Immunvermittelte Erkrankungen der neuromuskulären Übertragung

Zusammenfassung

Unter den immunologisch bedingten neurologischen Erkrankungen haben die Erkrankungen der neuromuskulären Übertragung große klinische Bedeutung. Klassisches Beispiel dafür ist die Myasthenia gravis. Diese erworbene Autoimmunerkrankung ist meist durch Antikörper gegen Azetylcholinrezeptoren bedingt. Eine Untergruppe der Myasthenie, bei der Azetylcholinrezeptor-Antikörper nicht nachweisbar sind, ist durch Antikörper gegen die muskelspezifische Rezeptor-Thyrosinkinase (MuSK-Antikörper) charakterisiert. Beim Lambert-Eaton-Syndrom bedingen Antikörper gegen spannungsabhängige Kalziumkanäle eine verminderte Ausschüttung von Azetylcholin in den synaptischen Spalt. Die Neuromyotonie ist eine Erkrankung, bei der häufig Antikörper gegen spannungsabhängige Kaliumkanäle nachweisbar sind. Diese neuromuskulären Übertragungsstörungen werden im Folgenden zusammenfassend vorgestellt.

Summary

Immune-mediated disorders occur at all levels of the nervous system. The most common disease of the neuromuscular junction is Myasthenia gravis. Myasthenia gravis is an autoimmune disorder caused by autoantibodies to the acetylcholine receptor or antibodies to the muscle-specific thyrosine-kinase (MuSK antibodies). There are two other immune-mediated disorders of the neuromuscular transmission. Antibodies to voltage-gated calcium channels are responsible for symptoms of Lambert-Eaton myasthenic syndrome. Neuromyotonia is an acquired disorder showing elevated antibodies levels to voltage-gated potassium channels. Diseases of the neuromuscular junction are reviewed.

Schlüsselwörter

Myasthenia gravis - MuSK-Antikörper - Lambert-Eaton-Syndrom - Neuromyotonie

Keywords

Myasthenia gravis - MuSK-antibodies - Lambert-Eaton-syndrome - neuromyotonia

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