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Erschienen in: Der Nervenarzt 2/2016

01.02.2016 | Amyotrophe Lateralsklerose | Übersichten

Amyotrophe Lateralsklerose

Eine Multisystemdegeneration

verfasst von: A. Hübers, A. C. Ludolph, A. Rosenbohm, E. H. Pinkhardt, J. H. Weishaupt, Dr. J. Dorst

Erschienen in: Der Nervenarzt | Ausgabe 2/2016

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Zusammenfassung

Hintergrund

In den letzten Jahren haben sich zunehmend Hinweise darauf ergeben, dass es sich bei der amyotrophen Lateralsklerose (ALS) nicht um eine reine Motoneuronerkrankung, sondern um eine Multisystemdegeneration mit einer Vielzahl nichtmotorischer Symptome handelt. Diese moderne Auffassung untermauerten neuropathologische und bildgebende Erkenntnisse.

Fragestellung

Es soll die Frage beantwortet werden, welche Erkenntnisse für das Vorliegen einer Multisystemdegeneration sprechen und was dies für Diagnostik und Therapie der Erkrankung bedeutet.

Material und Methode

Zusammenfassung und Bewertung neuester klinischer, bildgebender und neuropathologischer Studien.

Ergebnisse

Die aktuelle Studienlage belegt, dass Symptome der ALS weit über das motorische Nervensystem hinausgehen und insbesondere kognitive Funktionen, die Okulomotorik, das extrapyramidale System und die Sensibilität betreffen. Als neuropathologisches Korrelat findet sich eine stadienhafte Ausbreitung des Proteins „transactive response DNA binding protein 43 kDa“ (TDP-43) über funktionell verbundene kortikale Strukturen.

Schlussfolgerungen

Nichtmotorische Symptome kommen bei der ALS regelmäßig vor, auch wenn sie klinisch zumeist nicht im Vordergrund stehen. Das Wissen um ihr neuropathologisches Korrelat bietet neue Perspektiven für die Diagnostik, aber auch für die Therapie.
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Metadaten
Titel
Amyotrophe Lateralsklerose
Eine Multisystemdegeneration
verfasst von
A. Hübers
A. C. Ludolph
A. Rosenbohm
E. H. Pinkhardt
J. H. Weishaupt
Dr. J. Dorst
Publikationsdatum
01.02.2016
Verlag
Springer Berlin Heidelberg
Erschienen in
Der Nervenarzt / Ausgabe 2/2016
Print ISSN: 0028-2804
Elektronische ISSN: 1433-0407
DOI
https://doi.org/10.1007/s00115-015-0030-8

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