Die neuralgische Amyotrophie: eine häufige Ursache uni- oder bilateraler Zwerchfellparesen

Zusammenfassung

Eine ein- oder beidseitige Zwerchfellparese kann verschiedene Ursachen haben, an erster Stelle stehen (intraoperative) Verletzungen des Nervus phrenicus. In bis zu 20% der Fälle lässt sich jedoch trotz umfangreicher Abklärung keine Erklärung finden. Die neuralgische Amyotrophie (NA, syn. Parsonage-Turner-Syndrom) ist eine häufige, im Alltag unterdiagnostizierte, multifokale autoimmun-inflammatorische Erkrankung, die überwiegend proximale Nervenabschnitte der oberen Extremitäten betrifft. Klassische Symptome sind akut einsetzende, starke Schmerzen im Bereich des Schultergürtels mit zeitverzögert auftretenden Paresen der Schulter- und Armmuskulatur. In mindestens 7% der Fälle ist der N. phrenicus (mit-)betroffen. Legt man die jährliche Inzidenz einer NA von 1:1000 zugrunde, ist die Entität als Ursache einer Zwerchfellfunktionsstörung wahrscheinlich nicht so ungewöhnlich, wie bislang angenommen. Die klinische Erfahrung zeigt jedoch, dass diese Diagnose oft nicht in Betracht gezogen und die Zwerchfellparese somit fälschlicherweise als idiopathisch klassifiziert wird. Fatal ist dies v.a. im Hinblick auf die Möglichkeit einer medikamentösen Therapie mittels Kortikosteroiden im Akutstadium einer NA sowie einer möglicherweise zu frühen operativen Zwerchfellraffung aufgrund des Potenzials der Erkrankung einer spontanen Heilung auch viele Monate nach Symptombeginn.

Ziel des vorliegenden Artikels ist es, das Bewusstsein für die Entität der NA als Ursache einer Zwerchfellparese zu schärfen und einen standardisierten Ansatz für die Diagnostik und Behandlung zu etablieren.

Abstract

There are several causes for unilateral or bilateral diaphragmatic paresis. The most common cause is an (intraoperative) injury to the phrenic nerve.

However, in up to 20% of cases, no explanation can be found despite extensive workup. Neuralgic amyotrophy (NA, also known as Parsonage-Turner syndrome) is a common underdiagnosed multifocal autoimmune-inflammatory disease that predominantly affects proximal nerve segments of the upper extremities. Classic symptoms include acute onset of severe pain in the shoulder girdle with delayed onset of paresis of the shoulder and arm muscles. In at least 7% of cases, the phrenic nerve is also affected. Based on the annual incidence of NA of 1:1000, the entity as a cause of diaphragmatic dysfunction is probably not as uncommon as previously thought. However, clinical experience shows that this diagnosis is often not considered, and diaphragmatic paresis gets wrongly classified as idiopathic.

This is particularly disastrous because in the early stage of NA, medical therapy with corticosteroids is mostly not considered and the possibility that surgical repair of the diaphragm may be performed prematurely, given that the condition may resolve spontaneously many months after symptom onset.

The aim of the present article is to raise awareness of the entity of NA as a cause of diaphragmatic paresis and to establish a standardized approach to diagnosis and treatment.

Schlüsselwörter

Zwerchfellparese - Atemmuskuläre Diagnostik - Seltene Erkrankungen - Parsonage Turner Syndrom - Neuralgische Amyotrophie

Keywords

Diaphragmatic paresis - Respiratory muscular diagnostics - Rare diseases - Parsonage-Turner syndrome - Neuralgic amyotrophy

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