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Klinische Neurophysiologie 2003; 34(4): 182-196
DOI: 10.1055/s-2003-812580
Übersicht
© Georg Thieme Verlag Stuttgart · New York

Elektrophysiologische Diagnostik des Guillain-Barré-Syndroms und verwandter Neuropathien

Electrophysiological Evaluation of Guillain-Barré Syndrome and Associated NeuropathiesV.  I.  Leussink1 , K.  Reiners1
  • 1Neurologische Klinik des Universitätsklinikums Würzburg (Direktor: Prof. Dr. K. V. Toyka)
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Publication History

Publication Date:
09 January 2004 (online)

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Zusammenfassung

In typischen Fällen eines klassischen Guillain-Barré-Syndroms (GBS), heute spezifischer als akute inflammatorische demyelinisierende Polyradikuloneuropathie (AIDP) bezeichnet, ist die Diagnosestellung nicht schwierig. Kennzeichnend sind - oft nach vorangehendem Infekt - eine rasch progrediente Lähmung, deren Maximum in der Regel nach 2, spätestens nach 4 Wochen erreicht ist, allenfalls leichte sensible Defizite oder Reizzeichen und die typische Liquorkonstellation einer isolierten Eiweißerhöhung ohne Pleozytose. Die Diagnose wird zwar nach wenigen Tagen, häufig aber nicht von Anfang an, gestützt durch den Nachweis einer symmetrischen demyelinisierenden Neuropathie mit proximodistalem Schwerpunkt. Schwieriger ist die zweifelsfreie Diagnosestellung bei untypischen Verläufen, wie z. B. den axonalen Verlaufsformen des GBS und den georegionalen Varianten. Während in den Anfangsstadien der Erkrankung die Neurographie mit Erfassung der proximalen motorischen Nervenleitung durch F-Wellen-Untersuchung eine wichtige diagnostische Hilfestellung bietet, sind ab der zweiten Woche myographische Untersuchungen besonders aufschlussreich bezüglich des Ausmaßes der axonalen Beteiligung und der Prognose. Schwerwiegende Komplikationen treten in einem Teil der Fälle durch autonome Funktionsstörungen auf, die sich im Verlauf parallel zu den motorischen Ausfällen verhalten. Insbesondere durch eine parasympathische Entzügelung können letale Bradykardien und Asystolien entstehen. Die Gefährdung bezüglich einer solchen Komplikation lässt sich am sichersten durch den Bulbusdruckversuch erfassen. Darüber hinaus ist eine Analyse der Herzratenvariation hilfreich. Therapeutisch kommen im Akutstadium bei Ateminsuffizienz oder Einschränkung der Gehstrecke unter zehn Meter gleichrangig eine Plasmapherese oder die Gabe von hoch dosiertem Immunglobulin G infrage. Trotz bestmöglicher Betreuung in der Akutphase wie auch in der Rehabilitation wird die Erkrankung auch heute noch von einzelnen Patienten nicht überlebt. Bei etwa der Hälfte der Patienten bleiben relevante Funktionsdefizite bestehen.

Abstract

The classical form of the Guillain-Barré syndrome (GBS) is the monophasic acute inflammatory demyelinating polyradiculoneuropathy (AIDP), often following a bacterial or viral infection. The diagnosis can be easily made if symmetrical pareses are rapidly progressive within two weeks after onset. Sensory symptoms are mild at first; pain may develop later. Typical CSF findings are elevated protein levels without pleocytosis. Nerve conduction studies may fail to establish demyelination early in the course of the disease but always do so later on. F-wave studies may be diagnostic while ordinary nerve conduction is still within normal limits. Rare axonal forms of GBS may be more difficult to recognise both clinically and neurophysiologically; needle electromyography serves to demonstrate muscle denervation owing to primary axonal GBS or secondary axonal degeneration that may occur in severe forms of the classical demyelinating disease. The autonomic nervous system is affected to a clinically relevant extent in more than one - third of GBS patients. The most dangerous complication arises from parasympathetic hyperactivity that may lead to lethal bradyarrhythmias. Patients at risk can be identified by heart rate variation monitoring and appropriate testing, in particular employing the eyeball pressure test. Therapy in moderately and severely affected patients requires plasma exchange or high-dose intravenous immunoglobulin that have been shown to be equally effective. Despite all efforts, GBS may still be lethal in some cases, and clinically relevant sequels of the disease may persist in about one-half of the patients.

Key words

Guillain-Barré syndrome - electrodiagnostic evaluation - disease monitoring - autonomic dysfunction - therapy

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Prof. Dr. Karlheinz Reiners

Neurologische Klinik · Universitätsklinikum Würzburg

Josef-Schneider-Straße 11

97080 Würzburg

Email: k.reiners@mail.uni-wuerzburg.de

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