Melkersson-Rosenthal syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Jesus Rosario Hernandez, M.D. [2].

Overview

Melkersson-Rosenthal syndrome is a rare neurological disorder characterized by recurring facial paralysis, swelling of the face and lips (usually the upper lip), and the development of folds and furrows in the tongue. Onset is in childhood or early adolescence. After recurrent attacks (ranging from days to years in between), swelling may persist and increase, eventually becoming permanent. The lip may become hard, cracked, and fissured with a reddish-brown discoloration. The cause of Melkersson-Rosenthal syndrome is unknown, but there may be a genetic predisposition. It can be symptomatic of Crohn's disease or sarcoidosis.

Treatment

Treatment is symptomatic and may include medication therapies with nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids to reduce swelling, as well as antibiotics and immunosuppressants. Surgery may be recommended to relieve pressure on the facial nerves and to reduce swollen tissue, but its effectiveness has not been established. Massage and electrical stimulation may also be prescribed.

Prognosis

Melkersson-Rosenthal syndrome may recur intermittently after its first appearance. It can become a chronic disorder. Follow-up care should exclude the development of Crohn's disease or sarcoidosis.

Eponym

It is named for Ernst Melkersson and Curt Rosenthal.[1][2][3]

Physical examination

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Head

Oral cavity

References

  1. Template:WhoNamedIt
  2. E. Melkersson. Ett fall av recidiverande facialispares i samband med ett angioneurotiskt ødem. Hygiea, Stockholm, 1928, 90: 737-41.
  3. C. Rosenthal. Klinisch-erbbiologischer Beitrag zur Konstitutionspathologie. Gemeinsames Auftreten von Facialislähmung, angioneurotischem Gesichtsödem und Lingua plicata in Arthritismus-Familien. Zeitschrift für die gesamte Neurologie und Psychiatrie, 1931, 131: 475-501.

Source

The original version of this article was taken from the public domain source at http://www.ninds.nih.gov/disorders/melkersson/melkersson.htm

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