuskulatur mit Tetraparalyse kam, sind sie offenbar eher einer sekundär axonalen Variante zuzurechnen. Im Gegensatz zu positiven Literaturberichten blieb die immunmodulierende Therapie in allen 3 Fällen wirkungslos.
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  • 2
    ISSN: 1432-0533
    Keywords: Congenital nemaline myopathy ; Fiber-type disproportion ; Myofibrillar abnormalities ; Defective myogenesis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary 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.
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 80 (1990), S. 439-447 
    ISSN: 1432-0533
    Keywords: Unmyelinated nerve fibers ; Sural nerve biopsies ; Chronic inflammatory demyelinating polyneuropathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary 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 (≥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.
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 88 (1994), S. 174-179 
    ISSN: 1432-0533
    Keywords: Key words: Greater auricular nerve ; Unmyelinated nerve fibers ; Sural nerve ; Comparative morphometry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract 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.
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 88 (1994), S. 174-179 
    ISSN: 1432-0533
    Keywords: Greater auricular nerve ; Unmyelinated nerve fibers ; Sural nerve ; Comparative morphometry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract 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.
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 55 (1981), S. 307-318 
    ISSN: 1432-0533
    Keywords: Diphtheritic polyneuropathy ; Muscle biopsy ; Intramuscular nerves ; Electron microscopy ; Histochemistry ; Diphtherie-Polyneuropathie ; Muskelbiopsie ; intramuskuläre Nerven ; Elektronenmikroskopie ; Histochemie
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Biopsien aus Extremitätenmuskeln von vier Kranken mit schwerer tetraplegischer Form der Diphtherie-Polyneuropathie konnten nahe dem Höhepunkt der Erkrankung gewonnen und mit modernen Methoden einschließlich Histochemie, Elektronenmikroskopie und morphometrischer Verfahren untersucht werden. Vergleichbare Studien wurden bisher nicht veröffentlicht. Die Muskulatur bot vorwiegend neurogene Veränderungen mit disseminierten angulierten Fasern, eine besonders die Typ-2B-Fasern betreffende, mehr generaliierte Atrophie sowie Targetoidherde oder „Cores” in Typ-1-Fasern. Darüber hinaus fanden sich eindeutige myogene Veränderungen mit unterschiedlichen Degenerationsphänomenen bis zur totalen Nekrose, offenbar — wie am Herzmuskel —toxischer Herkunft. Das neurogen-myogene Mischbild entsprach den elektromyographischen Befunden. Die intramuskulären Gefäße waren unauffällig bis auf vereinzelte perivaskuläre, vorwiegend mononukleäre Zellinfiltrate mit reichlich zerebriformen lymphoiden Zellen, wahrscheinlich T-Lymphozyten. An 11 intramuskulären Nerven und zwei motorischen Endplatten waren eindeutig auf die Diphtherie zu beziehende Veränderungen nicht aufzudecken. Dies kann als Hinweis auf eine vorwiegend proximale Entmarkung bei der Diphtherie-Polyneuropathie des Menschen gedeutet werden, die damit eher der experimentellen Form des Kaninchens als der des Meerschweinchens entspricht.
    Notes: Summary Mucle 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 2B 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.
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  • 7
    ISSN: 1432-0533
    Keywords: HIV-related relapsing polyneuropathy ; Intrathecal HIV antibodies ; Nerve biopsy ; Electron microscopy ; Schwann cell morphology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary 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.
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  • 8
    ISSN: 1432-0533
    Keywords: Unmyelinated axons ; Sensory neuropathy ; Thalidomide ; Aging ; Senile peripheral nerves
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Sural nerve biopsies of four patients, aged 54–76 years, with a predominantly sensory type of neuropathy following high dosages of thalidomide were examined by light and electron microscopy. The present study includes a qualitative and quantitative evaluation of unmyelinated nerve fibers. Despite severe neuropathy, increased numbers of small unmyelinated axons per endoncurial area were noted in all patients. This numerical increase appeared to be independent of aging, since it was not seen in two senile controls, studied at the age of 83 and 88 years. The increase in the endoneurial density of unmyelinated axons, especially of small sized fibers, is likely to be related to regeneration following degeneration of unmyelinated axons although endoneurial shrinkage secondary to loss of large myelinated fibers could have caused an additional increase in the number of axons per endoneurial area. Axonal sprouting, despite degeneration of large numbers of myelinated and unmyelinated fibers, appears to be consistent with some of the characteristic clinical features of thalidomide neuropathy such as paresthesias, hyperesthesia for pain and temperature, and disturbances of autonomic functions. On the other hand, a variable number of empty Schwann cells (bands of Büngner) and pockets at the surface of many Schwann cells noted in the four patients with neuropathy were also seen in both senile controls with no signs of neuropathy. Thus, it is obvious that pockets and empty Schwann cells may be related to aging or other causes of slow axonal wasting with Schwann cell proliferation and are not necessarily associated with clinically manifest neuropathy.
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  • 9
    ISSN: 1432-1459
    Keywords: Tangier disease ; Polyneuropathy ; Ultrastructure ; Biopsy findings
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary 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 supected 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.
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