- A dictionary of medical eponyms

Oppenheim's disease

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A rare congenital, noninherited but sometimes familial disorder, characterised by absence of muscular development with the lower extremities being the first involved, hypotonicity, and hyperflexibility.

Description

A rare congenital, noninherited but sometimes familial disorder, characterised by absence of muscular development with the lower extremities being the first involved, hypotonicity, and hyperflexibility. It is observed in floppy infants (excessive mobility of the joints permitting them to assume bizarre postures). Respiratory complications frequently occur. Children who survive more than 18 months after the onset of symptoms may show slow improvement.

Because many well-defined neurological disorders produce this clinical picture, there is doubt concerning the existence of Oppenheim disease as a specific entity. Most writers consider the term obsolete.

Oppenheim proposed the title "myotonia congenita" for this condition, thus generating confusion which has persisted until the present day. Myotonia congenita described by Asmus Julius Thomsen (1815-1896) in his own family in 1876 is a completely different disorder which is characterised by episodic muscular rigidity.

See also Ullrich's syndrome, under Otto Ullrich, German paediatrician, 1894-1957.

We thank Søren Nørby for correcting an error in our original entry.

Bibliography

  • H. Oppenheim:
    Über allgemeine und lokalisierte Atonie der Muskulatur (Myotonie) im frühen Kindesalter. Vorläufige Mitteilung.
    Monatsschrift für Psychiatrie und Neurologie, Basel, 1900, 8: 232-233.
  • H. Oppenheim:
    Textbook of Nervous Disease. New York, G. E. Steckert, 1911.

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