Ziehen-Oppenheim syndrome
Related people
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.
Bibliography
- 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.