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DOI: 10.1055/s-0028-1090174
© Georg Thieme Verlag KG Stuttgart · New York
Myoklonien
MyoclonusPublikationsverlauf
Publikationsdatum:
18. Februar 2009 (online)
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Zusammenfassung
Das klinische Symptom Myoklonus ist charakterisiert durch eine plötzliche, unwillkürliche kurz anhaltende Muskelzuckung, die zu einem sichtbaren Bewegungseffekt führt. In der Regel ist der Ausgangspunkt eines Myoklonus zentralmotorisch. Klinische Merkmale und neurophysiologische Befunde helfen, den Ursprung eines Myoklonus auf kortikaler, subkortikaler, retikulärer oder spinaler Ebene näher zu bestimmen. Myoklonien können sich fokal, segmental, multifokal oder generalisiert manifestieren, wobei etwaige zeitliche Ausbreitungsmuster klinisch von diagnostischer Bedeutung sind. Myoklonien treten bei verschiedenen neurologischen Krankheitsbildern fakultativ oder obligat auf, finden sich aber auch im Rahmen primär internistischer Erkrankungen und bei Intoxikationen. Dies weist auf eine heterogene Pathophysiologie von Myoklonien hin, welche bisher nur in Teilaspekten verstanden ist. Myoklonien sind im klinischen Alltag häufiger ein Symptom als eine eigenständige Krankheit. Myoklonien treten bei epileptischen Syndromen auf oder sind zum Teil mit ihnen eng assoziiert. Neben diesen epileptischen Formen lassen sich physiologische, essenzielle und symptomatische Myoklonusformen abgrenzen. Symptomatische Myoklonien sind am häufigsten post-hypoxisch, entzündlich oder metabolisch bedingt, seltener durch Speichererkrankungen oder neurodegenerative Erkrankungen. Die Pharmakotherapie von Myoklonien gestaltet sich oft schwierig, kortikale Myoklonusformen respondieren in der Regel besser als nicht-kortikale Formen. Zum Einsatz kommen v. a. Antikonvulsiva oder Piracetam und 5-Hydroxytryptophan und Levetiracetam bei posthypoxischen Myoklonien. Breit angelegte Therapiestudien zur Behandlung von Myoklonien fehlen bislang. Dieser Übersichtsartikel legt den Schwerpunkt auf Krankheiten mit nicht epileptischen Myoklonien.
Abstract
Myoclonus is characterised by an involuntary sudden, brief and jerky movement caused by an abrupt muscle contraction. Usually, the generator of myoclonus is localised in the central nervous system. Clinical features and neurophysiological findings for different types of myoclonus are important to point out the anatomic level of myoclonus generation, which can be cortical, subcortical, reticular and spinal. Myoclonus can also be grouped by phenomenology with a focal, multifocal, segmental or generalised distribution. The pattern of myoclonic muscle activation yields clinical information on the type of myoclonus. Myoclonus is a symptom of various neurological disorders, but can also occur in metabolic disturbances, storage diseases and toxicities. Hence, with regard to these heterogeneous aetiologies of myoclonus, its pathophysiology is in part speculative and has to be further elucidated. In clinical practice myoclonus is rather a symptom, and disorders that constitute mainly of myoclonus are rare. Myoclonus can be classified into four aetiological categories: physiological, essential, epileptic, and symptomatic. Symptomatic myoclonus is by far the most common form of myoclonus and is caused most often by post-hypoxic encephalopathies, metabolic disturbances, and infectious encephalopathies. Neurodegenerative disorders and storage diseases are less frequent causes of symptomatic myoclonus. Treatment of myoclonus is more effective in cortical than in non-cortical forms of myoclonus and consists mainly of anticonvulsants. The most effective medications used to treat cortical myoclonus are clonazepam, valproic acid, and piracetam. Post-hypoxic myoclonus can be relieved by 5-HTP and levetiracetam. However, evidence of myoclonus treatment is based mainly on case series with only a few controlled studies. This review focuses on non-epileptic myoclonic disorders.
Schlüsselwörter
Myoklonien - Ätiologie der Myoklonien - myoklonische Syndrome - Behandlung der Myoklonien
Key words
myoclonus - aetiology of myoclonus - myoclonus syndromes - treatment of myoclonus
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Alexander Wolters
Universität Rostock, Neurologie
Gehlsheimer Str. 20
18147 Rostock
eMail: alexander.wolters@med.uni-rostock.de