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Amyotrophe Lateralsklerose

Symptomatische Therapie bulbärer Symptome

Amyotrophic lateral sclerosis

Management of bulbar symptoms

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Zusammenfassung

Die Symptome der amyotrophen Lateralsklerose (ALS) werden überwiegend verursacht durch die erkrankungstypische Degeneration des 1. und 2. motorischen Neurons. Weitere Systeme des zentralen Nervensystems (ZNS) sind ebenfalls betroffen, wie das autonome, sensorische und sensible System. Bei der ALS existiert bislang kein kurativer Therapieansatz. Daher kommt der symptomatischen Therapie für die Verbesserung der Lebensqualität, zur Prävention von Komplikationen und damit auch zur Prognoseverbesserung eine besondere Bedeutung zu. Etwa ein Drittel der ALS-Patienten klagen schon zu Beginn der Erkrankung über Veränderung des Sprech- und Schluckvermögens, verbunden mit Sialorrhö. Gelegentlich treten pathologisches Lachen und Weinen auf, die ohne entsprechende innere Regung ablaufen und als pseudobulbäre Symptome gelten. Die pathophysiologischen Zusammenhänge, die zu dieser pseudobulbären Affektstörung führen, sind bislang nicht ausreichend geklärt. In der Regel wird heute für die bulbären und pseudobulbären Symptome eine interdisziplinäre Betreuung angeraten. Für die bulbären ALS-Symptome kann eine leichte Verlangsamung der Progredienz durch Riluzol erreicht werden. Die Übersicht fasst die wesentlichen Erkenntnisse zur Epidemiologie und Pathophysiologie der die Lebensqualität und Prognose von ALS-Patienten stark beeinträchtigenden bulbären und der pseudobulbären Symptome zusammen. Die dargestellten symptomatischen Behandlungsmöglichkeiten berücksichtigen speziell pragmatische Aspekte in der ambulanten Therapie.

Summary

Symptomatic treatment of amyotrophic lateral sclerosis (ALS) is relevant in preventing complications and improving quality of life as long as curative therapies are still out of sight. About one third of ALS patients show disabling problems associated with dysarthria, dysphagia, sialorrhea, and a pseudobulbar affective disorder already in the early stages of ALS. A multidisciplinary approach is the cornerstone of symptomatic treatment of bulbar and pseudobulbar ALS features. Except for riluzole randomized controlled trials are lacking. Here, we review the current views with regard to epidemiology, pathophysiology, diagnosis, and practical aspects of treating bulbar and pseudobulbar symptoms.

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Interessenkonflikt

Der korrespondierende Autor weist auf folgende Beziehungen hin: MB, KR und KVT haben an verschiedenen Therapiestudien (Phase I/II und III) der ALS teilgenommen. Keine dieser Substanzen ist Gegenstand dieser Arbeit. Die Autoren erklären, dass keine Interessenkonflikte bestehen.

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  1. Neurologische Klinik und Poliklinik Würzburg mit Neuromuskulärem Zentrum, Josef-Schneider-Straße 11, 97080, Würzburg, Deutschland

    P. Kraft, M. Beck, A. Grimm, C. Wessig, K. Reiners & K.V. Toyka

  2. Abteilung Neurologie, Sana Klinikum Hof, Hof, Deutschland

    M. Beck

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  1. P. Kraft
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  2. M. Beck
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  4. C. Wessig
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  6. K.V. Toyka
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Correspondence to P. Kraft.

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Kraft, P., Beck, M., Grimm, A. et al. Amyotrophe Lateralsklerose. Nervenarzt 81, 1218–1225 (2010). https://doi.org/10.1007/s00115-010-3008-6

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