Zusammenfassung
Die amyotrophe Lateralsklerose (ALS) ist eine rasch progressive, degenerative Erkrankung des motorischen Nervensystems, welche gekennzeichnet ist durch einen fortschreitenden Untergang des 1. und 2. Motoneurons. Die Folge sind progrediente Paresen und Atrophien der Extremitätenmuskulatur, der Rumpfmuskulatur sowie der bulbär versorgten Muskeln. Der Großteil der Patienten verstirbt in der Regel innerhalb weniger Jahre nach Beginn der Erkrankung, meist an den Folgen einer respiratorischen Insuffizienz.
Die ALS ist die häufigste motorische Systemerkrankung, jedoch ist deren Ätiologie weitestgehend unbekannt. Die Erkenntnisse zu den genetischen Grundlagen, zur Pathogenese mit dem zugrunde liegenden Prinzip der selektiven Vulnerabilität von Neuronen sowie folglich auch zu möglichen Therapieansätzen sind noch lückenhaft.
Summary
Amyotrophic lateral sclerosis (ALS) is a progressive, degenerative disease of the motor system characterized by signs and symptoms of upper and lower motor dysfunction. This results in the presence of focal amyotrophies and pareses affecting voluntary muscles. Patients die after a few years, in most cases by respiratory failure. ALS is the most frequent motor neuron disease; however, its etiology and pathogenesis are only known in fragments. Its genetic basis is only partially understood and major gaps remain in the understanding of its pathogenesis with the basic principle of selective vulnerability and potentially resulting therapeutic consequences.
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Gastl, R., Ludolph, A. Amyotrophe Lateralsklerose. Nervenarzt 78, 1449–1459 (2007). https://doi.org/10.1007/s00115-007-2354-5
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DOI: https://doi.org/10.1007/s00115-007-2354-5
Schlüsselwörter
- Motoneuronerkrankung
- Amyotrophe Lateralsklerose
- Bulbärparalyse
- El-Escorial-Kriterien
- Respiratorische Insuffizienz