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<p>Amyotrophic lateral sclerosis (ALS) is a&#xa0;rapidly progressive, incurable neurodegenerative multisystem disorder that primarily affects the upper and lower motor neurons. Due to the low life expectancy of approximately 3&#xa0;years from diagnosis and the resulting low prevalence, the disease is considered rare despite an incidence of 2–3/100,000 population per year. Approximately 10% of cases are genetic, while 90% occur sporadically. The diagnosis is made clinically and supported by electrophysiology and neurofilament measurement. For the sporadic form, riluzole is the only therapy approved in Germany, prolonging life expectancy by an average of approximately 3&#xa0;months. For patients with an <i>SOD1</i> mutation (approximately 2% of all cases), the antisense oligonucleotide tofersen, which significantly slows down the disease process, has been available since 2024.</p>

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Diagnostik und Therapie der Amyotrophen Lateralsklerose

  • 期刊论文

  • 发表日期：

    2026年7月28日

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  • J. Dorst

摘要

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive, incurable neurodegenerative multisystem disorder that primarily affects the upper and lower motor neurons. Due to the low life expectancy of approximately 3 years from diagnosis and the resulting low prevalence, the disease is considered rare despite an incidence of 2–3/100,000 population per year. Approximately 10% of cases are genetic, while 90% occur sporadically. The diagnosis is made clinically and supported by electrophysiology and neurofilament measurement. For the sporadic form, riluzole is the only therapy approved in Germany, prolonging life expectancy by an average of approximately 3 months. For patients with an SOD1 mutation (approximately 2% of all cases), the antisense oligonucleotide tofersen, which significantly slows down the disease process, has been available since 2024.