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Nervenheilkunde 2019; 38(06): 402-408
DOI: 10.1055/a-0883-6709
Schwerpunkt
© Georg Thieme Verlag KG Stuttgart · New York

Therapie der Amyotrophen Lateralsklerose

From Bench to Bedside und aktuelle Studien in DeutschlandTreatment of amyotrophic lateral sclerosis (ALS): From bench to bedside and current trials in Germany
Moritz Metelmann
1   Klinik und Poliklinik für Neurologie, Universitätsklinikum Leipzig
,
Max Holzer
2   Paul-Flechsig-Institut für Hirnforschung, Medizinische Fakultät Leipzig
,
Andreas Hermann
3   Sektion für Translationale Neurodegeneration „Albrecht Kossel“, Klinik und Poliklinik für Neurologie, Universitätsmedizin Rostock
4   Deutsches Zentrum für Neurodegenerative Erkrankungen (DZNE) Rostock/Greifswald
› Author Affiliations
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Publication History

Publication Date:
12 June 2019 (online)

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ZUSAMMENFASSUNG

Die amyotrophe Lateralsklerose (ALS) stellt eine unheilbare neurodegenerative Erkrankung dar und ist durch eine Degeneration der Motoneuronen im Kortex und im Rückenmark charakterisiert. Durch einen rasch progredienten Abbau der Skelettmuskulatur, insbesondere der Atem- und Schluckmuskulatur, ist die Prognose der Patienten mit ca. 2 bis 5 Jahren nach Symptombeginn sehr eingeschränkt. Es bestehen unterschiedliche Hypothesen über die Pathophysiologie der ALS wie eine Glutamat-Exzitotoxizität, mitochondriale Störungen oder eine Degeneration der Motoneurone aufgrund von Proteinaggregatablagerungen im Zytoplasma. Ebenso konnten einige genetische Veränderungen mit der ALS in Verbindung gebracht werden. Aktuell stellt Riluzol das einzige zugelassene Medikament zur Behandlung der ALS in Deutschland dar, daneben wird in ALS-Zentren der Radikalfänger Edaravone im Off-label-use eingesetzt. Beide Medikamente sorgen nur für eine eingeschränkte körperliche Besserung bzw. einen Überlebensvorteil von wenigen Monaten. Daher werden weitere Therapiestudien u. a. an ALS-Zentren in Deutschland durchgeführt, die in dieser Übersichtsarbeit vorgestellt werden sollen.

ABSTRACT

Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease and shows a degeneration of motor neurons in motor cortex and spinal cord. The prognosis of the disease is restricted with a survival of 2 to 5 years after symptom onset caused by a progressive skeletal muscle reduction, especially of respiratory and swallowing muscles. Hypotheses regarding the pathophysiology of ALS include glutamate excitotoxicity, mitochondrial disruption or degeneration of motor neurons by protein aggregation in the cytoplasm. Likewise, genetic mutations have been associated with ALS. Currently, riluzole is the only approved drug in the treatment of ALS in Germany, while the radical scavenger edaravone is applied in ALS centres in off label use. Both drugs lead to a moderate physical improvement and survival benefit of a few months, respectively. Therefore, further therapy studies are ongoing inter alia at German ALS centres and are presented in this review article.

Schlüsselwörter

Amyotrophe Lateralsklerose - Therapie - Edaravone - Riluzol - Biomarker

Key Words

Amyotrophic lateral sclerosis - therapy - edaravone - riluzole - biomarker
 

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