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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PD Dr. Peter Young
Klinik und Poliklinik für Neurologie · Universitätsklinikum Münster
Albert-Schweitzer-Straße 33
48129 Münster
Email: young@uni-muenster.de
