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  • 1
    Electronic Resource
    Electronic Resource
    Glucocorticoid-sensitive hereditary inclusion body myositis (1996)
    Springer
    Journal of neurology 243 (1996), S. 126-130 
    Details
    ISSN: 1432-1459
    Keywords: Inclusion body myositis ; Heredity ; Immunosuppressive therapy ; Morphology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report a hereditary muscle disorder with features of inclusion body myositis (IBM) in two adult sisters with slowly progressive asymmetrical muscle weakness. The findings of light microscopic and ultrastructural investigations of muscle biopsy specimens were consistent with a diagnosis of IBM. Both patients improved and stabilized on immunosuppressive treatment with corticosteroids and azathioprine. This differentiates our patients from other sporadic and familial cases of IBM. Clinical and histological features are described and compared with those of other previously reported families with IBM.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02444002
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  • 2
    Electronic Resource
    Electronic Resource
    Akute Muskelschwäche – Differentialdiagnose neuromuskulärer Erkrankungen und ihre Behandlung (1997)
    Springer
    Intensivmedizin und Notfallmedizin 34 (1997), S. 110-123 
    Details
    ISSN: 1435-1420
    Keywords: Key words Neuromuscular diseases ; Guillain-Barré syndrome ; chronic inflammatory demyelinating polyneuritis ; myasthenia gravis ; Lambert-Eaton syndrome ; poly-/dermatomyositis ; Schlüsselwörter Neuromuskuläre Krankheiten ; Guillain-Barré-Syndrom ; Chronisch entzündliche demyelinisierende Polyneuritis ; Myasthenia gravis ; Lambert-Eaton-Syndrom ; Poly-/Dermatomyositis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Die Übersichtsarbeit befaßt sich mit der Differentialdiagnose und Therapie von Erkrankungen des neuromuskulären Systems. Kardinalsymptom ist eine generalisierte Muskelschwäche, die de novo auftritt oder als krisenhafte Dekompensation einer vorbestehenden Erkrankung entsteht. Typische klinische Befundkonstellationen erlauben die rasche diagnostische Eingrenzung, unterstützt durch wenige gezielte technische Zusatzuntersuchungen. Die Abgrenzung seltenerer Erkrankungen als Ursache einer bedrohlichen Muskelschwäche erfordert eingehendere Untersuchung und besonders ausführliche anamnestische und fremdanamnestische Erhebungen. Die Intensivbehandlung neuromuskulärer Krankheiten zeichnet sich durch einige Besonderheiten aus, aus denen sich Konsequenzen für Monitoring, Sedierung, Schmerztherapie und Beatmung ergeben. Im speziellen Teil wird die intensivmedizinisch relevante Stufentherapie neuromuskulärer Erkrankungen detailliert besprochen.
    Notes: Summary This review deals with the differential diagnosis and treatment of critically ill neuromuscular patients. Acute muscular weakness may be a sign of newly acquired neuromuscular disorders or of an exacerbation of a previously existing disease. Typical features of the more common neuromuscular conditions are presented and the problems of neurocritical care delineated. The appropriate ancillary tests are presented. Intensive care management includes adapted strategies for monitoring, sedation, pain treatment, and artificial respiration as applied to the critically ill neuromuscular patient. Specific treatments for disorders including Myasthenia gravis, the Lambert-Eaton myastenic syndrome, Guillain-Barré syndrome, chronic inflammatory neuropathies and myositis are summarized proposing a step-wise escalation of treatment modalities.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s003900050027
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  • 3
    Electronic Resource
    Electronic Resource
    Gene polymorphism at position –308 of the tumor necrosis factor α promotor is not associated with disease progression in multiple sclerosis patients (1999)
    Springer
    Journal of neurology 246 (1999), S. 949-954 
    Details
    ISSN: 1432-1459
    Keywords: Key words Multiple sclerosis ; Tumor-necrosis factor ; Genetic polymorphism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Tumor necrosis factor-α (TNFα) is a pluripotent proinflammatory cytokine and is thought to play an important role in the inflammatory process of multiple sclerosis (MS). A G→A transition in the TNFα promotor at position –308 (TNF2 allele) has been shown to be associated with increased TNFα production. This study was designed to detect wether the TNF2 allele is associated with disease progression in MS. We examined the TNFα–308 polymorphism with an allelic discrimination PCR to detect the G→A transition in the genomic DNA of 283 MS patients from Germany and in 72 patients with amyotrophic lateral sclerosis (ALS) and 66 with stroke from the same genetic background who served as controls. Disease severity was defined by the progression index (PI) and by progression to the important clinical landmarks of Extended Disability Status Score (EDSS) 3.5 and 6. In addition, we evaluated the TNFα mRNA expression in whole blood with quantitative PCR. No differences were found between the presence of the TNF2 allele in MS, ALS, or stroke patients. Among the MS patients the TNF2 allele was not associated with a certain disease course. No association was found between the accumulation of neurological deficits and progression to clinical landmarks. Although MS patients with the TNF2 allele tended to progress more rapidly from EDSS 3.5 to EDSS 6 this difference was nonsignificant (P = 0.2). Nevertheless, we observed significantly higher TNFα mRNA expression in blood cells of stable patients carrying the TNF2-allele in comparison to the group with the wild type (P = 0.024). To examine the effect of genetic background we examined the DNA of 60 MS patients and 20 healthy controls in a Cypriot population of Greek origin. There was a significantly lower frequency of the TNF2 allele in the Cyprus population than in Germans (P = 0.01). No significant differences were found between the frequencies of the TNF2 allele in Cypriot MS patients and controls. Although the TNF2 allele is associated with higher TNFα mRNA baseline levels, our data indicate that this allele appears not to contribute to MS susceptibility or severity. In addition our data demonstrate that the TNFα–308 polymorphism is segregated differentially in two European populations of different genetic origin.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004150050489
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  • 4
    Electronic Resource
    Electronic Resource
    Empfehlungen der bayerischen Muskelzentren in der DGM zur Heimbeatmung bei neuromuskulären Erkrankungen Erwachsener (1997)
    Springer
    Der Nervenarzt 68 (1997), S. 351-357 
    Details
    ISSN: 1433-0407
    Keywords: Schlüsselwörter Heimbeatmung ; Neuromuskuläre Erkrankungen ; Amyotrophe Lateralsklerose ; IPPV ; Respiratorische Insuffizienz ; Key words Home ventilation ; Neuromuscular disorders ; Amyotrophic lateralsclerosis ; Positive- pressure ventilation ; Respiratory insufficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary During recent years home ventilation has been shown to be useful for a growing number of patients with neuromuscular diseases and respiratory insufficiency caused by weakness of the respiratory muscles. Despite its technical simplicity, home ventilation leads to a number of social, ethical, medical and infrastructural problems. This consensus paper discusses the special situation of patients with neuromuscular diseases in home ventilation and describes the current thinking in the following topics: (1) definition of home ventilation; (2) aims of home ventilation; (3) indications and preconditions of home ventilation; (4) techniques and forms of home ventilatory support; (5) preparation for and practical questions of home ventilation; (6) stopping home ventilation.
    Notes: Zusammenfassung In den letzten Jahren wird eine zunehmende Zahl von Patienten mit neuromuskulären Erkrankungen und begleitender Ateminsuffizienz zu Hause oder in speziellen Einrichtungen dauerhaft oder intermittierend beatmet. Die praktische Durchführung einer Heimbeatmung wirft jedoch eine Fülle von organisatorischen, sozialen, ethischen und medizinischen Problemen auf. Mit dem Ziel, eine effiziente Durchführung von Heimbeatmungen zu gewährleisten, wurden die folgenden Empfehlungen der bayerischen Muskelzentren in der DGM unter besonderer Berücksichtigung der Situation von Patienten mit neuromuskulären Erkrankungen formuliert. Es wird im einzelnen zu folgenden Themenbereichen Stellung genommen: • Definition der Heimbeatmung, • Ziele der Heimbeatmung, • Indikationsstellung der Heimbeatmung und deren Voraussetzung, • technische Durchführung der Heimbeatmung, • Vorbereitung und praktische Durchführung der Heimbeatmung und • Beendung der Heimbeatmung auf Wunsch des Patienten.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001150050136
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  • 5
    Electronic Resource
    Electronic Resource
    Rezidivierende Querschnittsmyelitis Zwei Kasuistiken und Literaturübersicht (1998)
    Springer
    Der Nervenarzt 69 (1998), S. 1115-1122 
    Details
    ISSN: 1433-0407
    Keywords: Schlüsselwörter Myelits ; Querschnittssymptomatik ; Multiple Sklerose ; ADEM ; RDEM ; Key words Myelitis ; Tetraparesis ; Multiple sclerosis ; ADEM ; RDEM
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Recurrent transverse myelitis is a rare inflammatory demyelinating disorder, which is distinguished from acute monophasic transverse myelitis and from MS. We present two patients with recurrent transverse myelitis, who developed an acute remitting tetraparesis and sensible deficits with a cervical level. They were followed for 4 and 5 years. MRI showed an isolated lesion with contrast enhancement in the spinal cord but no pathological cranial findings. Oligoclonal bands were negative in repeated lumbar punctures. One patient showed lymphocytic pleocytosis during the first and second attack. Somatosensory and motor evoked potentials were abnormal during relapses while visually and brainstem acustically evoked potentials (VEP and BAEP) stayed within limits. Laboratory examinations for bacterial, viral or parasitic infections, antinuclear antibodies, Angiotensin-converting enzyme and the sedimentation rate were also normal. The differential diagnosis will be discussed in view of previously reported series. We give a review of the current literature and discuss the differential diagnoses.
    Notes: Zusammenfassung Die rezidivierende Querschnittsmyelitis (RQM) ist eine seltene entzündliche, vorwiegend demyelinisierende Erkrankung des Rückenmarks, die von der akuten monophasischen Querschnittsmyelitis und von der multiplen Sklerose mit Rückenmarksbeteiligung abgegrenzt wird. Wir stellen 2 Patienten mit mehrfach jeweils akut auftretender Tetraparese vor. In der Beobachtungszeit von 4 bzw. 5 Jahren zeigte die Magnetresonanztomographie (MRT) bei beiden einen solitären Herdbefund im Rückenmark bei jeweils unauffälligem cranialen Befund. Oligoklonale Banden wurden bei mehrfachen Liquoruntersuchungen nicht nachgewiesen. Bei einem Patienten fand sich im Liquor eine lymphozytäre Pleozytose während der ersten 2 Schübe. Die motorisch- und somatosensorisch-evozierten Potentiale (MEP und SSEP) waren pathologisch verändert, die visuell und akustisch evozierten Potentiale hingegen immer unauffällig. Laboruntersuchungen zum Nachweis bakterieller, vitaler und parasitärer Erreger, antinukleärer Antikörper, Angiotensin-konvertierendem Enzym sowie der BSG erbrachten keinen pathologischen Befund. Diese Patienten werden mit differentialdiagnostischen Überlegungen beschrieben und vor dem Hintergrund der Literatur diskutiert.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001150050391
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  • 6
    Electronic Resource
    Electronic Resource
    Standardisierter Fahrradergometertest bei mitochondrialen Myopathien Indikation, Störfaktoren und klinisch aussagekräftige Parameter (1998)
    Springer
    Der Nervenarzt 69 (1998), S. 472-484 
    Details
    ISSN: 1433-0407
    Keywords: Schlüsselwörter Koenzym Q ; Fahrradbelastungstest ; Laktat ; Laktat/Pyruvat-Quotient ; Mitochondriale Myopathien ; Key words Coenzyme Q ; Bicycle ergometry ; Lactate ; Lactate/pyruvate-ratio ; Mitochondrial myopathies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary
    Notes: Zusammenfassung
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001150050300
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