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Amyotrophe Lateralsklerose (ALS)

  • Albert C. LudolphEmail author
Living reference work entry
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Part of the Springer Reference Medizin book series (SRM)

Zusammenfassung

Bei der amyotrophen Lateralsklerose (ALS) kombinieren sich die Läsion der Betz-Zellen des motorischen Kortex und der Pyramidenbahn (des ersten Motoneurons), sowie der motorischen Vorderhornzellen (des zweiten Motoneurons). Dieses Degenerationsmuster erkannte der Erstbeschreiber der ALS, der französische Neurologe Jean-Martin Charcot, als pathognomonisch und stellte die bei seinen pathologisch-anatomischen Untersuchungen der ALS erhobenen Befunde zeichnerisch dar. Heute ist bekannt, dass die ALS dem Konzept einer Multisystemdegeneration unterzuordnen ist, die systematisch in neuroanatomisch definierten Stadien primär das zentrale Nervensystem erfasst. Die Ursachen der Erkrankungen der Motoneurone sind weitgehend unbekannt, und das Verständnis ihrer genetischen Grundlagen, der Pathogenese und die Entwicklung von Behandlungsansätzen stehen erst am Anfang.

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Copyright information

© Springer-Verlag GmbH Deutschland, ein Teil von Springer Nature 2018

Authors and Affiliations

  1. 1.Klinik für NeurologieUniversitätsklinikum UlmUlmDeutschland

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