Neuralgische Amyotrophie: Klinik, Elektrophysiologie und Therapie

Zusammenfassung

Die neuralgische Amyotrophie (NA) ist eine zweiphasig verlaufende temporäre Erkrankung, die meistens den Plexus brachialis betrifft. Initial führendes Symptom ist eine akut einsetzende, vorübergehende, neuralgiforme Zervikobrachialgie, auf die temporäre Paresen und Atrophien der Schulter und/oder der Armmuskulatur folgen. In der Mehrzahl der Fälle kommt es zu einer völligen Restitution. Die elektrophysiologischen Befunde sind unspezifisch und zeichnen sich im Verlauf der Erkrankung durch Zeichen der axonalen Schädigung betroffener Nerven aus. Therapeutische Strategien bestehen in einer suffizienten Schmerztherapie zu Beginn der Erkrankung und einer intensiven Physiotherapie im weiteren Verlauf. Die Ätiologie der NA ist weit gehend unbekannt. Es werden immunologische und inflammatorische Ursachen angenommen. Aus diesem Grund kann die systemische Anwendung von Kortikoiden genutzt werden, um den Krankheitsverlauf zu modulieren. Neben der sporadischen NA gibt es eine autosomal dominante hereditäre Form, die hereditäre neuralgische Amyotrophie (HNA). Die HNA ist selten, kann aber, wenn es gelingt den genetischen und pathobiologischen Defekt aufzuklären, als Modell der sporadischen Form dienen.

Abstract

Neuralgic amyotrophy (NA) is characterized by a biphasic course affecting mainly the brachial plexus. The most striking clinical feature is the acute onset of neuralgic cervicobrachialgia followed by temporary paresis and atrophy of the shoulder and/or arm muscles. In most cases full recovery is seen. Electrophysiological findings are non-specific, and show signs of axonal damage of affected nerves. Therapeutic strategies are based on sufficient treatment of the initial pain, and in the course of the disease on physiotherapy. The ethiology of NA is unknown. Immunological and inflammatory causes are discussed. For this reason systemically administered corticosteroids may be used to modulate the course of the. The autosomal inherited form, Hereditary Neuralgic Amyotrophy (HNA) is rare. HNA may serve as a model of the sporadic NA, if the genetic and pathobiological defect will be clarified.

Key words

Neuralgic amyotrophy - hereditary neuralgic amyotrophy - brachial plexus - electrophysiology

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