Alternative titles; symbols
SNOMEDCT: 82054006; ORPHA: 77296;
In addition to thickening of the inner table of the frontal bone, obesity and hypertrichosis may be present. This condition affects mainly females. Knies and Le Fever (1941) reported mother and 3 children affected. Thus, the disorder may be dominant, but whether autosomal or X-linked is not known. Lieberman (1967) has observed 5 affected females in 3 generations. No case of male-to-male transmission is known. Rosatti (1972) described a family with 12 affected members (10 of them females) in 4 successive generations. Gegick et al. (1973) found elevated serum alkaline phosphatase levels in about half of patients. Pawlikowski and Komorowski (1983) found hyperostosis frontalis in 43% of women with galactorrhea as compared with a population frequency of 2.5%. (At least 2 other groups reported similar frequencies.) Since hyperprolactinemia was found in many of these cases, the authors suggested that this and other features of the syndrome such as hirsutism, diabetes, and menstrual troubles may be related to hyperprolactinemia.
Gegick, C. G., Danowski, T. S., Khurana, R. C., Vidalon, C., Nolan, S., Stephan, T., Chae, S., Wingard, L. Hyperostosis frontalis interna and hyperphosphatasemia. Ann. Intern. Med. 79: 71-75, 1973. [PubMed: 4721177] [Full Text: https://doi.org/10.7326/0003-4819-79-1-71]
Knies, P. T., Le Fever, H. E. Metabolic craniopathy: hyperostosis frontalis interna. Ann. Intern. Med. 14: 1858-1892, 1941.
Lieberman, B. Personal Communication. Oakland, Calif. 1967.
Lieberman, B. Morgagni's syndrome--the evolution of an eponym. In: Proceedings of the XXIII International Congress of the History of Medcine, London, 2-9 September 1972. Vol. 1. London: Wellcome Institute of the History of Medicine (pub.) 1974. Pp. 117-122.
Moore, S. Hyperostosis Cranii (Stewart-Morel Syndrome, Metabolic Craniopathy, Morgagni's Syndrome, Stewart-Morel-Moore Syndrome (Ritvo), le Syndrome de Morgagni-Morel). Springfield, Ill.: Charles C Thomas (pub.) 1955.
Pawlikowski, M., Komorowski, J. Hyperostosis frontalis, galactorrhoea/hyperprolactinaemia, and Morgagni-Stewart-Morel syndrome. (Letter) Lancet 321: 474 only, 1983. Note: Originally Volume I. [PubMed: 6131191] [Full Text: https://doi.org/10.1016/s0140-6736(83)91470-8]
Rosatti, P. Une famille atteinte d'hyperostose frontale interne (syndrome de Morgagni-Morel) a travers quatre generations successives. J. Genet. Hum. 20: 207-252, 1972. [PubMed: 4659165]