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Essenzieller Tremor: State of the Art

Essential tremor: state of the art

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Zusammenfassung

Der essenzielle Tremor wird in der neuen Klassifikation als Tremorsyndrom mit im Vordergrund stehendem monosymptomatischem Aktionstremor beider Hände definiert. Ätiologisch liegen wahrscheinlich zahlreiche unterschiedliche Erkrankungen zugrunde, von denen erst einige monogenetische Erkrankungen bekannt sind. Wenn zusätzliche Symptome hinzukommen, die für sich kein neues Syndrom definieren, beispielsweise Haltungsstörungen oder Tandemgangstörungen, wird die Diagnose „essenzieller Tremor plus“ vorgeschlagen. Dem essenziellen Tremor liegt ein konsistent mit komplexer Elektrophysiologie erfassbares Netzwerk zugrunde. Trotz hoher Heritabilität ist die Genetik des essenziellen Tremors nicht aufgeklärt. Unter den vermuteten Untergruppen des essenziellen Tremors ist der altersassoziierte Tremor ein Kandidat. Bei spätem Auftreten ist er mit einer verkürzten Lebenserwartung verbunden. Therapeutisch werden Primidon und Propranolol als Mittel der ersten Wahl vorgeschlagen, danach soll Topiramat erprobt werden. Die tiefe Hirnstimulation des ventralen Thalamus ist eine erprobte Behandlungsoption für schwer betroffene Patienten.

Abstract

Essential tremor (ET) is currently classified as a syndrome rather than a unique disease, primarily involving monosymptomatic action tremor in both hands. Different etiologies are presumed to underlie this condition. Currently only a few monogenetic conditions are known to present with this syndrome. If accompanied by additional symptoms that do not in themselves constitute a new syndrome, such as abnormal tandem gait or postures, the syndrome should be diagnosed as “ET plus”. ET is associated with abnormal rhythmic activation of the cerebello-thalamo-cortical tremor circuit. Despite its strong heritability, the genetics of ET have not been elucidated as yet. Age-correlated tremor is one of the presumed subgroups of ET. Late onset is associated with a shortened life expectancy. From a treatment perspective, propranolol and primidone represent the drugs of first choice, followed by topiramate. Deep brain stimulation of the Vim nucleus of the thalamus is a proven treatment option in severely affected patients.

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Authors and Affiliations

  1. Klinik für Neurologie, Universitätsklinikum Schleswig-Holstein, Campus Kiel, Christian-Albrechts-Universität Kiel, Rosalind-Franklin-Str. 10, 24105, Kiel, Deutschland

    G. Deuschl & D. Berg

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  1. G. Deuschl
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Correspondence to G. Deuschl.

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G. Deuschl und D. Berg geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Deuschl, G., Berg, D. Essenzieller Tremor: State of the Art. Nervenarzt 89, 394–399 (2018). https://doi.org/10.1007/s00115-017-0475-z

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