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Hirayama-Syndrom in Deutschland

Fallberichte und Literaturübersicht

Hirayama disease in Germany

Case reports and review of the literature

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Zusammenfassung

Das Hirayama-Syndrom ist eine in Europa seltene, juvenile, benigne, da zunächst langsam progrediente und nach wenigen Jahren stationäre Amyotrophie der distalen oberen Extremitäten mit Beteiligung meist der C7- bis Th1-innervierten Muskeln gewöhnlich einer Seite. Die Pathogenese dieser meistens sporadisch auftretenden Erkrankung ist bislang noch nicht gesichert. Am besten ist die Hypothese einer zervikalen Flexionsmyelopathie mit mechanisch-ischämischer Schädigung der Vorderhornneurone etabliert, daneben werden neurodegenerative und autoimmune Genesen diskutiert. Betroffen sind in der Regel junge Männer asiatischer Provenienz. Wir beschreiben hier drei deutsche Patienten kaukasischer Herkunft, bei denen nach Ausschluss relevanter Differenzialdiagnosen ein Hirayama-Syndrom diagnostiziert wurde und geben anschließend eine Literaturübersicht über das Krankheitsbild.

Summary

Hirayama disease is a juvenile benign distal upper limb muscular atrophy rarely observed in Europe, usually monomelic involving C7–Th1 innervated muscles. It is characterized by insidious onset and a self-limited course within a few years. The pathogenesis of this mostly sporadic disease is not fully clarified. Cervical flexion myelopathy with mechanical ischemic damage of spinal motoneurons is the best established pathogenetic hypothesis, but neurodegenerative and autoimmune causes are also debated. Typically, young men of Asian origin are affected. Here we describe three German Caucasian patients with Hirayama disease and provide an up-to-date review of the literature.

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Interessenkonflikt

Der korrespondierende Autor weist auf folgende Beziehungen hin: Workshop- und Kongressreisen: Ipsen Pharma GmbH, Medtronic GmbH, Merz Pharmaceuticals GmbH, Pfizer Pharma GmbH, Pharm Allergan GmbH, Solvay Arzneimittel GmbH, TEVA. Vortragshonorar: GlaxoSmithKline.

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Authors and Affiliations

  1. Klinik für Neurologie, Klinikum der Goethe-Universität Frankfurt, Schleusenweg 2–16, 60528, Frankfurt/Main, Deutschland

    J.-S. Kang, H. Laufs & U. Ziemann

  2. Klinik für Psychiatrie, Psychosomatik und Psychotherapie, Klinikum der Goethe-Universität Frankfurt, Frankfurt, Deutschland

    S. Jochem-Gawehn

  3. Klinik für Neurologie, Klinikum Kassel, Kassel, Deutschland

    A. Ferbert

  4. Klinik für Neurologie, Klinikum Lippe Lemgo, Lemgo, Deutschland

    P. Vieregge

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  1. J.-S. Kang
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  2. S. Jochem-Gawehn
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  3. H. Laufs
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Correspondence to J.-S. Kang.

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Kang, JS., Jochem-Gawehn, S., Laufs, H. et al. Hirayama-Syndrom in Deutschland. Nervenarzt 82, 1264–1272 (2011). https://doi.org/10.1007/s00115-011-3320-9

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