Akuttherapie des Guillain-Barré-Syndroms und der myasthenen Krise

Zusammenfassung

Das Guillain-Barré-Syndrom (GBS), eine akute Polyradikuloneuritis, und die Myasthenia gravis (MG), die wichtigste neuromuskuläre Übertragungsstörung, repräsentieren die beiden neuromuskulären Krankheiten, die aufgrund krisenhafter Zuspitzung am häufigsten einer Intensivüberwachung und -therapie bedürfen. Dies begründet sich u. a. mit einer im Vorfeld unvorhersehbaren Krankheitsdynamik. Beim GBS gilt es durch Plasmapherese oder Gabe von Immunglobulinen die Schwere der Ausfallerscheinungen bis zum Überschreiten des spontanen Krankheitsnadirs abzuschwächen und bei eintretender Ateminsuffizienz durch Beatmung bzw. bei vegetativer Instabilität die kritische Krankheitsphase intensivmedizinisch zu überbrücken. Die Therapie der myasthenen Krise umfasst die symptomatische Therapie mit Azetylcholinesterasehemmern, die Immunsuppression mit Glukokortikoiden und Plasmaaustauschverfahren. Alternativ können Immunglobuline gegeben werden. Im Gegensatz zum monophasischen GBS ist bei der MG eine Langzeitimmunsuppression erforderlich, die individuell angepasst werden muss. Durch die moderne Intensivmedizin konnte die Mortalität des GBS und der myasthenen Krise entscheidend gesenkt werden.

Abstract

Acute inflammatory demyelinating polyradiculoneuritis or Guillain-Barré syndrome (GBS) and autoimmune myasthenia gravis (MG) represent the most prevalent neuromuscular diseases requiring intensive care due to respiratory or autonomic failure. Due to the unpredictable course of severely affected acute GBS patients and during myasthenic crisis, intensive clinical monitoring is mandatory for the early detection of impending respiratory insufficiency and timely elective intubation. Current immunomodulatory treatments in GBS by plasma exchange or IV immunoglobulins reduce the period of mechanical ventilation and the time required to achieve independent walking. Importantly, corticosteroids do not affect outcome. Patients with myasthenic crisis may partly respond to high-dose IV neostigmine together with glucocorticosteroids, but usually plasma exchange and / or immunoadsorption is required for rapid recovery. In contrast to GBS, MG requires long-term immunosuppression. Modern intensive care and immunomodulatory treatments have dramatically improved the prognosis of these potentially life-threatening neuroimmunological diseases.

Guillain-Barré-Syndrom - myasthene Krise - Akuttherapie

Guillain-Barré syndrome - myasthenic crisis - intensive care treatment

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