Therapie der Amyotrophen Lateralsklerose – aktueller Stand

Zusammenfassung

Die Amyotrophe Lateralsklerose (ALS) ist eine progressive neurodegenerative Erkrankung, gekennzeichnet durch den Verlust der oberen und unteren motorischen Neurone. Als ursächliche Therapie ist aktuell lediglich die lebensverlängernde Wirkung von Riluzol bewiesen. Die zusätzliche Gabe von Vitamin E und C könnte oxidative Schädigungen reduzieren, ein positiver Effekt wurde jedoch bisher nicht bewiesen. Die Hoffnungen, die in die Einnahme von Kreatin gesetzt wurden, konnten bisher nicht bestätigt werden. Vielfältige Therapiestudien werden zurzeit durchgeführt, die eine Erweiterung der ursächlichen therapeutischen Möglichkeiten erwarten lassen. In der Betreuung ist die symptomatische Behandlung der ALS entscheidend. Grundlage der symptomatischen Therapie ist die Physiotherapie, oft kombiniert mit Logopädie und Ergotherapie. Diese Basistherapie wird durch die medikamentöse symptomatische Therapie und die Bereitstellung von Hilfsmitteln ergänzt. Zwei entscheidende therapeutische Optionen, die Anlage einer PEG-Sonde und die Möglichkeiten maschineller Atemunterstützung, benötigen rechtzeitige Aufklärung und Beratung. Zur Gewährleistung der Patientenautonomie sind Patientenverfügungen hilfreich.

Summary

Amyotrophic lateral sclerosis is a progressive neurodegenerative disease characterized by the loss of upper and lower motor neurons. Riluzole is still the only proven therapy to prolong survival. The additional use of vitamin E and C might reduce oxidative damage, but a positive effect of vitamin E or C has not been proven yet. The positive effect of creatine seen in transgenic mice has not been confirmed in humans until now. Numerous trials with several compounds are undergoing. One can expect that soon further treatment options are available to slow the progression of the disease. Symptomatic treatment is essential in the care of ALS patients. Basic therapies are physiotherapy, speech and occupational therapy. These basic treatments are amended by symptomatic pharmacological treatment and mechanical aids. Two essential therapeutic options, insertion of PEG and mechanically assisted ventilation, need to be discussed in advance. To guarantee patient autonomy written directives are helpful.

Schlüsselwörter

Amyotrophe Lateralsklerose - Pathogenese - Therapie

Keywords

Amyotrophic lateral sclerosis - pathogenesis - treatment

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