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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 246 (1999), S. 93-96 
    ISSN: 1432-1459
    Keywords: Key words Sural nerve biopsy ; Adverse effects ; Polyneuropathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Sural nerve biopsy is a valuable tool in establishing the diagnosis and investigating the underlying causes of peripheral neuropathies. Few investigations have been carried out in which the sequelae of this procedure have been described systematically. We studied the short-term adverse reactions in 110 patients and the long-term outcome of sural nerve biopsy in a subgroup of 54 patients after 5–32 months. Long-lasting sensory deficits were reported in 93%, dysaesthesia in 19% and mild persistent pain in 33% of the 54 patients. No significant differences were found between patients followed for 1–2 years and those followed for more than 2 years in the frequency and distribution of hypaesthesia and anaesthesia. However, dysaesthesia was less frequent after more than 2 years (6/32 vs 1/16), and persistent pain completely subsided within our observation period. We conclude that disabling sequelae regress and finally subside over time. If we assume that returning for follow-up visits may cause bias towards more severely affected patients, the overall prognosis may be even better.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1459
    Keywords: Key words Multiple sclerosis ; Autonomic dysfunction ; Orthostatic intolerance ; Sympathetic nervous system ; Power spectrum analysis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Autonomic dysfunction is frequently observed in patients with multiple sclerosis (MS), but clinical studies disagree on the frequency and type of abnormalities in autonomic function tests. Orthostatic dizziness (OD) has been reported in up to 49% of patients, but the pathophysiological mechanisms are poorly understood. This study investigated cardiovascular reflex tests and their association with OD in patients with MS in order to examine the hypothesis that the sympathetic nervous system is specifically involved in these patients. Forty patients with clinically active relapsing-remitting (n = 27) and secondary progressive MS (n = 13), aged 35.0 ± 8.5 years, were studied by parasympathetic (heart rate responses to the Valsalva maneuver, deep breathing, and active change in posture) and sympathetic function tests (blood pressure responses to active change in posture and sustained handgrip), and by spectral analysis of heart rate variability during rest and during standing. Results were compared to those obtained in 24 healthy volunteers, aged 29.4 ± 7.2 years. A standardized questionnaire was used to evaluate symptoms of orthostatic intolerance. Abnormal responses on at least one cardiovascular reflex test were observed in 40% of MS patients, compared to 17% of the control group, with a statistically significant involvement of the sympathetic vasomotor system. Orthostatic intolerance was reported in 50% of patients (controls: 14%, P 〈 0.006). Subgroup comparison of patients with and without OD suggests that orthostatic intolerance results from impaired sympathetic vasoconstriction. These results provide further evidence that the sympathetic nervous system is involved in patients with MS.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1433-0407
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 244 (1996), S. 23-29 
    ISSN: 1432-1459
    Keywords: Key words Distal myopathy ; Muscular dystrophy ; Miyoshi ; myopathy ; Muscle biopsy ; Magnetic resonance imaging
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Miyoshi myopathy (MM) is a rare distal myopathy that mainly occurs in Japan. And that is characterized by prominent involvement of the gastrocnemius muscles. Here we report two patients, brother and sister, from a German family. Onset of the disease was at the age of 20 and 22 years, respectively. In both siblings, there was an early and predominant involvement of the gastrocnemius muscles. Creatine kinase activity was elevated 37- to 95-fold above normal. Electromyography revealed fibrillations, positive sharp waves and a myopathic pattern, particularly in the distal muscles of the lower limbs. Histology of the gastrocnemius muscles showed myopathic changes consistent with muscular dystrophy. Occurrence in these two siblings but in no other family members was indicative of an autosomal-recessive inheritance. Our report indicates that MM may also be found in Germany, and that it should be considered in the differential diagnosis of distal myopathies.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1433-0407
    Keywords: Schlüsselwörter Multiple Sklerose ; Therapie ; Immunsuppression ; Eskalierende Immuntherapie ; Mitoxantron ; Key words Multiple sclerosis ; Treatment ; Immunosuppression ; Rescue therapy ; Mitoxantrone
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Mitoxantrone is an anthracenedione antineoplastic agent that has recently been shown to be effective in ameliorating disease activity in multiple sclerosis (MS) as indicated by clinical and MRI data. However, the role of mitoxantrone in escalating treatment of patients with frequent and severe relapses and with rapid progression of disability is less clear. In this retrospective analysis we report on 15 patients with severe relapsing-remitting (9 patients) and secondary progressive MS with superimposed exacerbations (6 patients) treated openlabeled with mitoxantrone in our Clinical Research Group from July 1994 to October 1998. Eleven of these patients (73%) were treated with azathioprine, interferon-beta-1b or cyclophosphamide before. The patients received mitoxantrone over a period of at least 12 months (19±6 months) with a single dose of 10 mg/m2 monthly for the first three months. Thereafter, infusions were repeated every 3 months (total dose 141 mg±45 mg). The annual relapse rate could be significantly reduced from 3,0±1,5 in the year before therapy to 0,5±0,5 during therapy. Nine patients (60%) were stabilised, while four patients (27%) showed an improvement of disability. The treatment was well tolerated with only minor side effects. These results although retrospectively obtained confirm previous trials showing that mitoxantrone may be useful in MS patients with frequent and severe exacerbations and/or a rapidly progressive course of the disease who have had other immunomodulatory medication.
    Notes: Zusammenfassung Schwer verlaufende Formen der multiplen Sklerose (MS) mit mehreren Schüben pro Jahr und inkompletter Remission oder mit rasch progredientem Verlauf und drohendem Verlust der Gehfähigkeit erfordern den Einsatz intensiver immuntherapeutischer Maßnahmen. Die Wirksamkeit von Mitoxantron bei der MS konnte in kontrollierten Studien nachgewiesen werden, doch besteht nach wie vor Unklarheit über den Stellenwert dieser Substanz im Rahmen einer eskalierenden MS-Therapie. Im Zeitraum von Juli 1994 bis Oktober 1998 wurden in der Klinischen Forschungsgruppe für Multiple Sklerose und Neuroimmunologie von etwa 1300 Patienten/Jahr 50 Patienten mit schwerem schubförmigem oder sekundär chronisch-progredientem Verlauf und überlagerten Schüben mit Mitoxantron behandelt. Fünfzehn dieser Patienten (9 Patienten mit schubförmigem und 6 Patienten mit sekundär chronisch-progredientem Verlauf) mit einer Therapiedauer von mindestens 12 Monaten wurden retrospektiv analysiert. Elf dieser Patienten (73%) hatten eine immunsuppressive Vorbehandlung mit Azathioprin, Interferon-beta-1b bzw. Cyclophosphamid. Über einen mittleren Zeitraum von 19±6 Monaten wurde Mitoxantron in einer Dosierung von 10 mg/m2 Körperoberfläche zunächst in vierwöchentlichen, dann in dreimonatlichen Abständen (mittlere Gesamtdosis 141±45 mg) gegeben. Unter dieser Therapie konnte die jährliche Schubrate signifikant von 3,0±1,5 im Jahr vor Therapiebeginn auf 0,5±0,5 Schübe/Jahr unter Therapie gesenkt werden. Neun (60%) der Patienten waren gemessen an der EDSS stabil, 4 (27%) verbesserten sich unter der Behandlung. Die Therapie wurde bis auf leichtere Nebenwirkungen, vorwiegend Übelkeit und Erbrechen, im allgemeinen gut vertragen. Diese Therapiebeobachtungen entsprechen dem in kontrollierten prospektiven Studien beobachteten positiven Effekt von Mitoxantron. Diese Behandlungsform kann im Sinne einer eskalierenden Immuntherapie bei schwer verlaufender MS und nach Versagen anderer immunmodulatorischer Maßnahmen erfolgreich eingesetzt werden.
    Type of Medium: Electronic Resource
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