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Morgagni-Stewart-Morel syndrome
Also known as:
Morel's syndrome
Morel-Moore syndrome
Morgagni’s syndrome
Morgagni's trias
Morgagni-Morel-Stewart syndrome
Stewart-Morel syndrome
Stewart-Morel-Morgagni syndrome
Associated persons:
S. Moore
Ferdinand Morel
Giovanni Battista Morgagni
R. M. Stewart
Description:
The main features of this hereditary syndrome is a trias consisting of 1) hyperostosis frontalis interna, 2) adipositas, and 3) virilism and hirsutism.
A peculiar, noninflammatory, and usually benign osteopathy with usually symmetrical thickening of the frontal, parietal, or occipital bones due to deposits on the internal aspects of the squama frontalis. There may be associated menstrual disorders, virilism, hirsutism, mental disorders, fatigue, somnolence, visual disorders, vertigo, tinnitus, obesity, polyphagia, polydipsia, polyuria, loss of sense of smell, decrease in glucose tolerance, convulsions, and involvement of the second, fith, and seventh cranial nerves with hemiplegia and hemiparesis. Age of onset average about 45 years, and the incidence in females is ca 90 percent. Inheritance is autosomal dominant.
These typical lesions of the frontal bone with associated obesity and virilism were first described by Morgagni. Douglas Hunt Stewart in 1928 again emphasised the association with obesity; Ferdinand Morel in 1930 emphasised the accompanying menstrual disturbance, amenorrhea, and impotence; and S. Moore in 1955 delineated the specific nature of the changes that occur in the frontal bone. The term Morgagni-Trias was introduced by Folke Henschen (1881-) in 1937, but the term Morgagni-Stewart-Morel syndrome is now commonly used.
We thank Prof. Dr. Gerhard H. Müller, Germany, for correcting an error in the original entry.
Bibliography:
- G. B. Morgagni:
Adversaria anatomica. VI. Animadversio 74, Padua, 1719.
De sedibus et causis morborum.
Liv II. Venezia, 1761. A more detailed description.
- R. M. Stewart:
Localized cranial hyperostosis in insane.
Journal of Neurology and Psychopathology, London, 1928, 8: 321.
- F. Morel:
L'hyperostose frontale interne. Syndrome de l'hyperostose frontale interne avec adipose et troubles cérébraux.
Paris, 1930.
- F. Henschen:
Morgagni’s syndrome. Jena, 1937.
- S. Moore:
Hyperostosis cranii.
Springfield, Illinois, Thomas, 1955.
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