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Ziehen-Oppenheim syndrome

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A familial torsion spasm due to a lesion of the basal ganglia. It begins in one leg and gradually progresses to severe disability. It is characterized by an overextension or over flexion of the hand; inversion of the foot, producing a classical dromedary gait; latero- or retroflexion of the head, torsion of the spine with arching and twisting of the back, forceful closure of the eyes, and a fixed grimace. Usually unilateral. Onset between 5 and 15 years of age. Mostly in Semitic peoples. Both autosomal dominant, recessive, and X-linked forms have been described. Familial cases frequently in Jewish kindreds of eastern European origin. The Polish neurologists Edward Flatau (1869-1932) and Wladyslaw Sterling (1877-1943) described the same condition, also in 1911.


  • M. W. Schwalbe:
    Eine eigentümliche tonische Krampfform mit hysterischen Symptomen.
    Inaugural dissertation, Berlin, 1908. 36 pages.
  • G. T. Ziehen:
    Ein Fall von tonischer Torsionsneurose.
    Demonstrationen im Psychiatrischen Verein zu Berlin. Neurologisches Zentralblatt, Leipzig, 1911, 30: 109-110.
  • H. Oppenheim:
    Über eine eigenartige Krampfkrankheit des kindlichen und jugendlichen Alters (Dysbasia lordotica progressiva, Dystonia musculorum deformans).
    Neurologisches Zentralblatt, Leipzig, 1911, 30: 1090-1107.
  • E. Flatau and W. Sterling:
    Progressiver Torsionspasms bie Kindern. Zeitschrift für die Gesamte Neurologie und Psychiatrie. Originalien, Berlin, 1911, 7: 586-612.
We thank Piotr Flatau for information submitted.

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