Zusammenfassung
Neuromuskuläre Erkrankungen können in 4 Hauptgruppen unterteilt werden: die Motoneuronerkrankungen, die peripheren Neuropathien, die Transmissionsstörungen und die Myopathien, die die größte Gruppe bilden. Bei den Motoneuronerkrankungen ergeben sich Probleme hauptsächlich aufgrund der respiratorischen Probleme und der Prädisposition zu Aspirationen bedingt durch die fortschreitende Muskelschwäche. Depolarisierende Muskelrelaxanzien können myotone Reaktionen und massive Hyperkaliämien auslösen, gegenüber nicht depolarisierenden Muskelrelaxanzien besteht hingegen eine extreme Überempfindlichkeit. Bei den peripheren Neuropathien stehen kardiale Einschränkungen im Vordergrund, wobei autonome Regulationsstörungen die kardiovaskuläre Instabilität noch verstärken können. Auf negativ inotrope Effekte der Anästhetika muss daher besonders geachtet werden. Bei höhergradigen AV-Blockierungen kann der Einsatz eines Schrittmachers erforderlich sein. Beim Charcot-Marie-Tooth-Syndrom besteht eine erhöhte Sensitivität auf Thiopental. Die Transmissionsstörungen machen sich in erster Linie durch respiratorische Probleme bemerkbar. Die fluktuierende Schwäche der Bulbär- und Atemmuskulatur kann zu Schluckstörungen, rezidivierenden Aspirationen bis hin zu respiratorischen Insuffizienz führen. Aufgrund der reduzierten Anzahl der Acetylcholinrezeptoren kommt es zu einer stark erhöhten Empfindlichkeit gegenüber nicht depolarisierenden Muskelrelaxanzien, z. T. auch zu einem verminderten Ansprechen auf Succinylcholin. Zu beachten ist, dass verschiedene Medikamente (z. B. Antibiotika, β-Blocker u. a.), die die neuromuskuläre Übertragung verschlechtern, bei diesen Patienten zu einer erheblichen Verstärkung der Symptome führen können. Bei den progressiven Muskeldystrophien wird das anästhesiologische Risiko weitgehend durch die häufigen kardialen und pulmonalen Einschränkungen bestimmt. Succinylcholin birgt ein hohes Risiko eines hyperkaliämischen Herzstillstandes. Patienten mit einer metabolischen Myopathie sind besonders durch den Mitbefall des Myokards gefährdet, respiratorische Probleme sind hier seltener. Der Muskelstoffwechsel sollte mit entsprechender Substratgabe (je nach Krankheitsbild) unterstützt werden. Bei den Membranstörungen können Muskelrigidität (myotone Reaktion) oder -schwäche zu respiratorischer Insuffizienz führen. Neben depolarisierenden Relaxanzien können auch Cholinesterase-Inhibitoren und, je nach Krankheitsbild, auch Hypothermie und Erniedrigung oder Erhöhung des Serum-Kaliumspiegels myotone Reaktionen auslösen.
Abstract
The neuromuscular disorders described are divided into four groups: motoneuron diseases, peripheral neuropathies, disturbances of neuromuscular transmission and myopathies. In motoneuron diseases problems mainly result from respiratory insufficiency and the predisposition for aspiration caused by progressive muscular weakness. Depolarising muscle relaxants may elicit myotonic reaction and massive hyperkalemia. In contrast to non-depolarising muscle relaxants there may be an extreme hypersensitivity. In peripheral neuropathies the cardiac function is often limited whereby dysautonomia may enhance cardiovascular instability. The negative inotropic effect of anaesthetic agents must be observed with care and patients with higher degree of AV blocks may need a cardiac pacemaker during general anaesthesia. The Charcot-Marie-Tooth-Syndrom is characterized with a high sensitivity to thiopental. Disturbances of neuromuscular transmission frequently cause respiratory problems The fluctuating weakness of bulbar and respiratory muscles may impair swallowing and can lead to recurrent aspirations. Due to the reduced number of acetylcholine receptors the sensitivity to non-depolarizing muscle relaxants is elevated and the response to succinylcholine is reduced. Drugs reducing neuromuscular transmission such as antibiotics and β-blockers may enhance these symptoms and should be avoided. In progressive muscular dystrophies the anaesthetic risk is mainly dependent on cardiac and respiratory impairment. Administration of succinylcholine leads to the risk of hyperkalmic cardiac arrest. Patients with metabolic myopathies are also at risk due to the involvement of cardiac muscle but respiratory problems are less frequent. Muscle metabolism should be supported by administration of substrates depending on the underlying disorder. In membrane disorders muscle rigidity (myotonic reactions) or weakness may lead to respiratory insufficiency. In addition to the depolarising muscle relaxants also anticholinesterase drugs, hypothermia and dyskalaemia can evoke myotonic reactions.
Schlüsselwörter
Myopathie - Herzversagen - Respiratorische Insuffizienz - Maligne Hyperthermie - Anästhesierisiko
Key words
Myopathy - Cardiac failure - Respiratory insufficiency - Malignant hyperthermia - Anaesthetic risk
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Dr. Christoph P. Baur
Universitätsklinik für Anästhesiologie
Universität Ulm
Steinhövelstraße 9
89070 Ulm
eMail: christoph.baur@medizin.uni-ulm.de
