Amyotrophe Lateralsklerose

Zusammenfassung

Die amyotrophe Lateralsklerose (ALS) ist die häufigste Motoneuronerkrankung im Erwachsenenalter und durch einen selektiven Motoneuronverlust im mittleren Lebensalter charakterisiert. Die Pathogenese der ALS ist nicht vollständig geklärt. Vermutet wird eine multifaktorielle Ätiologie, wobei z. B. genetische Faktoren, oxidativer Stress, ein veränderter RNA-Metabolismus und ein Defizit an Wachstumsfaktoren als mögliche Einflussfaktoren diskutiert werden. Das klinische Bild ist durch ein Nebeneinander von peripheren und zentralen Paresen gekennzeichnet. Betroffen sind die durch die kaudalen Hirnnerven innervierten Muskeln sowie die Extremitätenmuskulatur. Nach einem fokalen Beginn kommt es meist rasch zu einer Generalisierung der Paresen. Die Diagnosestellung erfolgt nach den revidierten El-Escorial-Kriterien, die Sicherheit der Diagnose richtet sich dabei nach dem Ausmaß der Ausbreitung der Symptome.An apparativer Diagnostik kann in erster Linie die Elektromyografie zur Diagnosesicherung beitragen. Nach dem Versagen zahlreicher Medikamentenstudien ist zum aktuellen Zeitpunkt weiterhin nur der Glutamatantagonist Riluzol zur kausalen medikamentösen Therapie der ALS zugelassen. Daher nimmt die symptomatische Therapie zur Linderung, z. B. der Spastik, des Speichelflusses, der Muskelkrämpfe und anderer Symptome, einen hohen Stellenwert ein.

Schlüsselwörter

Amyotrophe Lateralsklerose - Pathogenese - Klinik - Diagnostik - Therapie

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