Myoklonien

Zusammenfassung

Myoklonien sind sehr kurzzeitige unwillkürliche Bewegungen umschriebener Körperregionen, die durch unwillkürliche Muskelaktivität oder aber auch durch kurzes, unwillkürliches Sistieren von Muskelaktivität ausgelöst werden (negative Myoklonien, Asterixis). Im Erwachsenenalter sind idiopathische Myoklonus-Syndrom selten, wesentlich häufiger repäsentieren Myoklonien ein Symptom einer anderweitigen Grunderkrankung. Myoklonien können spontan, aktions-induziert, stimulus-sensitiv, rhythmisch oder arrhythmisch, fokal oder segmental imponieren. Sie können klinisch auf Grund ihrer Phänomenologie meist von anderen Bewegungsstörungen abgegrenzt werden. Schwierig kann die Unterscheidung von rhythmischen Myoklonien und einem Tremor sein. Myoklonien entstehen nahezu ausnahmslos im zentralen Nervensystem, wobei elektrophysiologische Verfahren helfen können, zwischen kortikalen, subkortikalen und spinalen Myoklonien zu unterscheiden. Bei einer Reihe von Krankheitsentitäten stellen Myoklonien typische und oft charakteristische Symptome dar. Da symptomatische Formen im Erwachsenenalter die Regel sind, ist neben der Basisdiagnostik eine sorgfältige individuelle Stufendiagnostik zur Aufklärung und Behandlung möglicher Ursachen essenziell.

Abstract

Myoclonus refers to sudden, involuntary and very brief movements of body parts or segments, which may be triggered not only by involuntary activation, but also by sudden and very brief interruption of voluntary muscle activation (negative myoclonus, asterixis). While essential myoclonus syndromes exist, these syndromes usually are complex and diagnosed during childhood. In adults, myoclonus is much more likely to be symptomatic. Myoclonus can be focal or segmental, arrhythmic or rhythmic, and may occur spontaneously or be triggered by voluntary activation, or as a response to external stimuli. In most cases, myoclonus can be clinically differentiated from other movement disorders. However, it can be difficult to distinguish between rhythmic myoclonus and tremor. Myoclonus mostly originates from aberrant neuronal activity in the brain or spinal cord. Only very rarely is myoclonus associated with peripheral nerve lesions. Neurophysiology may help to identify cortical, subcortical and spinal generators. Since the majority of cases present with symptomatic myoclonus, it is essential to consider a wide variety of underlying diseases in the differential diagnosis and schedule an individual diagnostic work-up.

Schlüsselwörter

Myoklonus - Bewegungsstörungen - Epilepsie

Keywords

myoclonus - movement disorders - epilepsy

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